We report a case of paraneoplastic pemphigus (PNP) as an uncommon but severe cause of cicatrising conjunctivitis. Initially diagnosed as drug eruptions, the patient's condition did not improve despite cessation of chemotherapy. Immunohistological confirmation of PNP has led to the use of combined oral prednisolone and intravenous immunoglobulin. Her ocular conditions stabilised with complete recovery of vision. PNP is a rare disease that can present with ocular involvement. Ophthalmologists should play an active role in monitoring and treatment of ocular surface complications such as symblepharon formation, severe dry eye and epithelial breakdown. Vigorous and prompt treatment is the key to successful prevention of irreversible and blinding complications. The atypical feature in this case is the presence of eosinophilic infiltration on histology that is a feature of allergic aetiologies rather than classical PNP.
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http://dx.doi.org/10.1136/bcr.12.2008.1306 | 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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