Paraneoplastic pemphigus appearing as bullous pemphigoid-like eruption after palliative radiation therapy.
Paraneoplastic pemphigus is a recently described disease in which patients have polymorphous skin lesions suggestive of both erythema multiforme major and pemphigus vulgaris in association with internal neoplasms, especially non-Hodgkin's lymphoma. These patients have characteristic autoantibodies that bind specific epidermal proteins. A Nikolsky-negative bullous pemphigoid-like eruption developed within the radiation therapy field in a 72-year-old man receiving palliative treatment for recurrent large cell lymphoma. The eruption rapidly progressed to a Nikolsky-positive bullous process more typical of pemphigus vulgaris with extensive involvement of respiratory epithelia. Despite aggressive treatment with high-dose corticosteroids and antibiotics, the patient rapidly succumbed. Results of immunofluorescence studies and autopsy findings confirmed the diagnosis of paraneoplastic pemphigus.
Fried
R
R
Dermatology Service, Department of Veterans Affairs Medical Center, Brooklyn, NY 11209.
Lynfield
Y
Y
Vitale
P
P
Anhalt
G
G
D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D005455:Fluorescent Antibody Technique; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000532:radiotherapy*; D008297:Male; D010166:Palliative Care / Q000009:adverse effects*; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000473:pathology; D010392:Pemphigus / Q000209:etiology* / Q000473:pathology; D011855:Radiodermatitis / Q000209:etiology* / Q000473:pathology
D001323:Autoantibodies