An Unusual Case of Congenital Linear Eccrine Spiradenoma

Abstract

Eccrine spiradenoma (ES) is a benign uncommon tumor of skin adnexa with a characteristic clinical and histopathological presentation. Typically, it presents as a painful, slow growing and solitary nodule on the head or upper trunk in adult patients. We report a child with linear ES which presented with asymptomatic papulonodular lesions in a blaschkoid distribution on the face. Cases reported in the literature of multiple spiradenomas are very rare and multiple linear lesions are even rarer. To date, 21 cases of linear/multiple ES have been reported. Of these, eight were in children or adolescents. We report an additional case of this rare clinical presentation and review the literature.

Neonatal Spiradenoma

“…Neonatal spiradenoma is rare. A research of the literature revealed only five reported cases (2,5,6). They can present at birth as solitary or multiple, asymptomatic papulonodules.…”

“…They can present at birth as solitary or multiple, asymptomatic papulonodules. Malignant transformation is uncommon and mostly occurs in tumors that have been present for many years with no age, sex, or site predilection, and more often in multiple spiradenomas (5,6).…”

Multiple linear spiradenomas and ALPK1 mutation: A case report and literature review

“…Multiple spiradenomas are assumed to originate from pluripotent stem cells in the folliculosebaceous‐apocrine unit during embryogenesis 2 . Familial cases of multiple localized spiradenomas have also been reported, suggesting that genetic predispositions such as germ cell mutations or mosaicism are present in such cases 4 …”

“…Of these, only 6 case reports developed along Blaschko's lines, as in our case (Table 1). [4][5][6][7][8][9] None of these reported cases underwent genetic testing for ALPK1 mutation.…”

“…2 Familial cases of multiple localized spiradenomas have also been reported, suggesting that genetic predispositions such as germ cell mutations or mosaicism are present in such cases. 4 On the other hand, the pathogenesis of Blaschkoid multiple spiradenomas is yet unclear. Skin appendages are formed by the cooperative action of epidermal buds and mesenchyme extending from the epidermis.…”