Notes on Bullous Dermatoses
Bullous Dermatoses
- Diverse group of skin diseases characterized by the formation of blisters on the skin and mucous membranes.
Classification of Bullous Dermatoses
- Pemphigus:
- True Pemphigus
- Pemphigus vulgaris
- Vegetative pemphigus
- Pemphigus foliage
- Erythematous pemphigus
- Brazilian pemphigus
- Pemphgoid:
- Bullous pemphigoid
- Scarring pemphigoid
- Herpetiform Dermatoses:
- Herpetiform dermatosis of Dühring
- Herpes of pregnant women
- Subcorneal pustulosis
- Familial Benign Pemphigus:
- Transient Acantholytic Dermatosis:
Pemphigus
- More common in women, especially in married individuals after age 50.
- Characterized by long-term course and the development of blisters that may lead to general health decline.
- Blisters rupture easily, leading to painful erosions.
Nikolsky Sign
- Erosion occurs when lateral pressure is applied to normal skin near lesions.
Diagnosis
- Monomorphic rashes of blisters on unchanged skin or mucous membranes.
- Acantholytic cells detected in smears.
- Histological examination reveals intraepidermal blisters.
- IgG complexes found in the epidermis.
- Circulating antibodies detected using indirect immunofluorescence.
Differential Diagnosis
- Other pemphigus variations, bullous pemphigoid, dermatitis herpetiformis, and various infections.
Treatment
- Corticosteroids, immunosuppressive agents, plasmapheresis, antibiotics, and vitamins.
Bullous Pemphigoid
- Typically a benign disease with blisters mainly affecting the elderly.
- Characterized by itchy blistering, primarily on the skin (not mucous membranes), and a negative Nikolsky sign.
- Skin biopsies show subepidermal blisters and linear IgG and C3 deposits on immunofluorescence.
Scarring Pemphigoid
- Primarily affects mucous membranes, notably the conjunctiva and oral cavity.
- Requires corticosteroid treatment and sometimes cytotoxic agents.
- Intensely itchy vesicular eruption; often symmetrical on extensor surfaces.
- Associated with gluten-sensitive enteropathy.
- Diagnosis made via IgA deposits and responsiveness to dapsone.
- Dapsone therapy combined with a gluten-free diet for effective management.