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Bilateral Facial Apocrine Fibrosing Hamartoma Mimicking Microcystic Adnexal Carcinoma

Llamas-Velasco, Mar MD*; Fraga, Javier PhD; Schaller, Jörg MD; Requena, Luis MD§; Daudén, Esteban MD*; Rütten, Arno MD

The American Journal of Dermatopathology: October 2019 - Volume 41 - Issue 10 - p 767–770
doi: 10.1097/DAD.0000000000001433
Extraordinary Case Report
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Abstract: An otherwise healthy 50-year-old woman was evaluated for the presence of 2 erythematous, and slightly pruritic plaques, involving both cheeks for 30 years. Left-side skin biopsy showed a diffuse proliferation of ductal structures horizontally arranged and involving the reticular dermis that resembled tubular adenoma embedded in a sclerotic stroma and surrounded by a peculiar periductal desmoplasia. Nuclear atypia or mitosis was not found. Contralateral biopsy showed identical findings. Differential diagnosis included microcystic adnexal carcinoma (MAC) and plaque-like syringoma and a peculiarly horizontally arranged tubular adenoma. We ruled out MAC as the lesions were long-standing, without infundibular cysts, solid strands, or perineural infiltration. Our case closely resembled those previously described as sweat duct proliferation associated with aggregates of elastic tissue and atrophoderma vermiculatum, although striking differences were observed, as our case did not present aggregates of elastic tissue, did not involve the papillary and superficial reticular dermis, and presented evidences of decapitation secretion as a sign of apocrine differentiation. We consider our case as a MAC simulator and we propose the descriptive name of bilateral facial apocrine fibrosing hamartoma.

*Department of Dermatology, Hospital Universitario de La Princesa, Madrid, Spain;

Department of Pathology, Hospital Universitario de La Princesa, Madrid, Spain;

Department of Dermatology, Helios Clinic, Duisburg, Germany;

§Dermatology Department, Fundación Jimenez Diaz, Madrid, Spain; and

Dermatopathology Department, Dermatopathologie Friedrischafen/Bodensee, Friedrischafen, Germany.

Correspondence: Mar Llamas-Velasco, MD, C/Diego de León 62, CP 28006, Madrid, Spain (e-mail: mar.llamasvelasco@gmail.com).

M. Llamas-Velasco has colaborated as lecturer and in clinical assays with the following drug companies: Abbvie, Almirall, Celgene, Gebro, Leo-Pharma, Lilly, Janssen, Novartis, UCB, and Reig-Jofre. E. Dauden has colaborated has colaborated as lecturer, clinical advisor and/or in clinical assays with the following drug companies: Abbvie, Amgen, Almirall, Celgene, Gebro, Leo-Pharma, Lilly, Janssen, Novartis, and UCB. The remaining authors have no conflicts of interest.

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