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1.
Pediatr Dermatol ; 39(4): 587-589, 2022 Jul.
Article in English | MEDLINE | ID: mdl-35613693

ABSTRACT

Multispecialty clinics can be exceedingly helpful for diagnostically challenging and clinically complicated patients. This study highlights the diagnostic outcomes of the multispecialty Pediatric Dermatology-Genetics clinic at Children's Mercy-Kansas City over a 5-year period.


Subject(s)
Dermatology , Ambulatory Care Facilities , Child , Humans
2.
Pediatr Dermatol ; 38(5): 1370-1371, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34272772

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.


Subject(s)
Dermatitis, Atopic , Eczema , Genetic Diseases, X-Linked , Antibodies, Monoclonal, Humanized , Dermatitis, Atopic/drug therapy , Dermatitis, Atopic/genetics , Diabetes Mellitus, Type 1/congenital , Diarrhea , Eczema/drug therapy , Eczema/genetics , Forkhead Transcription Factors/genetics , Genetic Diseases, X-Linked/drug therapy , Genetic Diseases, X-Linked/genetics , Humans , Immune System Diseases/congenital , Mutation , Pruritus/drug therapy , Pruritus/genetics
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