Familial pemphigus vulgaris in mother and daughter

International Journal of Dermatology
Z StarzyckiS Jablonska

Abstract

Pemphigus vulgaris is an autoimmune disease in which genetics appears to be of basic importance. Although association with certain human leukocyte antigen (HLA) alleles has been found in some ethnic groups and individuals, no true disease susceptibility genes have been established, and familial cases are very unusual. We report a Polish family with pemphigus vulgaris in the mother and daughter. The diagnosis was confirmed by cytologic, histologic, and immunofluorescence studies. The course was severe and the disease long-lasting in the mother, probably due to treatment with small doses of corticosteroids without immunosuppressive drugs. In the daughter, treated with larger doses of corticosteroids and azathioprine, the lesions regressed within 4 months, after which maintenance therapy was instituted with 10 mg of prednisone daily. The HLA studies performed in the daughter and her three children after the mother had died showed identical haplotypes in both the patient and the healthy children. The patient has given birth to a healthy child while still having a high titer of intercellular (IC) antibodies. The familial occurrence of pemphigus in first-degree relatives is suggestive of inherited susceptibility to the disease, trans...Continue Reading

Citations

Apr 20, 2007·International Journal of Dermatology·Evangelia Z MichailidouDemetrios Z Antoniades
May 13, 2005·Oral Diseases·M BlackC Scully
Jun 9, 2005·International Journal of Dermatology·Cheyda Chams-DavatchiZahra Naraghi
May 3, 2005·The Journal of Dermatology·Ayşe KavakSevim Gönen
Feb 1, 2006·International Journal of Dermatology·Gonca GokdemirAdem Köşlü
Mar 1, 2011·The Journal of Dermatology·Tetsuya YamamotoKeiji Iwatsuki
Nov 30, 2004·Dental Clinics of North America·Dominik A Ettlin
May 15, 2016·Human Immunology·Adriana Martinelli SalathielAna Maria Roselino
Jul 13, 2016·Case Reports in Dermatological Medicine·Ali Haydar EskiocakSoner Uzun
Oct 24, 2002·Critical Reviews in Oral Biology and Medicine : an Official Publication of the American Association of Oral Biologists·Crispian Scully, Stephen J Challacombe

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Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

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