Eccrine Squamous Metaplasia and Periadnexal Granulomas: New Cutaneous Histopathologic Findings in Cardiofaciocutaneous Syndrome

:  Cardiofaciocutaneous syndrome is a rare genetic disorder characterized by dysmorphic facial features and neurologic, cardiac, ophthalmologic, and dermatologic findings. Previously reported skin and hair findings in cardiofaciocutaneous syndrome include sparse, slow‐growing curly hair, atopic derm...

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Bibliographic Details
Published in:Pediatric dermatology 2010-05, Vol.27 (3), p.274-278
Main Authors: Jeffries, Michelle L., Aleck, Kyrieckos A., Bernert, Richard A., Hansen, Ronald C.
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - genetics
Abnormalities, Multiple - pathology
Administration, Topical
Biological and medical sciences
Child, Preschool
Clobetasol - therapeutic use
Dermatologic Agents - therapeutic use
Dermatology
Granuloma - pathology
Heart Defects, Congenital - genetics
Heart Defects, Congenital - pathology
Humans
Hydrocortisone - therapeutic use
Male
Medical sciences
Metaplasia - pathology
Mometasone Furoate
Pregnadienediols - therapeutic use
Proto-Oncogene Proteins B-raf - genetics
Skin Diseases - genetics
Skin Diseases - pathology
Sweat Glands - pathology
Syndrome
Tacrolimus - analogs & derivatives
Tacrolimus - therapeutic use
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Summary::  Cardiofaciocutaneous syndrome is a rare genetic disorder characterized by dysmorphic facial features and neurologic, cardiac, ophthalmologic, and dermatologic findings. Previously reported skin and hair findings in cardiofaciocutaneous syndrome include sparse, slow‐growing curly hair, atopic dermatitis, ichthyosis, follicular hyperkeratosis, and keratosis pilaris. We report the case of a 4‐year‐old boy who has cardiofaciocutaneous syndrome with previously unreported histopathologic findings of eccrine squamous metaplasia and periadnexal granuloma.
ISSN:0736-8046
1525-1470
DOI:10.1111/j.1525-1470.2009.00982.x