Paraneoplastic Pemphigus in a 34-year-old.

Related Articles

Paraneoplastic Pemphigus in a 34-year-old.

Dermatol Online J. 2017 Dec 15;23(12):

Authors: Melnick LE, Beasley JM, Kim R, Brinster N, Sicco KL

Abstract
Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Diagnosis of PNP requires direct immunofluorescence of perilesional skin and indirect immunofluorescence. Treatment of PNP is difficult and largely limited to glucocorticoids, steroid-sparing immunomodulators, rituximab and intravenous immunoglobulin (IVIG). Despite therapies, prognosis is poor. We report a case of paraneoplastic pemphigus in a 34-year old male with severe stomatitis and lichenplanus-like cutaneous lesions.

PMID: 29447659 [PubMed - indexed for MEDLINE]

https://ift.tt/2HskOeV