We present a series of five cases who presented to our institution with treatment-refractory mucosal ulceration, all of whom were subsequently diagnosed with paraneoplastic pemphigus (PNP). This case series highlights the diagnostic and treatment considerations for PNP - in particular, the steroid-dependent, recalcitrant, polymorphic manifestations; the combination of histopathological and clinical findings that may overlap with clinically similar diseases, for example, pemphigus vulgaris and lichen planus; the importance of immunopathological findings for its diagnosis, and the need for surveillance and management of life-threatening bronchiolitis obliterans.
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http://dx.doi.org/10.1002/ski2.200 | DOI Listing |
Clin 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.
View Article and Find Full Text PDFAntibodies (Basel)
November 2024
Interdisciplinary Department of Medicine, University of Bari, 70124 Bari, Italy.
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