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The first case of paraneoplastic pemphigus positive for IgG autoantibodies against integrin α6.
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:
Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation.
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 PDFLife-threatening dermatoses.
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 PDFCase Report: A rare pediatric case of paraneoplastic pemphigus associated with Castleman disease misdiagnosed as Behçets disease.
Front 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 PDFUse of multivariant enzyme-linked immunosorbent assay (ELISA) in the diagnosis of autoimmune bullous disorders in a resource-limited setting: A single-center experience.
Indian J Dermatol Venereol Leprol
November 2024
Department of Dermatology, Venerology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Article Synopsis
- Autoimmune blistering disorders (AIBD) are conditions where the body produces auto-antibodies against adhesion proteins in the skin, detectable through direct immunofluorescence (DIF) or blood tests like ELISA.
- This study aimed to assess how well the results from a new multivariant ELISA method agreed with AIBD diagnoses established from clinical assessments, histopathology, and DIF.
- The results showed a good overall correlation between ELISA and diagnoses, particularly strong in pemphigus vulgaris, but with variable agreement levels in other types of AIBD and limitations due to the retrospective nature of the study.
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