Pemphigus vulgaris with nail involvement presenting with vegetating and verrucous lesions.
We report the case of a 68-year-old female with longstanding insulin-treated diabetes mellitus, observed for the first time in our department in August 1999 with multiple painful erosive lesions of the oral cavity and many bullous or erosive lesions on the abdominal wall, back, and thigh. She also had vegetating and verrucous lesions, similar to common warts, involving the hands and feet, mainly on the palms, palmar surface of the fingers, and nail folds. Her lesions were present for 1 year. Skin and mucous biopsies showed the characteristic histopathologic findings of pemphigus vulgaris, with an epidermal intercellular IgG deposition on direct immunofluorescence. Histology of a warty lesion of the finger also showed suprabasal acantholysis. After partial improvement with low doses of oral steroids and azathioprine, her disease progressed to involve the oral cavity, trunk, hands, feet, and scalp. Control of her disease required successive treatments of mycophenolate mofetil and cyclophosphamide, as well as corticosteroids. A partial response was obtained with all these treatments. After being controlled by cyclophosphamide that was slowly tapered, she is now well controlled with azathioprine and oral steroids, showing only discrete lesions of the oral mucosa after 1 year of followup. We report this case of pemphigus vulgaris with unusual clinical aspects, namely vegetating and verrucous lesions as well as nail involvement, rarely described in this disease.
Mascarenhas
R
R
Dermatology Department, University Hospital, Coimbra.
Fernandes
B
B
Reis
J P
JP
Tellechea
O
O
Figueiredo
A
A
D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000188:drug therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007328:Insulin / Q000627:therapeutic use; D008775:Methylprednisolone / Q000009:adverse effects / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009260:Nail Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D010392:Pemphigus / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D012867:Skin / Q000737:chemistry / Q000473:pathology*
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D007328:Insulin; D003520:Cyclophosphamide; D009173:Mycophenolic Acid; D001379:Azathioprine; D008775:Methylprednisolone