PubMed HealthSearch

PubMed · 7943635

Multiple nevoid spiradenomas.

Abstract

This report delineates a new clinical presentation of multiple benign spiradenoma (BS), a diffuse linear nevoid pattern. A 16-year-old girl, in otherwise good health, had many papulonodular lesions, varying in color from blue-gray to bright red, disposed linearly on the right half of her body, involving the periorbital region, cheek, lateral side of the neck, trunk, linea alba, right thigh, and leg. Only a few nodules were slightly painful. Lesions were reported to be present at birth; no family members had similar lesions, either in solitary or multiple form. Excisional biopsies of three lesions from the neck, abdomen, and leg, respectively, were performed; histologically, all three samples showed a picture of BS. In some areas, the tumor was directly connected to a pilosebaceous unit. Furthermore, a focal area with pilar differentiation at the periphery of the lobules was observed, which might suggest that BS can differentiate toward various cutaneous adnexal structures. Our patient probably is the first one reported with diffuse linear nevoid lesions of BS, present at birth and involving the right half of the body.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

G Noto, M R Bongiorno, G Pravatà, M Aricò. 1994. Multiple nevoid spiradenomas.. https://doi.org/10.1097/00000372-199406000-00009

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

Eccrine syringofibroadenoma: multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors.

BACKGROUND: Eccrine syringofibroadenoma (ESFA) is a rare eccrine tumor that has characteristic histopathologic features and variable clinical findings. OBJECTIVE: Our purpose was to define the clinical and histopathologic features of ESFA, to assess a possible syndromic association and heredity, and to propose a clinicopathologic classification. METHODS: Three solitary and five multiple cases of ESFA were analyzed and the findings compared with those of previously published cases. RESULTS: Clinically, solitary ESFA was typically a nonhereditary verrucous growth. Multiple examples presented as palmoplantar keratoderma and keratotic papules in a mosaic pattern. In the multiple form, four patients had the Schöpf syndrome (hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities); one had an incomplete form of the syndrome. Histologically, ESFA showed a reticulate proliferation of thin strands of acrosyringeal cells with focal lumen formation, and a fibrovascular stroma. Malignant ESFA was present in one multiple case. CONCLUSION: Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome.

Adenoma, Sweat Gland

Syringofibrocarcinoma versus squamous cell carcinoma involving syringofibroadenoma: is there a malignant counterpart of Mascaro's syringofibroadenoma?

Although a benign syringofibroadenoma has been recognized as an eccrine tumor exhibiting net-like ductal proliferation embedded in fibroma-like stroma, a malignant counterpart has not been described heretofore. An 82-year-old man with severely sun-damaged skin had a 3 cm plaque in the dorsum of his left hand. The clinical diagnosis was squamous cell carcinoma. Histologically, the lesion was a bowenoid squamous cell carcinoma, both in situ (Bowen's disease-like) and bluntly invasive, with a concurrent phase showing unequivocal and widespread ductal differentiation, local expression of carcinoembryonic antigen, and sclerotic stroma. This syringofibrocarcinoma also impinged laterally on a mature and benign syringofibroadenoma and occurred above a minute deep dermal trichoepithelioma. This report presents a probably unrecognized new malignant tumor, clearly distinct yet related to both squamous cell carcinoma and eccrine carcinoma. Because its organoid architecture and the resemblance to and impingement on syringofibroadenoma, the term syringofibrocarcinoma is proposed.

Adenoma, Sweat Gland