ABSTRACT
BACKGROUND: Cellular neurothekeoma is a relatively rare, benign cutaneous neoplasm, which usually presents as a solitary papule or nodule involving the head and neck area of young adults. Multiple neurothekeomas have not, to date, been known to occur. METHODS: We report a 30-year-old, otherwise healthy, male who presented with multiple neurothekeomas (15) in the head and neck area over a period of 12 years. RESULTS: While the unifying feature of all biopsied (10 of 15) lesions was the presence of epithelioid cells--the lesions differed in their cellularity and the degree of sclerosis of the stromal component. Antigenic profiling of the lesional cells revealed expression of vimentin, NKI/C3, PGP 9.5, factor XIIIa and CD68 but not S100, HMB45, MelanA, EMA, MSA, desmin, CD57 or NGF-R. CONCLUSIONS: This case report is the first to document the occurrence of multiple cellular neurothekeomas. An unusual histologic feature of some of the biopsied lesions was the presence of a markedly sclerotic stroma.
Subject(s)
Immunohistochemistry/methods , Neoplasms, Multiple Primary/pathology , Neurothekeoma/pathology , Skin Neoplasms/pathology , Adult , Biomarkers, Tumor/analysis , Biopsy , Humans , Male , Neoplasm Recurrence, Local , Neoplasms, Multiple Primary/chemistry , Neoplasms, Multiple Primary/surgery , Neurothekeoma/chemistry , Neurothekeoma/surgery , Skin Neoplasms/chemistry , Skin Neoplasms/surgeryABSTRACT
D-Penicillamine, a heavy metal chelator used in the treatment of Wilson's disease and other conditions, may be associated with both noncutaneous and cutaneous side effects. Some of the cutaneous lesions are due to a toxic-metabolic effect on connective tissue; some may be explained on the basis of autoimmunity; some are acute sensitivity reactions, and some are secondary to unknown mechanisms. The types of cutaneous manifestations may, in some instances, be correlated with the disease being treated and the dosage and duration of penicillamine therapy.
