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...

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Bibliographic Details
Published in:	Cutis (New York, N.Y.) Vol. 46; no. 1; p. 46
Main Authors:	Berberian, B J, Sulica, V I, Kao, G F
Format:	Journal Article
Language:	English
Published:	United States 01-07-1990
Subjects:	Adenoma - genetics Adenoma - pathology Adenoma, Sweat Gland - genetics Adenoma, Sweat Gland - pathology Adolescent Adult Carcinoma, Adenoid Cystic - genetics Carcinoma, Adenoid Cystic - pathology Facial Neoplasms - genetics Facial Neoplasms - pathology Female Humans Male Middle Aged Neoplasms, Multiple Primary - genetics Pedigree Scalp - pathology Skin Neoplasms - genetics Skin Neoplasms - pathology
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Summary:	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.
ISSN:	0011-4162