Special Issues in Genital Dermatology

Special Issues in Genital Dermatology

Peter J. Lynch

Libby Edwards

Psychological Aspects

Clinical discussions about genital disorders and their role in the patient’s psychological, social, and sexual function have historically been indirect, played down, or even avoided completely. In order to maximize the quality of care offered, these aspects need to be explored with virtually every patient presenting with a genital problem. There are two ways of doing so. One is to offer patients a chance to express these concerns indirectly by way of a computerized questionnaire filled out privately by the patient prior to a face-to-face interaction with the clinician. The other is for the clinician to take the initiative in bringing up this discussion at the time of the examination. Rarely, with individuals who by culture or temperament are very shy, it may be appropriate to defer this discussion until the second or third visit. Because of space limitations, only a few aspects or psychosocial and sexual function related to genital disease can be covered in this chapter.

Psychological factors obviously can play some role in the pathophysiology of all diseases and the degree to which they do so probably exists on a spectrum. Unfortunately, for most diseases, there is no consensus as to what these factors are and how important a role they play in the etiology of the disorder. Information is needed about the extent, severity, and duration of the psychosexual aspects that may be playing a part in the patient’s problem. Also unfortunately, even when this information is available, there is always controversy over the degree to which psychological factors cause the disease vs the degree to which they are the consequence of the disease. Essentially, this presents a “chicken and egg” situation that, while it can be discussed, can never be resolved to the satisfaction of all participants. With this in mind, we have divided this section into three segments: (1) psychosocial and sexual dysfunction may cause the disease, (2) psychosocial and sexual dysfunction may influence the course of the disease, and (3) psychosocial and sexual dysfunction may occur as a result of the disease.

Psychosocial Dysfunction May Cause Disease

The major genital disorders for which one of us (PJL) believes psychosocial and sexual dysfunction plays a significant etiologic role are (1) chronic idiopathic genital pain; (2) chronic itching and scratching in the absence of a recognizable disease; (3) fixed ideation that some aspect of the genitalia, although normal on clinical examination, is in fact abnormal (body dysmorphic disorder [BDD]); and (4) intentional or unintentional self-mutilation (dermatitis artefacta).

Chronic Idiopathic Genital Pain

Mucocutaneous pain can arise secondary to an underlying cutaneous or neurologic disorder or can occur as an idiopathic problem. The main idiopathic mucocutaneous pain disorders include unexplained pain involving the head (tongue, lips, face, and scalp) and the anogenital area (vulva, penis, scrotum, and anus). Idiopathic pain that occurs at these latter four sites is generally termed vulvodynia, penodynia, scrotodynia, and anodynia. Of these, only vulvodynia has been studied sufficiently to warrant discussion here. Nevertheless, one of us (PJL) believes that the information regarding vulvodynia, as cited below, can be generalized to pain occurring at the three other anogenital sites of involvement.

Nearly all patients with vulvodynia have psychological, social, and sexual dysfunction, although the degree to which this occurs is quite variable.1,2 The major question, of course, is whether the presence of pain causes this dysfunction or whether the dysfunction causes the pain. While the majority of clinicians currently favors the former explanation, the minority including one of us (PJL) favors the latter. Support for this latter point of view is perhaps best established by examination of the data suggesting that psychosocial and/or sexual dysfunction precedes the development of the pain. In that regard, there is arguably acceptable evidence that significant depression, anxiety, somatization, relationship dysfunction, and painful physical, sexual, or psychological trauma precede the development of vulvar pain.

Regardless of whether psychosocial and sexual dysfunction causes the pain or arises from it, all agree that vulvodynia is a very debilitating condition and that it has a dramatic adverse effect on quality of life (QoL).3 In fact, it appears that women with this condition have significantly poorer QoL than do women with most other
general dermatologic disease and also worse than women with other vulvar disorders.4 Finally, while both medical and procedural therapy can lead to good results for most patients with vulvodynia, it is clear that these individuals can also benefit from a variety of psychological approaches to therapy.5,6 A generally ignored aspect of chronic vulvovaginal symptoms, especially vulvodynia, is the impact on the partner, which can be enormous.7

Chronic Itching and/or Scratching in the Absence of Recognizable Disease

As is true for cutaneous pain, pruritus can arise either as an idiopathic process (psychogenic pruritus) or develop secondary to an underlying systemic, cutaneous, or neurologic (neuropathic) disorder (see Chapter 13). Psychogenic pruritus (including such disorders as neurotic excoriation, prurigo nodularis and pruritus with delusions of parasitosis) is associated with a variety of psychogenic problems, notably obsessive-compulsive behavior, anxiety, and depression.8,9,10

Psychogenic excoriation (“neurotic excoriation”) and prurigo nodularis are the terms used for those patients who chronically scratch, gouge, or pick at skin that is otherwise visibly normal.11,12 These conditions differ from the scratching and rubbing that occurs in pruritic skin disease such as atopic dermatitis and lichen simplex chronicus in several ways. First, there is no atopy or other recognizable underlying disorder. Second, the excoriations are appreciably deeper and thus usually appear as ulcers rather than erosions. And, third, individual gouge marks are separated one from another by areas of intervening normal skin.

On the other hand, patients with the more severe form of psychogenic excoriation, those who believe that their skin contains “bugs” (delusions of parasitosis) or fibers (Morgellons disease), are almost always delusional. These latter two conditions (which are essentially identical from a psychological viewpoint) only occur in the anogenital region when other parts of the body are also involved.

The fact that psychogenic pruritus responds well to psychotropic medications such as the tricyclics, benzodiazepines, SSRIs (selective serotonin reuptake inhibitors), and antipsychotic agents support the supposition that this form of itching is primarily related to psychological dysfunction.

Body Dysmorphic Disorder

Body dysmorphic disorder is defined as preoccupation with some slight, or nonexistent, defect in appearance that causes significant distress resulting in psychosocial or sexual dysfunction. BDD is quite common with an overall prevalence of about 2% of the general adult population, ranging up to about 20% of individuals presenting for rhinoplasty.13 In the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM 5), BDD is classified under the category of obsessive-compulsive disorder (OCD), disorders with an extremely high comorbidity existing between BDD and OCD. Recent data suggest that OCD and BDD share common genetic and environmental risk factors, clinical features, and sociodemographic profiles.13 Patients with both have higher rates of anxiety, schizotypal features, and suicidal ideation than with either alone. There is also comorbidity between BDD and other mental disorders such as depression, anxiety disorder, and social phobia.14,15 Patients with real but slight anatomical abnormalities may only be preoccupied at the OCD level of severity, whereas in patients in whom no abnormalities can be detected may be delusional.14

Not surprisingly, preoccupation with minor or imagined defects most often involves the face, head, and hair but it can also involve the genitalia.16,17 The level of preoccupation may be relatively mild and only obsessive in nature or it may be more severe, representing fully developed BDD. Preoccupation regarding the genitalia most often revolves around size or color.

In terms of preoccupation with size, men not surprisingly, most often focus on the penis perceiving that their normal size penis is too small.18 On the other hand, in women it is usually the misperception that their labia minora are too large or too asymmetric.19 Penile concerns in men have resulted in a huge industry involving the use of nonprescription medications advertised as “guaranteed” to result in penile enlargement. Similarly, misperception about labia size or asymmetry has resulted in a booming business for female genital cosmetic surgery.

There is very little published about preoccupation with genital color. However, a search of the internet reveals a considerable level of concern on the part of women regarding the perception that their external genitalia (primarily the labia majora) or perianal area are too darkly pigmented. Because of this concern, many products and services are offered for genital and anal bleaching. In addition, many women who develop vestibular pain examine the vulvar vestibule and perceive that the color is abnormally red. This perception may be reinforced by clinicians who on examination confirm the presence of “excessive” redness. This “excessive” redness is then perceived as an inflammatory process responsible for the occurrence of vestibular pain. This led to use of the term “vulvar vestibulitis.” However, several studies have demonstrated that normal, asymptomatic women, with entirely normal biopsies, frequently have a similar degree of vestibular redness and, in any event, no form of anti-inflammatory therapy has led to a decrease in redness nor an improvement in pain. On the other hand, some studies support the presence of inflammation but these studies are not convincing, leaving the subject unresolved.20 We believe that the red color is a normal finding unrelated to inflammation and the development of pain and thus support
the consensus classification of vulvodynia that continues the elimination of the word “vestibulitis.”21

A similar situation also occurs in men. A small number of men develop idiopathic scrotal skin pain and on self-examination, they perceive that excessive redness is present. They then believe that the redness is abnormal and that it is directly related to the development of their pain. This association may be supported by clinicians who are unfamiliar with genital skin color. The severity of preoccupation with this redness often reaches the level of BDD. However, invariably, examination by experienced clinicians reveals that the redness is within the normal variability of scrotal wall color and that there is no local pathology present. Very little has been written about this “red scrotum syndrome”22 but we have seen nearly 50 such patients, and an informal show of hands at dermatology meetings suggesting that it is appreciably more common than the medical literature would suggest. Management is discussed in Chapter 13.

Self-mutilation (Dermatitis Artefacta, Factitial Dermatitis, Nonsuicidal Self-injury)

Self-mutilation involving the skin is an uncommon condition in which individuals knowingly and repeatedly damage the skin through burning, cutting, abrasion, chemical application, or other similar behaviors.23,24 We exclude damage due to fingernails and those conditions that occur on a one-time basis such as tattooing and skin piercing. Self-mutilation can occur at any age, but most instances occur in adolescents and young adults. Characteristically, patients strenuously deny that they are doing anything injurious to the skin, and they may show an irrational lack of concern, considering the severity of the injuries.23 Women are over represented,23 and suicide ideation and even suicide sometimes occurs.24

Self-mutilation occurs in two major settings: with malingering, where secondary gain is the driving factor, and in those individuals with moderate to severe psychological impairment, where the behavior is carried out to satisfy an internal and unrecognized emotional need. The underlying psychiatric abnormalities present in these individuals are variable but include anxiety, depressive, bipolar, and personality disorders.25 In fact, personality disorders are so common, nonsuicidal self-injury is a diagnostic criterion for borderline personality disorder.25

These traumatically self-induced lesions are fairly easy to recognize although it is extremely difficult to prove that they occur directly as a result of self-induction. A major clue is the observation that they develop only where the patient can reach. The most common sites are the face, arms, and legs, but the genitalia may be involved in a small percentage of cases.26,27 There are very few publications in the medical literature about genital self-mutilation, but a short search on the internet suggests that the problem is much more common than physicians believe. Based mostly on anecdotal information, one of us (PJL) has concluded that major forms of mutilation (such as penile self-amputation) are more common in men, whereas less damaging behaviors such as genital cutting are more likely to occur in young women. A dramatic and disturbing example of nonsuicidal self-injury takes place in the 2010 movie, The Black Swan, and genital cutting figured prominently in the 2001 movie, The Piano Teacher.

Female genital mutilation (FGM) as a cultural ritual is not self-induced and does not lie within this area of self injury.

Psychosocial Dysfunction Influences the Course of Disease

Psychosocial problems play an important, but not causative, role for many genital disorders. Since this is true of numerous genital diseases, this section will focus on only two examples, atopic dermatitis and psoriasis, wherein psychological factors may play an important role regarding the time of onset, extent, severity, and duration of the disease. The published literature on these two disorders relates almost entirely to generalized forms of the disease, but it is reasonable to expect that when the genital area is involved, there will be even greater psychosocial dysfunction than is described for generalized involvement.

Atopic Dermatitis and Lichen Simplex Chronicus

As indicated in Chapter 5, we believe that lichen simplex chronicus represents the localized form of atopic dermatitis, and these two conditions will be treated as a single entity in this section. Atopic dermatitis occurs in only a portion of patients who are biologically predisposed to develop the disease because of underlying atopy or through defects (such as filaggrin mutations) in the differentiation of epithelial keratinocytes. Psychological dysfunction appears to be one of the major factors influencing which of these predisposed individuals develop the disease. Often these psychological aspects are present very early in life and frequently occur as a result of a dysfunctional family relationship.28 Moreover, infants and children with atopic dermatitis have an increased risk of developing attention-deficit hyperactivity disorder, major depression, anxiety, oppositional defiant disorder, and autistic spectrum disorder.29

For adults, several studies of patients with atopic dermatitis reveal increased levels of anxiety and depression.30,31 The presence of these two disorders leads to increased itching that in turn leads to incessant scratching. This then is responsible for the development of the “itch-scratch cycle,” which characterizes the disorder. In addition, there is evidence that patients with atopic dermatitis and lichen simplex have increased levels of somatization, obsession-compulsion, and suicidal ideation.32,33,34,35 In more psychoanalytic terms, atopic dermatitis patients
are often described as irritable, resentful, guilt-ridden, and hostile. Improved outcomes with psychological and educational intervention demonstrate the high level of importance that psychological factors play in the development of atopic dermatitis and lichen simplex chronicus.36


It is clear that there is a genetic, biologic predisposition for the development of psoriasis. But, as for atopic dermatitis, psychological factors appear to influence the time of lesion development, severity of disease, and response to therapy. There is some consensus that high stress levels, at least for some patients, precede the development and exacerbation of psoriasis.37,38 Patients with psoriasis have significantly higher levels of anxiety and depression than control populations and it seems reasonable to believe that their presence is caused at least in part by stress.33,39 Moreover, men with psoriasis consume more alcohol and patients of both genders, but especially women are more likely to smoke than do controls.40 These behavioral aspects may also be associated with the noted anxiety and depression.

Patients with psoriasis are also appreciably more likely to exhibit alexithymia (an inability to understand, process, or describe emotions), and it is possible that this personality trait plays a role in the development of their disease.41 Lastly, psoriatic patients are found to have higher than normal levels of stigmatization and social avoidance/attachment and tend to perceive that social support is lacking.42

Psychosocial Dysfunction Occurs as a Result of Disease

Poor health always has a detrimental effect on patients’ QoL and measurement of QoL by way of validated questionnaire surveys is probably the best approach to use in measuring psychosocial dysfunction.43 These surveys have been carried out for a very large number of dermatologic diseases but because of space limitations, only psoriasis and atopic dermatitis will be considered here. Five aspects regarding the results of these QoL studies are worth exploring.

First, chronic dermatologic disorders when compared to significant systemic medical disease appear to have a disproportionably large detrimental effect on overall QoL. This is not surprising given the adverse social reaction to those with visible disease compared to those with “invisible” diseases such as hypertension and diabetes. Moreover, there is an unwarranted fear in many the general population that skin diseases are contagious.

Second the magnitude of effect that the presence of our two sample diseases, psoriasis and atopic dermatitis, has on QoL is not trivial but instead is quite significant.44,45

Third, the detrimental effect on QoL of patients with psoriasis and atopic dermatitis increases with cutaneous disease severity. This too is to be expected because as severity increases, social and intimate interactions are likely to become more troublesome to the patient.

Fourth, a distribution of psoriasis and atopic dermatitis that involves the face and/or anogenital region increases the detrimental effect that these two diseases have on QoL in general and on intimacy in particular.46,47

Fifth, the assessment of disease severity and its impact on QoL is quite different between patients and clinicians. Clinicians very frequently believe that the decrement in QoL is much less than that perceived by patients. This is largely related to the fact that clinicians base their estimate of disease effect on QoL almost entirely on their observation of the extent and severity of the disease without taking into account what even trivial disease may mean to individual patients. The use of patientcompleted QoL questionnaires would alleviate this discrepancy between the viewpoint of the patient and the clinician, but, unfortunately, such surveys have almost only been used in a clinical research setting rather than for individual patient encounters. This needs to change as such underestimation of a patient’s perception of disease effect on QoL is demeaning to the patient and will inevitably have an adverse effect on the clinician-patient relationship and possibly also on the patient’s response to therapy.

Genital Disease in Childhood

Some genital conditions occur only in children or are more common in prepubertal children. Also, many skin diseases that affect both children and adults present unique questions or management when occurring in children.

Genital complaints produce anxiety in patients generally, with fears of malignancy, sexual functioning, fertility, and sexually transmitted diseases. Genital symptoms and abnormalities in children are fraught with even more anxiety in parents, who feel responsibility for their child’s disease, while being unable to alleviate it. Often, providers have investigated the possibility of sexually transmitted disease, or have broached the subject of sexual abuse, producing even more anxiety, defensiveness, and anger. Or, a parent’s overattention to the child’s symptoms can lead to either anxiety on the part of the child or enjoyment of the attention with subsequent manipulation.

The primary therapy for parents of children with genital complaints is reassurance, and words of caution regarding information they will find in social media. Printed handouts are supportive, as the written word is perceived as authoritative, and assures the parent that the problem is one recognized and shared by others. Their child is not alone, and both information and treatment of the condition are available. Specific reassurance that their condition is not the parent’s fault, and that it will not
affect fertility, sexual functioning, or the development of malignancy, is crucial to many families.

Normal Genitalia

The appearance of the prepubertal external genitalia varies with the age of the child and with the normal differences among individuals. The range of normal is often not appreciated, since the genitalia are generally not examined closely on routine childhood examinations. And, except for the hymen, there is little information on the normal variants and changes in the external genitalia.


Newborn female genitalia reflect the effects of maternal hormones. The presence of estrogen produces generous labia minora, which extend beyond puffy labia majora (Fig. 15-1). Symmetrical physiologic hyperpigmentation can occur. The mucosa is pink, resilient, and moist, with thick hymenal folds covering the small vaginal and urethral openings. Milky, physiologic secretions are often present.

When the effects of maternal estrogen disappear, the fat pads of the labia majora diminish, and the underlying labia minora become vestigial, and generally represent only anterior remnants of the clitoral frenulum (Fig. 15-2). The thin hymenal ring is present within the vaginal opening. There are several normal variations of the appearance and shape of the hymen. The mucosa is now thin and atrophic, often with erythema that worries parents. Friction, urine, stool, overwashing, soaps, etc. produce irritation in this atrophic, fragile skin. Tissues lack elasticity in early childhood, and this contributes to tearing with trauma. Many children also experience adhesions of the clitoral hood to the clitoris simply as a result of this mild vulvar irritation, just as midline labial adhesions occur. Both processes generally reverse in later childhood. However, a recent study has reported that a third of college women exhibit synechiae of variable severity of the clitoris to the clitoral hood,48 and these actually often persist into adulthood.49

Fig. 15-1. This newborn girl shows a vulva with a strong maternal estrogen effect, with puffy labia minora that extend beyond the large labia majora, and the hymen is pink, thick and protrudes as well.

Fig. 15-2. The prepubertal vulva shows only vestigial labia minora that consist of only the clitoral frenulum; the labia minora begin to enlarge when estrogen appears. The labia majora have lost their fat pad and are nearly flat.

With the onset of puberty, there is thickening of all vulvar and vaginal tissues, and midline hair growth is noted. The labia majora again produce more prominent fat pads, and the labia minora elongate and thicken to variable degrees. The clitoris enlarges, and the hymen thickens with enlargement of the central opening. As in infancy, the mucosa becomes pink, soft, elastic, and moist. White discharge is common as endogenous estrogens stimulate vaginal secretions, and pruritus occurs in some girls as a result of the constant and unaccustomed wetness.


The influence of maternal hormones on male genitalia is less pronounced. Newborn boys often exhibit swelling of the scrotum resulting from fluid collection in the tunica vaginalis during the birthing process or a hydrocele.
Hyperpigmentation of the scrotum and penile shaft may be more prominent at birth, especially in those with darker natural skin colors. Nearly all newborns exhibit phimosis, but by adolescence, only 6.8% of boys could not retract the foreskin.50 By adulthood, only 3.4% were found to have phimosis in a later review of the literature.51

The debate regarding the risks, benefits, and ethics of routine newborn circumcision rages on. Some point to the medical benefits of decreased penile cancer and skin diseases occurring on the glans in those who have been circumcised. Others strongly feel that circumcision for anything but a specific medical indication in a person below the age of consent is unethical. In fact, childhood circumcision was criminalized in Germany in 2012, although the outcry from the Jewish and Muslim communities resulted in recension of this law. The American Academy of Pediatrics policy statement reports that there are health benefits to circumcision, but not great enough to recommend this to all boys. However, with the complications being lower with newborn rather than later circumcision as well as the known health benefits, the American Academy of Pediatrics writes that parental decisions should be honored. The Canadian Pediatric Society also does not recommend routine newborn circumcision, and rates of circumcision are falling in both the United States and Canada.

Before puberty, the phallus is short and thin. Pigmentation is similar to the general body pigment. The scrotum is less pendulous, and the skin is soft, thin, and pink, with few rugae. Both testes are palpable as soft masses within the scrotal sac. Premature infants are more likely to exhibit undescended testes, which are usually unilateral. Generally, these will descend by age 9 months. Surgical correction is sometimes required, and those that do not descend are at greater risk of malignancy, even when corrected. Small milia may be present on the scrotum.

At puberty, there is enlargement of the penis, both in length and circumference, with thickening and development of the glans. The scrotal sac and testes also enlarge and become more pendulous. The skin of the scrotum darkens and thickens, developing a coarse texture and folding into rugae in most but not all males. Pubic hair is noted first along the base of the shaft and then extending into the inguinal creases and onto the medial thighs as it becomes darker, coarser, and curlier.

Labial Adhesions

Clinical Presentation

Labial adhesions are a fairly common occurrence, occurring in up to 3% of girls.52 These are seen primarily under the age of 3 years.53 These appear as midline fusion of the labia majora, resulting in variable narrowing of the introitus (Fig. 15-3). A survey of 108 girls showed that adhesions were posterior in 79%-93% of affected girls, sometimes with mid or anterior adhesions forming.53 Uncommonly, the vulvar vestibule fuses entirely, covering the urethra (Fig. 15-4). Girls with labial agglutination are usually asymptomatic unless urinary retention or infection occurs behind the adhesions. In a series of 425 girls with labial adhesions, over 82% were asymptomatic, although 4% experienced urinary tract infections
(UTIs).54 Occasionally, even incomplete adhesions are bothersome as urine can become partially trapped above the synechiae, with dribbling when the child stands.

Fig. 15-3. Posterior labial agglutination and very mild anterior labial agglutination have occurred in this child with lichen sclerosus, a known association.

Fig. 15-4. This child has experienced near complete labial agglutination, although there is still no functional disruption in urine flow.


The diagnosis is made by the clinical appearance. The smooth, flat surface that overlies the introitus can suggest absence of the vagina, but it is distinguished by the presence of a central thin translucent line of fibrous tissue at the site of fusion.


Synechiae usually occur as a result of mild chronic inflammation of the vulvar skin, resulting from mechanical trauma, infection, or skin disease, including lichen sclerosus or irritant dermatitis (Fig. 15-5). Some have suggested that, because topical estrogen generally lyses labial adhesions, low estrogen levels may play a role in the formation of these adhesions. However, serum levels of estrogen in girls with labial adhesions are the same as those in girls without adhesions. Still, the normal infantile estrogen-deficient (compared to postpubertal girls) vulvar epithelium is thin, easily irritated, and more likely to scar compared to well estrogenized vulvar skin. Vulvar epithelium scars easily in general, and even more so in atrophic prepubertal skin.


Generally, no treatment beyond robust reassurance is required, as adhesions regularly resolve by puberty.

The traditional therapy for complete or symptomatic adhesions is the local application of estrogen cream combined with gentle massage and good hygiene. A survey of the literature shows 50%-90% of those treated with estrogen cream and massage cleared, with recurrence of 30%, and 68%-80% of girls receiving topical corticoids cleared with 23% of recurrences.52 Surgical intervention has a success rate of 100% and recurrence of 0.55 However, no series divide out the girls with primary adhesions (idiopathic) from those with adhesions due to lichen sclerosus. The use of a topical corticosteroid is crucial for the treatment management of girls with adhesions on the basis of skin disease, especially lichen sclerosus, which can cause permanent scarring of other structures if not treated appropriately.

Fig. 15-5. Very slight posterior labial agglutination has formed, again in a setting of lichen sclerosus, but resolved with a topical corticosteroid.

Most vulvologists treat symptomatic labial adhesion with a superpotent topical corticosteroid ointment (much less irritating than a cream) such as clobetasol propionate 0.05% or betamethasone dipropionate ointment in augmented or optimized vehicle 0.05% applied very, very sparingly twice a day, with monthly follow-up. Those children with skin disease should be maintained on a lower potency corticosteroid ointment such as desonide ointment 0.05% daily or thrice weekly superpotent medication for several weeks beyond lysis of adhesions, or at least through puberty in the case of lichen sclerosus.

Rarely, surgical separation is required because of pain, infection, or urinary retention. In a cooperative child, this can be done using topical anesthesia with lidocaine/prilocaine; the frightened child is best treated under general anesthesia/conscious sedation. A well-lubricated cotton swab sometimes can be used to separate the labia. Estrogen cream or a corticosteroid ointment should be applied daily afterward until healed to prevent reformation of the adhesions. The area should be re-evaluated every few days initially by the clinician to detect early recurrence during healing.

Red Plaques and Papules

Diaper Dermatitis (Napkin Dermatitis)

Although diaper dermatitis is discussed as though this is a specific skin disease, this is actually any rash that occurs under a diaper. These rashes occur as a result of the unique environment of the diaper area and consist mostly of irritant contact dermatitis, friction, and, sometimes, Candidiasis. Modern diapers have rendered recalcitrant diaper dermatitis much, much more uncommon event.

Clinical Presentation

Diaper dermatitis is characterized by red plaques that show various degrees of scaling that may be somewhat obscured by moisture. This is covered by the diaper and sometimes exhibits maceration and erosion when severe. The morphology depends upon what factors are producing the dermatitis.

The most common issue causing diaper dermatitis is irritant contact dermatitis produced by urine and feces held against the skin. This appears as a rash occurring on the more convex surfaces that directly contact urine
and stool, and relative sparing of the skin creases (Figs. 15-6 and 15-7). The perineum, buttocks, mons, and upper thighs are most commonly involved. Sharp margination where the diaper ends can occur. The skin is pink to bright red with a shiny, glazed appearance, occasionally with edema and sometimes even blisters or superficial erosions (Fig. 5-30). Eroded skin is tender and painful, especially when it is in contact with irritating substances such as diarrhea, alcohols, or propylene glycol that is contained in diaper wipes.

Fig. 15-6. Diaper dermatitis most often occurs as an irritant contact dermatitis as a result of urine and feces held against the skin by the diaper.

A severe form of irritant dermatitis is termed diaper dermatitis of Jacquet, granuloma gluteale infantum, or pseudowarts (see section “Granuloma Gluteale Infantum”). This is characterized by sharply marginated, red nodules, often with overlying erosion that confers an umbilicated appearance. These typically affect the labia majora, perianal skin, and perineum (Figs. 15-8 and 15-9).

Fig. 15-7. Diarrheal stool is incredibly irritating, producing not only redness on the convex surfaces of the buttocks while sparing the relatively protected skinfold but even causing coalescing erosions.

Fig. 15-8. Genital skin sometimes demonstrates an unusual reaction pattern of discrete, infiltrated, monomorphous, usually eroded papules in response to chronic inflammation, in this case to long-lasting diarrhea. This pattern is called Jacquet diaper dermatitis, pseudowarts, or granuloma gluteale infantum.

Frictional dermatitis is most pronounced on the inner thighs, under the fastening tabs and waistband of the diaper, and on other surfaces that rub against the diaper. It consists of mild erythema and papules that wax and wane and respond quickly to diapering more often so that moisture is minimized.

Intertrigo from moisture and friction occurs within the skinfolds of the diaper area but also can occur in the neck creases, under the axilla, and between the fat rolls on the thighs. A relatively sharp cutoff where the dry skin begins is noted. Affected areas may be oozing, macerated, and red. Superficial sloughing of white hydrated skin is common.

When Candida albicans complicates diaper dermatitis, the infection preferentially affects the skinfolds, with deep, bright erythema showing surrounding peripheral scale (Fig. 15-10). Satellite collarettes or round erosions are characteristic.

The shiny, glazed appearance of buttocks and the coalescing satellite papules and pustules are characteristic
of Candida diaper dermatitis. This is confirmed by microscopic examination, culture, or response to therapy.

Fig. 15-9. These flat-topped papules in a symmetrical pattern are typical of Jacquet diaper dermatitis.

Fig. 15-10. When yeast plays a role in diaper dermatitis, the wet skinfolds are prominently involved, and classically with satellite lesions.

Psoriasis is another skin disease that less often contributes to diaper dermatitis, because psoriasis is a skin disease that is precipitated by irritation and injury, as occurs from the irritant diaper dermatitis. Often, psoriasis in the diaper area is unaccompanied, at least initially, by extragenital psoriasis.


The diagnosis of diaper dermatitis is made by the presence of erythema and scale, with or without erosions and maceration in the diaper area.

Specific diseases that preferentially affect the diaper area and can mimic routine diaper dermatitis include acrodermatitis enteropathica and Langerhans cell histiocytosis (see below). Patients with Kawasaki disease typically exhibit accentuation of their rash in the diaper area with desquamation late in the course of their disease.


Diaper dermatitis is multifactorial but primarily irritant contact, sometimes with candidiasis. The irritants include wetness and friction of a diaper over skin exposed to urine and stool. Occasionally, atopic dermatitis or psoriasis plays a role.


The prevention and treatment of diaper dermatitis is primarily aimed at changing the environment to eliminate common irritants and re-evaluating the dermatitis that remains. With the advent of gel-containing disposable diapers, diaper rashes have become much less problematic. For the past 30-40 years, these disposable diapers have been the preferred option for diapering infants and toddlers. Disposable diapers were advertised as more convenient, hygienic, and less likely to cause diaper dermatitis than a cotton diaper with a vinyl or rubber pant covering. This cotton core and vapor impermeable outer layer was a set up for causing irritant contact dermatitis and many families gladly welcomed a readily disposable solution. However, the cost is high, and diapers can be left on for prolonged periods. In the last 15 years, cloth diapers have received a makeover to create a modern reusable option that can be cost effective, less likely to produce diaper rash than traditional cloth diapers, and more environmentally conscious. Data regarding comparisons between disposable diapers and current reusable diapers in regard to the frequency of diaper rash are generally lacking. There are suggestions that disposable diapers are superior, but all cloth diapers are not identical, nor are all disposable diapers.56 There are a number of reports of increased numbers of infants with severe papulo-erosive Jacquet diaper dermatitis in those using reusable diapers, however.57,58

In addition to high-quality diapers and frequent diaper changes, the application of barrier cream to the involved areas is useful to protect from urine and feces, and supplying lipids to the epidermis; these should be applied at least twice weekly to the healthy diaper area.59 When diaper dermatitis is present, large amounts should be applied to the skin with each diaper change, and avoidance of further irritation from cleansing soaps and some diaper wipes. Most wipes are not problematic.59 However, care should be taken to ensure that the pH is appropriate to counteract the alkaline effects of faces and urine and maintain the slight acidity of the area, and that the chosen wipes are free of potential irritants such as alcohol, nonallergy screened fragrances, essential oils, soap, suboptimal surfactants, and harsh detergents (eg, sodium lauryl sulfate).59 Allergens to be avoided include methylisothiazolinone (MI), methylchloroisothiazolinone (MCI), bronopol (2-bromo-2-nitropropane-1,3-diol), and iodopropynyl butylcarbamate.59 Or, Water Wipes can be used. These contain only water and a trivial amount of grapeseed oil. These lack pH adjustment and antibacterial agents, but studies have shown wipes to be equivalent to washcloths, which also lack these features, and both avoid the potential irritating additives.

However, adherent stool is best removed by gently rinsing the perianal area with warm water and patting the skin dry. The application of a barrier cream to anogenital skin is more effective than the aggressive removal of feces by rubbing or the use of harsh cleansers. Mild corticosteroid preparations such as 1% or 2.5% hydrocortisone ointment (not cream) hastens resolution of inflammation when applied twice daily and covered with barrier cream or ointment. Potent corticosteroids should not be applied under diaper occlusion, which enhances the effect of the medication. Adrenal suppression and deaths due to immunosuppression have been reported in several infants treated with ultra potent corticosteroids under
diaper occlusion.60 Anticandidal agents should be added if there is a question of yeast. Nystatin is available in an ointment base and is less irritating than the azole creams. When disease is severe and erosions are present, antifungal medication should be administered orally until healing begins.

Persistence of dermatitis when these measures are taken usually indicates an ongoing irritant process or an alternative diagnosis. Chronic diarrhea is a common cause for persistence of dermatitis, and this predisposes to yeast. An evaluation of the child’s diet and growth parameters and a search for underlying disease such as infection or malabsorption are indicated. In the absence of diarrhea, a skin biopsy may be helpful to identify other primary cutaneous disease.

Seborrheic Dermatitis

Seborrheic dermatitis is a common form of dermatitis of the scalp infants but only affects anogenital skin when very severe, as part of a generalized eruption that preferentially affects other skinfolds as well, to include the axillae and neck.

First occurring at about 4-6 weeks of life but rarely after a year of age. The scalp is usually involved first, with greasy, yellow scales with minimal inflammation. The diaper area is the second most common area to develop a rash. Seborrheic diaper dermatitis consists of erythematous, sharply marginated scaling plaques with greasy, macerated scales (Fig. 15-11). Candida frequently superinfects the area. The inguinal creases are more severely affected, but in many cases, the groin and perineum are confluently involved. Spread to other flexural areas and even onto the trunk as isolated scaly plaques can occur. Unlike atopic dermatitis, seborrheic dermatitis is nonpruritic, although newborns do not have the ability to rub and scratch.

Fig. 15-11. The yellow scale with a somewhat greasy feel, and accompanying cradle cap and rash on other areas of the body demonstrate the diagnosis of seborrheic dermatitis.

The diagnosis of seborrheic dermatitis is usually made on a clinical basis in an infant with scalp and intertriginous erythema and yellowish scale.

The treatment of seborrhea of the diaper area consists of low-potency hydrocortisone ointment and barrier creams or ointments. Antifungal therapy can be helpful in the event of secondary candidiasis. The scalp should be treated for maximal improvement; this can be achieved by removing scale with mineral or baby oil. Antiseborrheic shampoos or mild baby shampoos, and mild topical steroid scalp solutions, such as hydrocortisone 1% or 2.5% to reduce recurrent inflammation, are also helpful. Scrubbing and shampoos with acids and other keratolytic agents should be discouraged.

Atopic Dermatitis (Eczema)

Atopic dermatitis is an extremely pruritic eruption that occurs when irritation precipitates rubbing and scratching, which, in turn, cause erythema, lichenification, and excoriation (Figs. 15-12 and 15-13) (see Chapters 5 and 13). The diaper area is relatively spared in infants because the skin is covered and remains moist, and the diaper helps to protect the skin from rubbing and scratching. Treatment consists of a topical corticosteroid and avoidance of irritants.


Psoriasis is a skin condition that is relatively uncommon in children in the genital area (see Chapter 5). This is a skin disease characterized by increased turnover of epidermal
cells, so the skin becomes thickened and covered by dense scale. It preferentially affects irritated or injured skin, so the genitalia and diaper area are often affected.

Fig. 15-12. Lichenification of the scrotum and penis are signs of the rubbing of atopic dermatitis.

Fig. 15-13. Chronic scratching of atopic dermatitis has produced this poorly demarcated red plaque with scale and crusting.

Plaques are red, well demarcated, and thickened (Figs. 15-14 and 15-15). Often, psoriasis also affects the umbilicus, scalp, and gluteal cleft, and the fingernails may exhibit small pits. Often, however, there are no extragenital, pathognomonic signs of psoriasis. Psoriasis of the skinfolds and diaper area can mimic seborrheic dermatitis as well as cutaneous candidiasis, which is often a secondary factor. Even a biopsy is characteristic but often not diagnostic. In unclear cases, the course of the disease eventually provides a diagnosis, because psoriasis is chronic and other areas of the skin nearly always become involved eventually, showing typical psoriatic lesions.

Fig. 15-14. Psoriasis exhibits sharply demarcated red plaques that, in the genital area, may show a glazed surface appearance rather than typical white scale.

Fig. 15-15. The prominent flaking scale of the glans is classic for psoriasis.

Treatment consists of careful local care and topical corticosteroids. For the occasional unfortunate child with severe or widespread disease, systemic therapy may be required.

Perianal Streptococcal Dermatitis (Perianal Bacterial Dermatitis, Perianal Streptococcal Cellulitis)

Perianal streptococcal dermatitis is a superficial bacterial infection that produces redness, scale and irritation of perianal skin. See also Chapter 5.

Clinical Presentation

Perianal streptococcal dermatitis typically occurs in 3- to 5-year-old children, and it is more common in boys than in girls. Most often, 2 to 3 cm of erythema extends outward around the anus with minimal induration (Figs. 15-16, 15-17, 15-18). In other individuals, this erythema is accompanied by painful anal fissuring and a mucoid discharge that makes defecation painful. Constipation and withholding of stool are common, and it is often unclear whether the fissure was produced by the primary disease or by passage of hard stool. Finally, well-demarcated, scaly, beefy red, crusted dermatitis with maceration of the anal verge can occur. In addition, induration of the perianal skin
and satellite-crusted plaques can be seen. Bleeding from the area is common. Girls can exhibit vulvar and vaginal involvement, and boys can experience penile involvement.

Fig. 15-16. Perianal redness and scale, sometimes with painful fissuring, are characteristic of perianal streptococcal dermatitis

In all forms of anogenital bacterial dermatitis, pruritus, excoriations, and lichenification from scratching and rubbing are common. These superimposed eczematous changes can delay the diagnosis.


The diagnosis of perianal bacterial dermatitis is made on clinical grounds and is confirmed by a routine culture that yields Streptococcus, most often group A β-hemolytic Streptococcus and sometimes group B β-hemolytic Streptococcus. It is important to ask the laboratory to culture for these organisms because many laboratories select for enteric pathogens from perianal swabs.

Fig. 15-17. The perianal redness of perianal streptococcal dermatitis mimics psoriasis, pinworms, irritant dermatitis, and Candidiasis.

Fig. 15-18. Occasionally, the infection of perianal streptococcal dermatitis can affect the vagina producing a purulent vaginal discharge with vulvitis, and this child also has an accompanying folliculitis confirmed on culture.

The differential diagnosis of perianal bacterial dermatitis includes pinworm infestation, irritant or Candida dermatitis, atopic dermatitis, psoriasis, inflammatory bowel disease, and sexual abuse. Scratching and rubbing of the skin resulting from pruritus are common and can confuse the diagnosis. All perianal dermatitis should be cultured to rule out a bacterial component.


Bacterial skin infection of the perianal skin, nearly always with group A and sometimes group B β-hemolytic Streptococcus, produces this distinctive dermatitis in children. Many patients diagnosed with perianal streptococcus have throat cultures positive for the organism without symptoms of pharyngitis. Presumably, digital contamination of the anus from the infected pharynx or other body site is the source of the infection.


The treatment of choice for perianal bacterial dermatitis consists of oral antibiotic therapy with concomitant mupirocin ointment several times a day. Beta-lactamase-resistant antibiotics, such as cephalosporines, amoxicillinclavulanate, macrolides and clindamycin, appear to be less associated with recurrence than penicillin and amoxicillin, which have about a 32% recurrence rate.61,62 Because recurrences are common, some clinicians support antibiotic therapy for as long as 21 days to allow for the skin to heal before discontinuation of medication. Mupirocin ointment applied two to four times a day may minimize recurrences as well. Stool softeners can be useful in children with painful defecation due to perianal inflammation and fissures, and hydrocortisone 1% or 2.5% ointment can minimize pain while awaiting improvement with the antibiotic.


Pinworms infection is a common cause of perianal pruritus in small children.

Clinical Presentation

The only symptoms of a pinworm infestation usually are dermatitis and pruritus of the perianal skin and/or vulva. Excoriations are often more prominent than obvious redness and scaling. Complaints of nighttime pruritus are the greatest complaint and may be severe enough to wake the child. Occasionally, a child describes pain rather than itching. Uncommonly, the worms migrate to the vagina in girls and cause vulvar dermatitis and a vaginal discharge or produce balanitis in boys. An uncommon complication is involvement of the appendix with pinworms, a cause of concomitant pinworms and abdominal pain.63


The diagnosis of pinworms is made in the setting of an unexplained perianal or vulvar pruritus, with or without a mild dermatitis, and is confirmed by response to therapy. Pinworms are usually difficult to find for diagnostic evaluation. At night, adult worms are occasionally seen on the perineum or in the anal canal when it is gently everted. Eggs can usually be collected using clear tape placed over the anus first thing in the morning before the eggs are disturbed. The eggs can be viewed adherent to the tape as thick-walled, ovoid structures most easily seen by light microscopy under low power.

The differential diagnosis of pinworm infection includes perianal bacterial dermatitis, atopic dermatitis, irritant dermatitis, and candidiasis. The presence of severe nighttime symptoms of perianal pruritus with minimal inflammation can help to distinguish this disorder from other forms of dermatitis.


Pinworm infestation is the most common form of helminth infestation seen in humans and is produced by infestation with Enterobius vermicularis. Pinworms are much common in children than in adults, with more than 20% of school-aged children acquiring this ubiquitous infestation.

Pinworm infestation begins by ingestion of eggs, usually on fingers that have contacted perianal skin or dirt with eggs. Eggs hatch in the duodenum and mature during passage through the intestines. Migration to the perianal skin occurs, and gravid females deposit their eggs on the anal verge, causing pruritus. Four to six hours after the eggs are deposited, they become infectious, and when the eggs are swallowed, the cycle begins again.


Time honored treatment of pinworms has been with mebendazole 100 mg in a single dose, and application of a mild topical steroid ointment for any dermatitis that is present. For unexplained reasons, mebendazole is no longer available commercially, but it can be obtained through compounding pharmacies. Pyrantel pamoate, in a single dose of 11 mg/kg, can also be used. Treatment of adults and children with albendazole is 400 mg orally, repeated once in 2 weeks.

Multiple family members are often affected at the same time, necessitating widespread simultaneous treatment to cure the infestation. Reinfection is common in schoolaged children.

Retreatment in 2-3 weeks is recommended because medication is ineffective against ova that may have been ingested at the time of the first treatment.

Acrodermatitis Enteropathica and Acrodermatitislike Eruptions

Acrodermatitis enteropathica is a rare skin disease produced by zinc deficiency, showing characteristic findings. Similar findings are associated with other nutritional deficiencies.

Clinical Presentation

The classic presentation of acrodermatitis enteropathica is a triad of periorificial dermatitis, diarrhea, and alopecia. Infants are irritable and have severe failure to thrive.

The dermatitis consists of periorificial erythematous, well-demarcated, and scaling plaques. At times, the skin may exhibit vesicopustular plaques and crusting (Figs. 15-19 and 15-20). It is most pronounced around the mouth, eyes, and genital area. Neck folds are often involved in infants, and the acral extremities may have similar scaly, erythematous, well-marginated plaques, especially in older children and adults. The diaper dermatitis is usually severe and refractory to standard therapy. Secondary infections with Staphylococcus and Candida are common. Nail dystrophy and paronychia are also common, as is stomatitis.

Fig. 15-19. These well-demarcated, annular, eroded plaques with crusted borders are characteristic of acrodermatitis enteropathica.

Fig. 15-20. Acrodermatitis enteropathica affects the perioral skin as well, again showing eroded and crusted plaques.


The diagnosis of acrodermatitis enteropathica is suspected based on the classical triad of dermatitis in a periorificial and acral distribution, alopecia, and diarrhea, and it is confirmed by low serum levels of zinc, alkaline phosphatase, and lipids. A skin biopsy is characteristic but not diagnostic; the upper part of the epidermis appears pale because of the presence of clear cells with balloonlike cytoplasm and loss of normal basophilia in early lesions. Subcorneal vesicles may be present in the upper epidermis, and there is diffuse parakeratosis. Hyperplasia of the epidermis and parakeratosis are seen in older lesions in a nonspecific pattern.

Severe cases of seborrheic dermatitis or psoriasis, especially those complicated by superinfection with Candida, can have a similar appearance, but accompanying extragenital skin is often pathognomonic. The presence of scalp dermatitis suggests seborrhea, and normal laboratory findings rule out acrodermatitis enteropathica.


Acrodermatitis enteropathica is the classic manifestation of zinc deficiency. There are two pathways, both involving inherited mutations of zinc transporters; one is a transient neonatal zinc deficiency, the other is classic acrodermatitis enteropathica.64 However, the skin findings can occur also in acquired nutritional deficiency states. All patients with acrodermatitis enteropathica have low levels of plasma zinc and other zinc-dependent metalloproteins such as alkaline phosphatase.

Symptoms of acrodermatitis enteropathica usually do not appear in breast-fed infants until weaning. A zinc-binding ligand that may be absent from the newborn’s intestine appears to be present in breast milk, thus accounting for this observation.

Similar acral dermatitis is seen in other nutritionaldeficiency states including biotin, protein, and essential fatty acid deficiency. These occur with cystic fibrosis, short bowel syndrome, anorexia nervosa, chronic illness, and Crohn disease. Organic acidemias also produce these skin findings, including maple syrup urine disease, methylmalonic acidemia, and phenylketonuria. When screening for zinc deficiency is normal, serum metabolic screening can differentiate among these diseases. In Middle Eastern adolescents, the defect develops as a result of ingestion of high quantities of phytate, which binds zinc and prevents intestinal absorption.


Treatment for acrodermatitis enteropathica consists of supplementation of zinc. Oral supplementation using zinc gluconate, acetate, or sulfate in doses of 5 mg/kg/d is recommended. Symptoms improve within 1-2 days of supplementation, and a response is noted in the dermatitis by 3-4 days. Hair growth begins in 2-4 weeks. After resolution of the disease symptoms, supplementation should be continued in the inherited form of the disease, with measurement of serum zinc levels once or twice yearly. The acrodermatitislike eruptions produced by other nutritional deficiencies are managed by identification of the cause of the deficiencies, and correction of these, just as the therapy of each metabolic disorders, requires its unique management.

Langerhans Cell Histiocytosis, Letterer-Siwe Disease Type

Langerhans cell histiocytosis, formerly called histiocytosis X, is a group of diseases produced by proliferation of Langerhans cells, antigen presenting cells of the immune system that reside primarily in the skin. Generally, this is a rare, multisystem disease that occurs primarily in children, favoring boys slightly over girls.

Clinical Presentation

Letterer-Siwe disease usually manifests in children aged 2 years or younger and affects internal structures as well as skin. Skin features are the presenting signs in most children, and systemic manifestations typically occur in weeks to months after the onset of the cutaneous disease.

Affected infants develop an erythematous, scaly rash on the scalp that mimicks seborrheic dermatitis. This also affects the intertriginous areas, including the inguinal creases and diaper area. Unlike seborrheic dermatitis or other inflammatory genital skin rashes, the scaling papules are infiltrated and firm (Fig. 15-21). The posterior auricular area and axilla are generally affected as well. Moist scale and crust often cover the papules, but the reddish brown or purpuric nature of the lesions can usually be appreciated. Ulcerations are common and may be present on the oral mucous membranes. Purpuric nodules on the palms and soles are a poor prognostic sign.

Letterer-Siwe disease is a systemic process, with dissemination of abnormal Langerhans cells into the skin and extracutaneous locations including the liver, bone marrow, lymph nodes, and central nervous system. This fulminant process can be fatal, even with early treatment.
Infiltration into the pituitary gland can lead to diabetes insipidus, and orbital infiltrates produce exophthalmos. Fever, anemia, thrombocytopenia, hepatosplenomegaly, and adenopathy are typical. Bony tumors may be hard to appreciate clinically. Some infants have a typical histologic appearance and never develop extracutaneous manifestations. These patients retrospectively have what is called self-healing reticulohistiocytosis.

Fig. 15-21. Letterer-Siwe form of histiocytosis X superficially resembles seborrheic dermatitis or yeast, with papules within the crural creases. However, the papules are infiltrated and do not respond to treatment for yeast or seborrhea.

The prognosis depends on the age of onset, the duration of symptoms, and the degree of systemic involvement. Onset after 6 months of age, the absence of thrombocytopenia and lung involvement, the lack of extensive systemic involvement, and the lack of purpuric skin lesions are all good prognostic signs.


The diagnosis of Letterer-Siwe disease is made with skin biopsy. Immunohistochemistry and occasionally electron microscopy can be used to confirm the diagnosis.

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Jan 8, 2023 | Posted by in GENERAL | Comments Off on Special Issues in Genital Dermatology

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