Herein, we report a case of paraneoplastic pemphigus with mild skin features of pemphigus foliaceus and lichenoid stomatitis associated with B-cell lymphoma. A 49-year-old man presented with scattered blisters and erosions on the trunk along with mucosal blisters and erosions. Skin biopsy showed subcorneal acantholytic bulla and oral mucosal biopsy demonstrated lichenoid dermatitis. Direct immunofluorescence showed cell surface deposits of IgG and C3. Indirect immunofluorescence identified circulating IgG autoantibodies to the cell surfaces of normal human skin and also on the transitional epithelium of rat bladder. Enzyme-linked immunosorbent assay using recombinant baculoproteins showed positive antidesmoglein 1 autoantibodies (index 46) but negative antidesmoglein 3 autoantibodies (index 8). Immunoblot analysis using normal human epidermal extract detected BP230 and the 190 kDa periplakin, while immunoprecipitation using radiolabeled cultured keratinocyte immunoprecipitated BP230 and the 210 kDa envoplakin. We consider that the skin lesion was produced by humoral immunity whereas the oral lesion was produced by cellular immunity.
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http://dx.doi.org/10.1155/2010/931340 | DOI Listing |
An Bras Dermatol
January 2025
School of Public Health and Laboratory Medicine, Hunan University of Medicine, Huaihua, China; Department of Laboratory Medicine, Chronic Disease Research Center, Medical College, Dalian University, Dalian, China. Electronic address:
Dermatol Ther (Heidelb)
January 2025
Department of Dermatology, University Hospital Heidelberg, Im Neuenheimer Feld 440, 69120, Heidelberg, Germany.
Pemphigus vulgaris is a severe and often therapy-resistant bullous autoimmune disease. Standard therapy with steroids often administered together with another immunosuppressant does not respond in all patients or may not be a good therapeutic option in patients with severe underlying diseases. Intravenous immunoglobulins (IVIgs) represent a treatment alternative, often showing a rapid response which allows one to reduce concomitant immunosuppression.
View Article and Find Full Text PDFClin Dermatol
December 2024
Department of Dermatology, Yale University School of Medicine, New Haven, CT. Electronic address:
While rare, life-threatening dermatoses encompass various inflammatory, infectious, vasculitic/vasculopathy, paraneoplastic, and neoplastic skin diseases. Complications include skin barrier dysfunction, secondary infection, and internal organ involvement. Skin signs may serve as a critical window into systemic disease.
View Article and Find Full Text PDFFront Pediatr
November 2024
Department of Pediatric Nephrology, Rheumatology and Immunity, The Affiliated Hospital of Qingdao University, Qingdao, China.
Castleman disease (CD) is a rare lymphoproliferative disease known as angiofollicular lymph node hyperplasia, firstly reported in 1954. It mainly occurs in adults, presenting with a wide range of clinical manifestations, including paraneoplastic pemphigus (PNP). PNP is a rare and often life-threatening autoimmune disorder characterized by painful blisters and erosions on the skin and mucous membranes.
View Article and Find Full Text PDFIndian J Dermatol Venereol Leprol
November 2024
Department of Dermatology, Venerology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
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