Chapter 50: Acantholytic Disorders Of Skin Flashcards

Question

Management for AKV

Answer 1

No therapy may be needed. Topical retinoids may flatten lesions. Destruction by cryosurgery, shave, curettage, or laser surgery can be effective. Oral acitretin has been helpful.

Answer 2

HHD usually presents between the second and fourth decades of life, predominantly at sites of friction (neck, axillae, inframammary, groin, perineum). The diagnosis is often delayed because HHD simulates common dermatoses such as eczema, tinea, and impetigo. Itch, pain, and malodor are common complaints. Signs include crusted weeping erosions, vesicopustules, expanding annular plaques with peripheral scaly borders and vegetating plaques with fissures (rhagades). Postinflammatory hyperpigmentation is frequent. Some patients have longitudinal white lines on the fingernails, and these can help to confirm the diagnosis. Disease may be limited to one or two sites, more widespread, or rarely generalized with erythroderma, but even mild disease reduces quality of life. Painful malodorous inguinal or perineal disease is particularly disabling. HHD koebnerizes into inflammatory dermatoses and has been exacerbated (or diagnosed) after triggers such as contact dermatitis, removal of adherent patch tests, UV irradiation, cutaneous infections, and scabies infestation.

Answer 3

**Segmental Disease:** Type 1 mosaicism, in which a postzygotic mutation in **ATP2C1** manifested as localized streaks of HHD along Blaschko lines, was reported in one patient. Type 2 mosaicism has been confirmed in a patient with severe linear involvement superimposed on symmetrical HHD. In the more severely affected skin, a postzygotic mutation caused loss of heterozygosity at the ATP2C1 locus **Papular Acantholytic Dyskeratosis:** presents as localized, whitish papules that tend to cluster on the genitalia and the inguinal folds. The identification of ATP2C1 mutations in two unrelated cases

Answer 4

1. Superimposed bcterial or candidal infection 2. HSV 3. Tinea 4. ACD 5. Skin cancers - SCC

Answer 5

ATP2A2 HHD is dominantly inherited with complete penetrance and is caused by mutations inactivating one ATP2C1 allele.

Answer 6

Involved skin displays widespread partial loss ofcohesion (keratinocytes may still be linked together by adherens junctions) between suprabasal keratinocytes with an appearance likened to a **dilapidated brick wall** . Clusters of loosely coherent cells float in suprabasal clefts or bullae. Dyskeratosis is usually mild

Answer 7

Minimize friction and sweating Antiseptic cleansing products and bathBadditives Topical antimicrobials (antibacterial, antifungal) Moderately potent or potent topical corticosteroids in combination with topical or oral anti-biotics or topical antifungal agents to control secondary infection Pain relief (topical or oral). Some patients require narcotics

Answer 8

GD is nonfamilial and often self-limited. It typically presents with an itchy rash on sun-damaged skin of the trunk. Itch may be intense and out of proportion to the signs, which comprise scattered pinkish or red-brown papules with variable hyperkeratosis, papulovesicles (rarely bullae), or less often eczematous plaques The neck, proximal limbs or both can be affected. GD has been described in association with widespread sun-induced lentigines. Guttate leukoderma, similar to that seen in DD, has been observed in dark skin. Unilateral GD has been reported, but this may have been segmental DD

Answer 9

Although GD shares clinical, histologic, and ultrastructural features with DD, **no mutation in ATP2A2** has been identified in GD; the pathogenesis remains unknown. GD may be triggered by factors that promote sweating, such as febrile illnesses, bed confinement, or occlusion, but is also common in the winter in older men with dry skin Other factors: UV or ionizing radiation, inflammatory dermatoses such as atopic eczema, renal failure, HIV infection, malignancies,norgan transplant, and some drugs.

Answer 10

Treatment is symptomatic and difficult, and the therapeutic response (or lack thereof) can be often disappointing. Precipitating factors, including heat, sweating, sunlight, and topical irritants,should be avoided. Emollients and antipruritics may be soothing. Topical medicaments, such as corticosteroids, retinoids, calcipotriol, or tacalcitol, relieve irritation. Oral retinoids (acitretin), oral corticosteroids, UVB, psoralen and ultraviolet A light (PUVA), and methotrexatehave been advocated for persistent disease, but controlled trials are lacking at this time. The combination of acitretin and phototherapy has been reported to suppress symptoms