Paraneoplastic pemphigus (PNP) has been described as an antibody-mediated mucocutaneous disease occurring almost exclusively in patients with lymphocytic neoplasms. We describe 4 patients with the clinical features of the lichenoid variant of PNP in the absence of detectable autoantibodies. On the basis of these findings, we conclude that the spectrum of PNP likely includes patients with disease predominantly or exclusively mediated by cytotoxic T cells rather than autoantibodies. The pathophysiology and range of PNP disease are likely more complex than was initially believed.
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http://dx.doi.org/10.1016/j.jaad.2006.06.007 | DOI Listing |
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.
BMJ Case Rep
December 2024
General Surgery, PGIMER, Chandigarh, India.
Castleman disease is a benign lymph node hyperplasia with two histological subtypes. In the case reported here, a young adult male presented with mucocutaneous lesions mimicking common dermatological conditions. Suspicion of an underlying systemic disease arose when the routine clinical care of the skin lesions failed to cure them.
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