Lichen Sclerosus

Epidemiology

• Prevalence unk (often asymptomatic, underreported) (Obstet Gynecol Surv 2012;67:55)

• Bimodal distribution: Prepubertal & postmenopausal females, w/ a mean age btw the 5th & 6th decade (Obstet Gynecol 2008;111:1243)

• Risk of malig transformation to squamous cell carcinoma

Pathology

• Atrophic epidermis ± hyperkeratinization (typically due to persistent scratching), homogeneous collagen layer w/ underlying lymphocytic infiltrate, blunting of rete ridges

Etiology

• Autoimmune component & genetic predisposition suspected

• Hormonal influences (low estrogen) & local inflamm responses may also play a role

Clinical Characteristics

• Vulvar pruritis is most common symptom

May also present w/ vulvar irritation, pain, burning, dyspareunia

• Ddx: Psoriasis, lichen simplex chronicus, lichen planus, menopausal atrophy, candidiasis, autoimmune disorders such as vitiligo

Physical Exam (Obstet Gynecol Surv 2012;67:55)

• Exterior vulva thinned w/ a white plaque-like appearance, “cigarette paper”

• “Keyhole” distribution around vulva, introitus, & anus

• Excoriations & lichenification may be present due to persistent scratching

• Labia majora & minora may eventually lose distinction & fuse

• No vaginal involvement

Diagnostic Workup

• H&P exam

• Bx of affected area

• Rule out concurrent infxn

Treatment (Obstet Gynecol 2008;111:1243)

• Topical antihistamines for symptom relief

• High-dose topical steroids: Clobetasol 0.05% ointment nightly for 6–12 w, followed by maint 1–3×/w (1 of many rx regimens). See steroid chart, below.

• Topical retinoids for sev cases

• Topical tacrolimus, 0.1% ointment twice daily, or pimecrolimus 1% cream twice daily (do not use for extended periods)

• Triamcinolone injections: 2nd-line agents, indicated for persistent dz

• Pts should return in 3-mo intervals during initial rx stages, until stable

• Lifetime surveillance in 6–12-mo intervals recommended

LICHEN SIMPLEX CHRONICUS

Epidemiology (Dermatol Clin 2010;28:669)

• Common cause of vulvar pruritis: Prevalence is unk

• Personal &/or FHx of atopy is common

Pathophysiology

• Vulvar irritation (caused by heat, sweat, clothing, contact dermatitis, topical products, atopic conditions, infxn) → intense & persistent scratching → lichenification

Clinical Manifestations

• Pruritis

• Sleep disturbances, often due to pruritis & intense scratching

Physical Exam

• Erythematous thickened epidermis & scaly vulvar plaques

• Vulvar skin may be hyperpigmented or hypopigmented, & appear leathery

• Excoriations may be present

Diagnostic Workup/Studies

• Bx shows chronic inflamm changes, hyperkeratinization, acanthosis

Treatment (Obstet Gynecol 2005;105:1451)

• Vulvar hygiene, sitz baths

• Rx of underlying d/o (ie, infxn)

• Avoidance of scratching: Gloves at night, barrier creams, occlusive dressing, cold pack

• Topical steroids: Hydrocortisone 1% applied to affected area daily for mild dz. Betamethasone 0.05% or clobetasol 0.05% applied daily for mod–sev dz.

• Antihistamines: Diphenhydramine or hydroxyzine 25–100 mg po q4–6h prn

LICHEN PLANUS

Epidemiology

• Prevalence of ∼1% of women (Obstet Gynecol 2008;111:1243)

• Most common in the 5th–7th decade of life in females

Pathology

• Chronic inflamm changes, band-like dermal lymphocytic infiltrate, basal layer liquefactive necrosis, colloid bodies, acanthosis, hyperkeratinization

• Erosive lichen planus = most common form: Painful, desquamative, ulcerative lesions of vulva, vagina, & mucous membranes (including oral). Can form scar tissue, adhesions, or synechiae.

• Dev of squamous cell carcinoma is uncommon but poss

Etiology

• Presumed autoimmune process resulting in chronic inflammation

Clinical Manifestations (Am Fam Physician 2000;61:3319; Obstet Gynecol 2008;111:1243)

• “P’s”: Planar, Purple, Pruritic, Polygonal, Papules, & Plaques

• Pruritis is most common symptom

• May also present w/ vulvar or vaginal irritation, pain, burning, dyspareunia, discharge refrac to conventional rx

Physical Exam (Obstet Gynecol 2008;111:1243)

• Erythematous, shiny plaques of the vulva & occ the vagina

• May present w/ desquamation, ulcerations, & loss of architecture

• Wickham striae: White, lacy formation overlying papular lesions

• Bullae, ulceration, erosion in sev cases

• Oral & nongenital cutaneous lesions often coincide

Diagnostic Workup

• H&P exam

• Bx of affected area:

Immunofluorescence staining reveals basement membrane fibrinogen & IgM cytoids

Treatment (Obstet Gynecol Surv 2012;67:55)

• Symptom relief: Sitz baths, vulvar hygiene, barrier creams or petroleum jelly

• High-dose topical steroids: Clobetasol 0.05% cream applied nightly for 6–12 w, followed by maint 1–3×/w

• Topical tacrolimus, 0.1% ointment twice daily

• Triamcinolone injections

• Oral steroids for sev erosive dz: Prednisone 40 mg po daily × 1 w → taper

• Immune mediators (after failure of other methods): Methotrexate, azathioprine, cyclosporine, hydroxychloroquine

• Surgical procedures for adhesions or synechiae: Indicated when other treatments have failed

• Chronic condition, w/ relapsing-remitting course depending on resp to rx

• Routine yearly surveillance, as dev of squamous cell carcinoma is poss

SEBORRHEIC DERMATITIS

Epidemiology

• Overall prevalence unk

• Higher prevalence in immunocomp

• Most common in 3rd–4th decade of life

Etiology

• Lipophilic fungi of genus Malassezia implicated as potential pathogens

Grow in sebaceous glands

May be related to impaired immune resp

Clinical Characteristics and Physical Exam

• May present as asymptomatic plaques, as dandruff, or as pruritic, inflamed lesions where sebaceous glands are present

• Erythematous, yellow, oily scaly plaques in areas of sebaceous glands: Scalp, face, eyebrows, nasal folds, auricular surfaces, chest, back, body creases, vulva

Treatment (NEJM 2009;360:387)

• Antifungal meds: Ketoconazole 2% shampoo/foam/gel/cream BID for 4 w (evid is based on rx of scalp seborrheic dermatitis)

• Topical steroids to control itching, erythema: Hydrocortisone 1% daily or BID for 4 w, clobetasol 17-butyrate 0.05% cream daily or BID for 4 w, betamethasone dipropionate 0.05% lotion daily or BID for 4 w

• Calcineurin inhib: Pimecrolimus 1% cream BID for 4 w

• Recurrent dermatitis: Maint rx once or twice weekly

• Oral steroids or isotretinoin in sev cases; usually in the immunocomp or for refract dz

HIDRADENITIS SUPPURATIVA

Epidemiology (NEJM 2012;366:158)

• Prevalence 1–4%

• Most common in the 2nd–3rd decade of life

• 3 times more common in women

Etiology

• Often related to hormonal changes (hyperandrogenism), obesity, smoking, & meds

Pathophysiology

• Abn shedding of keratinocytes → terminal follicles in areas w/ apocrine glands become occluded & rupture → chronic inflammation, abscesses, sinus tract formation

Clinical Characteristics and Physical Exam (NEJM 2012;366:158)

• P/w erythematous, painful, nodular lesions, hyperhidrosis, odor

• Axilla & perineal regions most common, in addition to inguinal, perianal, & vulvar regions

• Less commonly p/w strictures, fistulae, lymphedema, osteomyelitis

• Nodular lesions form abscesses → resultant drainage causes sinus tracts & scarring

• Depression, decreased quality of life

• Hurley staging: Stage 1 – localized nodules or abscesses w/o scarring or tract formation, Stage 2 – recurrent nodules or abscesses w/ scarring or tract formation, Stage 3 – widespread nodules or abscesses w/ scarring & tracts

Treatment (Am Fam Physician 2005;72:1547)

• Initial treatments: Proper hygiene, use of neutral soaps, warm compresses, lightweight loose-fitting clothing, weight loss, smoking cessation

• Anti-inflamm meds

• Antiandrogen meds (spironolactone, drospirenone, finasteride)

• Topical Abx (tetracycline, clindamycin), oral Abx for more sev cases (clindamycin, rifampin)

• Retinoids (isotretinoin)

• Intralesional or oral steroids

• Immune mediators (infliximab, cyclosporine)

• Surgical treatments: Incision & drainage, wide local excision, laser excision, unroofing or debridement. Usually reserved for widespread & sev dz.

FOX-FORDYCE DISEASE

Epidemiology

• Infrequent; <1%

• Most common in 2nd–4th decade of life

• Predominance in females (female to male ratio 9:1)

Etiology

• Keratotic occlusion of apocrine glands → gland rupture & papular eruption → pruritis & chronic inflammation

• Apocrine gland involvement is necessary for dx

• Often related to humidity, obesity, hormones, stress

Clinical Characteristics

• May be asymptomatic, but most often p/w intense pruritis

• Affects the axilla, areolar, perineum, & pubic regions

• Multi small, darkened or flesh-colored papules

• May be a/w anhidrosis

• Acanthosis, or thickened skin, may be present

Treatment (J Pediatr Adolesc Gyn 2011;24:108)

• Combination OCP

• Topical steroids (0.05% desonide or 2.5% hydrocortisone once to twice daily)

• Topical or oral retinoids (0.025% tretinoin cream once daily)

• Topical or oral Abx

• Surgical excision of apocrine glands or liposuction curettage in sev cases

GYN-DERM CYSTS

COMMON DERMATOLOGIC MANIFESTATIONS OF SYSTEMIC DISEASE

Crohn’s Disease

• Approximately 30% of pts w/ Crohn’s dz have gyn-derm complications. See Ch. 15.

Findings: Vulvar edema, ulcerations, inflammation, granulomas, “knife cut” lesions or fissures. Inflammation, granulomas of the ovary & fallopian tube. Sinus tracts, enteric fistulae to the female reproductive tract.

Rx: Topical steroids, topical metronidazole, intralesion steroid injections, surgical correction of fistulae

Autoimmune Disorders (Obstet Gynecol 2008;111:1243)

• Thyroid dz, vitiligo, pernicious anemia, SLE, atopic dermatitis, & alopecia areata have been a/w lichen simplex chronicus, lichen sclerosus, & lichen planus

Behçet Disease

• Diagnostic criteria: Recurrent oral ulcers & 2 or more of the following: Recurrent genital ulceration, ocular lesions (uveitis), skin lesions, or positive pathergy testing

• Rule out infxn as source of ulceration, such as HSV, syphilis, HIV, chancroid

• Treatments: Topical or intralesional steroids; may require systemic rx

Stevens–Johnson Syndrome

• Systemic hypersensitivity rxn causing edema, sloughing, &/or necrosis of mucous membranes, including lower genital tract

• Usually caused by meds; can also be secondary to infxn

• Rx: D/c medication, supportive care, Abx, wound care; systemic steroids & IVIG may be helpful

Drug Reaction

• Small, hyperpigmented lesions, erythematous plaques or bullae

• Genital, oral, & facial lesions are most common

• Local rxn to systemic or local administration of some meds, most commonly: Tetracycline, phenolphthalein, sulfa medications, NSAIDs & ASA

• Resolves w/ discontinuation of the drug

Erythema Multiforme

• Small, cutaneous target-like lesions

• Bullae & erosions of the genital, oral, & ocular mucous membranes

• May be a/w infxn (HSV most common) or due to drug rxn

• Rule out infectious source (ie, HSV, syphilis, mycoplasma PNA)

• Rx: Withdrawal of causative agent, oral antihistamines, topical steroids, wound care, rx of infxn if present