Other Dermatoses

16
Other Dermatoses


Any dermatological condition has a potential to occur on the genital skin but there are some where involvement at this site is more common (see Table 16.1) and these are discussed here.


Table 16.1 Other dermatoses.

























Example
Genetic disorders Hailey–Hailey disease
Autoimmune bullous disease Bullous pemphigoid
Pemphigus vulgaris
Mucous membrane pemphigoid
Bullous dermatosis of childhood
Drug eruptions Fixed drug eruption
Stevens–Johnson syndrome
Toxic epidermal necrolysis
Manifestations of underlying disease Necrolytic migratory erythema
Acrodermatitis enteropathica
Inflammatory ulcers Aphthous ulcers
Behcet’s disease
Lipschtuz ulcers
Others Graft‐versus‐host disease
Zoon’s vulvitis
Vulvo‐vaginal adenosis
Langerhans cell histiocytosis

Genetic disorders


Hailey–Hailey Disease (Familial Benign Chronic Pemphigus)


This is a rare blistering disorder inherited as an autosomal dominant trait. The lesions start in the 30s and tend to improve with age but the course is relapsing and remitting. The bullae are intraepidermal and recurrent vesicles and erosions occur in the flexures. The erosions may be crusted, forming plaques with a scaly border (Figure 16.1) and are often initially mistaken for intertrigo or candida infection. The problem is exacerbated by friction, heat and moisture. The outer vulva and inguinal folds are therefore commonly affected.

Photo displaying a vulva with Hailey-Hailey disease (pruritic papules on labium majus.

Figure 16.1 Hailey‐Hailey disease – pruritic papules on labium majus.


Histology shows acantholysis in the suprabasal layers of the epidermis. Squamous cell carcinoma has been reported to occur but this is rare.


Management


Simple measures include reducing frictional factors with weight loss being an important part of management. Control of any secondary infection is vital and long‐term antibiotics may be required in some patients. Emollients and a moderately potent topical steroid can be helpful in mild disease. Topical tacrolimus has been reported to be of benefit but a case of squamous cell carcinoma developing after treatment has been reported and it should therefore be used with caution. Other options include photodynamic therapy, CO2 laser, alefacept and botulinum toxin, but the evidence for these treatments is limited.


When to refer



  • If biopsy is required for diagnosis.
  • In cases of failure to respond to first‐line management or for more severe disease.

Practice points



  • Think of the diagnosis in persistent inflammatory disease of the flexures, not responding to simple measures.
  • Ask about family history.

Further reading



  1. Burge, S. M. (1992) Hailey‐Hailey disease: clinical features, response to treatment and prognosis. British Journal of Dermatology 126, 275–282.

Useful Web Site for Patient Information


The Hailey‐Hailey disease society:



Auto‐Immune Bullous Disease


In autoimmune bullous disease (Table 16.2), autoantibodies react against a component of the epidermis or basement membrane to produce a split in the epithelium, which presents as blistering or erosion. The genital area is mainly involved in bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP), where about half of the patients will have some vulval lesions. In linear IgA disease, 80% of children will have vulval involvement and it can present at this site. Pemphigus vulgaris (PV) and epidermolysis bullosa acquisita (EBA) may involve the vulva and vagina less commonly.


The diagnosis is made by taking a skin biopsy and sending for direct immunofluorescence (see Chapter 5) as the patterns seen are specific for each disease. In bullous pemphigoid, mucous membrane pemphigoid and Linear IgA disease, tense blisters are seen (Figure 16.2) as the split in the skin is deep at the level of the dermo‐epidermal junction. In pemphigus, blisters are rarely seen as the split is very superficial occurring in the epidermis. Erosions are therefore common.

Photo displaying bullous pemphigoid on the surface of skin.

Figure 16.2 Bullous pemphigoid – tense bullae.


Table 16.2 Clinical features and immunofluorescence patterns in auto‐immune bullous disease.


































Age Clinical features Immunofluorescence
Bullous pemphigoid Elderly Tense blisters Linear IgG and C3 at basement membrane (Figure 16.3)
Mucous membrane pemphigoid Adults, rare in children Vulval and vaginal lesions with scarring; ocular lesions Linear IgG at basement membrane
Pemphigus vulgaris Adults, usually middle aged Flacid, painful erosions; vaginal disease can cause a discharge IgG between keratinocytes in epidermis (Figure 16.4)
Linear IgA disease Children most commonly Tense blisters, may be clustered in children. IgA at basement membrane
Epidermolysis bullosa acquisita Rare, mainly adults Tense blisters  
Micrograph displaying a bullous pemphigoid with a linear deposition of IgG and C3 both along the basement membrane zone and the roof of the subepidermal blister.

Figure 16.3 Bullous pemphigoid: direct immunofluorescence shows a strong linear deposition of IgG (shown) and C3 both along the basement membrane zone and the roof of the subepidermal blister


(courtesy of Dr C. Stefanato)

Micrograph of the pemphigus with a positive intraepidermal intercellular deposition of IgG and C3, along with an intraepidermal acantholytic blister.

Figure 16.4 Pemphigus: Direct immunofluorescence shows a positive intraepidermal intercellular deposition of IgG (shown) and C3. Please notice the intraepidermal acantholytic blister


(courtesy of Dr C Stefanato).


Mucous membrane pemphigoid (cicatrical pemphigoid) is particularly important as the genital lesions lead to scarring (Figure 16.5), which can be difficult to distinguish from lichen planus and lichen sclerosus. The oral lesions are those of a desquamative gingivitis (Figure 16.6). Urethral and vaginal stenosis can occur and perianal lesions lead to pain on defaecation. Ocular involvement can be severe and lead to symblepharon and expert ophthalmological intervention is required in these patients.

Photo displaying vulva with mucous membrane pemphigoid with scarring and erosions.

Figure 16.5 Mucous membrane pemphigoid – scarring and erosions.

Photo displaying a gums and teeth with mucous membrane pemphigoid – desquamative gingivitis.

Figure 16.6 Mucous membrane pemphigoid – desquamative gingivitis.


In pemphigus vulgaris, vaginal lesions may occur. These can be erosive and scarring is therefore a potential problem. Significant vaginal disease can present with a discharge. The vulval lesions are those of erosions and superficial ulceration (Figure 16.7).

Photo displaying pemphigus vulgaris of the vulva.

Figure 16.7 Pemphigus vulgaris of the vulva.


Practice points



  • If an autoimmune bullous disease is suspected, refer to a dermatologist as these patients require specialized management with topical and systemic steroids and sometimes immunosuppression.
  • Direct immunofluorescence and indirect immunofluorescence is required to make the diagnosis.

Further Reading



  1. Batta, K., Munday, P. E. and Tatnall, F. M. (1999) Pemphigus vulgaris localized to the vagina and presenting as a chronic vaginal discharge. British Journal of Dermatology 140, 945–947.
  2. Marren, P., Wojnarowska, F., Venning, V. et al. (1993) Vulvar involvement in auto‐immune bullous diseases. Journal of Reproductive Medicine 38, 101–107.

Drug Eruptions


Fixed Drug Eruption


A fixed drug eruption causes the same lesions at the same site every time the drug is ingested and the genital area is one of the preferential sites for this type of drug reaction. It occurs within hours of taking the causative medication and itchy, erythematous lesions develop, which rapidly progress to blisters and erosions. The problem may be intermittent as common agents that cause a fixed drug eruption are analgesics and antibiotics. A very careful history is often required to identify the trigger. Occasionally a challenge test is needed to confirm the diagnosis. The lesions may heal with postinflammatory hyperpigmentation. The treatment is avoidance as the problem will recur with every exposure to the drug.


Common Drugs Causing a Fixed Drug Eruption



  • Nonsteroidal anti‐inflammatory drugs.
  • Paracetamol.
  • Aspirin.
  • Antibiotics – tetracyclines and sulphonamides.
  • Barbiturates.
  • Benzodiazepines.

Further Reading



  1. Fischer, G. (2007) Vulvar fixed drug eruption: a report of 13 cases. Journal of Reproductive Medicine 52, 81–86.
  2. Ozkaya‐Bayazit, E. (2003) Specific site involvement in fixed drug eruption. Journal of the American Academy of Dermatology 49, 1003–1007.

Useful Web Site for Patient Information


DermNet:



Stevens–Johnson syndrome


Erythema multiforme is a cutaneous reactive process either to a drug or infection (most commonly herpes simplex) where typical target lesions are seen. Stevens–Johnson syndrome is a severe form of this where mucosal disease is present in addition to the cutaneous lesions.


Symptoms

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Mar 15, 2018 | Posted by in OBSTETRICS | Comments Off on Other Dermatoses
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