当前位置： SCI文献检索 > JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY期刊下所有文献 > Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) associated with pemphigoid nodularis: a case report and review of the literature.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) associated with pemphigoid nodularis: a case report and review of the literature.

Abstract：

:The immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) is a rare disorder caused by mutations of the FOXP3 gene. The FOXP3 gene encodes a DNA-binding protein of the forkhead/winged-helix family and is the central controller of the development of CD4+CD25+ regulatory T cells. CD4+CD25+ regulatory T cells help prevent autoimmune disease; a deficiency of these cells causes increased immunologic reactivity and autoimmunity. We describe a 14-year-old boy with IPEX syndrome confirmed by mutation analysis of the FOXP3 gene. The patient had chronic dermatitis and later developed bullous pemphigoid. He subsequently formed diffuse prurigo nodularis-like lesions resistant to multiple topical and systemic immunosuppressive medications. These lesions were confirmed by biopsy, direct immunofluorescence, and enzyme-linked immunosorbent assay of the 180 kd bullous pemphigoid antigen to be pemphigoid nodularis. He recently responded to rituximab, allowing discontinuation of his oral prednisone.

journal_name

J Am Acad Dermatol

journal_title

Journal of the American Academy of Dermatology

authors

McGinness JL,Bivens MM,Greer KE,Patterson JW,Saulsbury FT

doi

10.1016/j.jaad.2005.08.047

subject

Has Abstract

pub_date

2006-07-01 00:00:00

pages

143-8

issue

1

eissn

0190-9622

issn

1097-6787

pii

S0190-9622(05)02700-3

journal_volume

55

pub_type

杂志文章,评审

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