Familial Eccrine Spiradenoma: A Case Report and Review of the Literature

Abstract

Familial eccrine spiradenoma is a benign autosomal dominantly inherited condition that is characterized by tender, slow-growing, adnexal tumors of the head and neck. Surgical tumor extirpation and CO2 laser ablation offer both an effective symptomatic and cosmetically elegant treatment option.

A solitary fast growing red nodule of the abdomen

“…It classically occurs in adulthood between the second and the fourth decade of life. Since the original description of spiradenoma, many different variants were reported in literature, including multiple, giant, linear, blaschkoid, and malignant types . Spiradenomas may occur jointly with cylindroma, and multiple lesions should prompt consideration of a diagnosis of Brooke‐Spiegler syndrome.…”

De Novo Malignant Eccrine Spiradenoma: A Case Report and Review of the Literature

“…[3][4][5][6] In one case report of familial ES, a rare autosomal dominant disorder characterized by multiple slow-growing spiradenomas, the combination of staged surgical resection of larger lesions, and CO 2 laser ablation of smaller lesions offered symptomatic relief with a superior cosmetic result. 7 Another inherited syndrome of multiple spiradenomas with concomitant adnexal tumors such as trichoepitheliomas and dermal cylindromas suggests the diagnosis of Brooke-Spiegler syndrome, also known as familial cylindromatosis or turban tumor syndrome. 8 Autosomal dominantly inherited, Brooke-Spiegler syndrome has been mapped to 16q12-13 and associated with mutations in the CYLD gene, for which both a In 1972, Dabska 11 first described the malignant transformation of long-standing ES, most often to carcinoma though rarely to sarcoma.…”

Multiple eccrine spiradenomas in a linear or zosteriform distribution involving the right side of the body

“…4 A familial occurrence of multiple eccrine spiradenomas has been reported, and autosomal dominant transmission has been suggested. 5,6 This case is reported because of the extremely rare presentation. The patient had late-onset eccrine spiradenomas with no family history.…”