From the Guidelines
Pemphigus vulgaris skin lesions are characterized by fragile, flaccid blisters that easily rupture, leaving painful erosions and ulcerations, as described in the most recent guidelines for the management of pemphigus vulgaris 1. These blisters typically begin in the oral mucosa before spreading to the skin, particularly on the scalp, face, chest, back, groin, and axillae. The lesions demonstrate a positive Nikolsky sign, where slight rubbing of apparently normal skin causes the epidermis to separate from the dermis. When intact, the blisters contain clear fluid and arise from normal-appearing or erythematous skin. After rupturing, they leave behind raw, painful, eroded areas that may coalesce into large denuded regions that can become infected and heal slowly with possible hyperpigmentation.
The characteristics of the skin lesions in pemphigus vulgaris can be summarized as follows:
- Fragile, flaccid blisters that easily rupture
- Painful erosions and ulcerations
- Positive Nikolsky sign
- Blisters contain clear fluid and arise from normal-appearing or erythematous skin
- Raw, painful, eroded areas that may coalesce into large denuded regions after rupturing
These lesions result from autoantibodies targeting desmoglein proteins (particularly desmoglein-3), which are essential components of desmosomes that maintain cell-to-cell adhesion in the epidermis, as noted in the guidelines for the management of pemphigus vulgaris 1. This autoimmune attack causes acantholysis, or separation of keratinocytes, leading to intraepidermal blister formation at the suprabasal layer. Prompt diagnosis and treatment with corticosteroids and immunosuppressive agents are essential to prevent extensive skin involvement and potentially life-threatening complications, as recommended in the guidelines 1.
From the Research
Characteristics of Skin Lesions in Pemphigus Vulgaris
The skin lesions in pemphigus vulgaris are characterized by:
- Flaccid bullae that appear on the skin and mucous membranes 2, 3
- Erosions and crusted lesions that can be painful and tender 2
- Erythematous plaques that can occur on various parts of the body, including the chest, back, abdomen, arms, and legs 2
- Vegetating lesions in the large folds of the skin, which are characteristic of the pemphigus vegetans variant 3
- Oral ulcers that can be tender and painful 2
Clinical Presentation
The clinical presentation of pemphigus vulgaris can vary, but it typically starts with:
- Blisters and erosions on the oral mucosa 3
- Lesions on other mucous membranes and flaccid blisters on the skin 3
- Disseminated skin lesions that can occur on various parts of the body 3
Diagnosis and Treatment
The diagnosis of pemphigus vulgaris can be confirmed by:
- Cytological examination 3
- Histopathological examination 3
- Direct and indirect immunofluorescence tests 3 The treatment of pemphigus vulgaris typically involves:
- Systemic corticosteroids 4, 2, 3, 5
- Immunosuppressive drugs, such as azathioprine and mycophenolate mofetil 4, 2, 3, 5
- Rituximab, which can be effective in recalcitrant cases 4, 6