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 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.
Jeffries
Michelle L
ML
Division of Pediatric Dermatology, Phoenix Children's Hospital, Phoenix, Arizona 85006, USA.
Aleck
Kyrieckos A
KA
Bernert
Richard A
RA
Hansen
Ronald C
RC
D000015:Abnormalities, Multiple / Q000235:genetics / Q000473:pathology; D000287:Administration, Topical; D002675:Child, Preschool; D002990:Clobetasol / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D006099:Granuloma / Q000473:pathology*; D006330:Heart Defects, Congenital / Q000235:genetics / Q000473:pathology; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D008297:Male; D008679:Metaplasia / Q000473:pathology; D000068656:Mometasone Furoate; D011244:Pregnadienediols / Q000627:therapeutic use; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics*; D012871:Skin Diseases / Q000235:genetics / Q000473:pathology*; D013545:Sweat Glands / Q000473:pathology; D013577:Syndrome; D016559:Tacrolimus / Q000031:analogs & derivatives / Q000627:therapeutic use
D003879:Dermatologic Agents; D011244:Pregnadienediols; D000068656:Mometasone Furoate; C117268:pimecrolimus; D002990:Clobetasol; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; D006854:Hydrocortisone; D016559:Tacrolimus