Familial multiple eccrine spiradenomas with cylindromatous features associated with epithelioma adenoides cysticum of Brooke.
Four cases of rare familial multiple eccrine spiradenomas showing features of dermal cylindromas and associated with epithelioma adenoides cysticum of Brooke are reported. Skin biopsy specimens were obtained from three generations of this family and routine histochemical and immunoperoxidase stains were used. The eldest affected family member had multiple disfiguring facial and scalp tumors, which precipitated episodes of depression. Unlike other cutaneous genetic disorders, such as neurofibromatosis and tuberous sclerosis, the cutaneous adnexal tumors occurring in these patients continue to erupt and grow during their lifetimes.
Berberian
B J
BJ
Division of Dermatology, Georgetown University Medical Center, Washington, DC 20007.
Sulica
V I
VI
Kao
G F
GF
D000236:Adenoma / Q000235:genetics* / Q000473:pathology; D006607:Adenoma, Sweat Gland / Q000235:genetics* / Q000473:pathology; D000293:Adolescent; D000328:Adult; D003528:Carcinoma, Adenoid Cystic / Q000235:genetics* / Q000473:pathology; D005153:Facial Neoplasms / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics*; D010375:Pedigree; D012535:Scalp / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology