Lichenoid Dermatitis in an Adult with Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome

Young M Choi; Yen T Wang; Bob Geng; Maria Garcia-Lloret; Chandra N Smart

Lichenoid Dermatitis in an Adult with Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome

Skinmed. 2017 Jun 1;15(3):231-234. eCollection 2017.

Authors

Young M Choi¹, Yen T Wang², Bob Geng³, Maria Garcia-Lloret³, Chandra N Smart⁴

Affiliations

¹ David Geffen School of Medicine at University of California Los Angeles (UCLA), Los Angeles, CA.
² Division of Dermatology, David Geffen School of Medicine at UCLA, Los Angeles, CA; jywanga@gmail.com.
³ Division of Clinical Immunology and Allergy, UCLA, Los Angeles, CA.
⁴ Department of Pathology and Laboratory Medicine, UCLA, Los Angeles, CA.

PMID: 28705291

Abstract

A 23-year-old man presented to our practice with erythroderma and an unusual retiform eruption, along with alopecia universalis and nail dystrophy. He had had no skin findings at birth, but since early infancy had had localized eczematous eruptions of his skin. At 10 years of age, he had developed a generalized eczematous flare requiring hospitalization, and another generalized episode occurred in October 2010. He was prescribed prednisone 60 mg daily, which initially provided an improvement, but tapering of the corticosteroid resulted in another generalized flare.

Publication types

Case Reports

MeSH terms

Alopecia / etiology*
Dermatitis, Exfoliative / etiology*
Diabetes Mellitus, Type 1 / complications
Diabetes Mellitus, Type 1 / congenital*
Diabetes Mellitus, Type 1 / diagnosis
Diabetes Mellitus, Type 1 / genetics
Diarrhea / complications*
Diarrhea / diagnosis*
Diarrhea / genetics
Forkhead Transcription Factors / genetics
Genetic Diseases, X-Linked / complications*
Genetic Diseases, X-Linked / diagnosis*
Genetic Diseases, X-Linked / genetics
Humans
Immune System Diseases / complications
Immune System Diseases / congenital*
Immune System Diseases / diagnosis
Immune System Diseases / genetics
Lichenoid Eruptions / etiology*
Male
Nails, Malformed / etiology
Young Adult

Substances

FOXP3 protein, human
Forkhead Transcription Factors

Supplementary concepts

Alopecia universalis
Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome