Generalized eczematous dermatitis and pruritus responsive to dupilumab in a patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.

Creator(s)

Maeve C. Maher, Children's Mercy Hospital
Erin Hall, Children's Mercy HospitalFollow
Kimberly A. Horii, Children's Mercy HospitalFollow

Document Type

Article

Publication Date

9-2021

Identifier

DOI: 10.1111/pde.14717

Abstract

This case describes a patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome with diffuse eczematous dermatitis and severe, intractable pruritus. Despite a bone marrow transplant and immunosuppressive therapy, his skin findings and pruritus persisted. Off-label dupilumab provided significant improvement and almost complete clearance of the dermatitis and pruritus. This is the first known report of dupilumab being used in a patient with IPEX syndrome.

Journal Title

Pediatric dermatology

Volume

38

Issue

5

First Page

1370

Last Page

1371

MeSH Keywords

Antibodies, Monoclonal, Humanized; Dermatitis, Atopic; Diabetes Mellitus, Type 1; Diarrhea; Eczema; Forkhead Transcription Factors; Genetic Diseases, X-Linked; Humans; Immune System Diseases; Mutation; Pruritus

Keywords

IPEX; dupilumab; eczema; eczematous dermatitis

Recommended Citation

Maher MC, Hall EM, Horii KA. Generalized eczematous dermatitis and pruritus responsive to dupilumab in a patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Pediatr Dermatol. 2021;38(5):1370-1371. doi:10.1111/pde.14717