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Necrobiosis lipoidica (NL) is a chronic granulomatous idiopathic disorder. It usually presents as yellow-brown, atrophic, telangiectatic plaques with an elevated violaceous rim, typically in the pretibial region of both extremities with and without ulceration [1, 2]. There are few reported cases of childhood-onset NL in atypical locations. We report a case of type 1 diabetic 13-year-old girl who developed yellow atrophic plaque with telangiectasia on the extensor aspect of her upper arm.

<b><i>Introduction:</i></b> Trichilemmomas are a form of benign adnexal neoplasm with differentiation toward the follicular outer sheath. Nevus sebaceous (NS) of Jadassohn is a congenital malformation characterized as a nonhereditary hamartoma of the adnexal structures of the skin, mainly the pilosebaceous unit. NS represents a fertile field for the development of secondary adnexal neoplasms, commonly benign but occasionally malignant. To our knowledge, one case of a pigmented desmoplastic trichilemmoma (DT) has been reported in the English literature. <b><i>Case Presentation:</i></b> We report a case of a 36-year-old male with pigmented DT that developed on long-standing congenital NS, mimicking pigmented basal cell carcinoma (BCC), which was successfully excised with 4-mm margins. <b><i>Conclusion:</i></b> Pigmented DT may mimic pigmented BCC on clinical and dermatoscopic examination; therefore, histological evaluation is always recommended to confirm the diagnosis.

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