Case report Hereditary multiple fibrofolliculomas, trichodiscomas and acrochordons: syndrome of Birt-Hogg-Dube ` Massimiliano Scalvenzi, Giuseppe Argenziano, Elena Sammarco, Mario Delfino* Department of Dermatology, University of Naples ‘Federico II’, Via Pansini 5, 80131 Naples, Italy Abstract The syndrome of Birt-Hogg-Dube ` (BHD) is an autosomal dominant syndrome, characterized by a triad of cutaneous lesions including multiple fibrofolliculomas, trichodiscomas, and acrochordons. There are many clinical expressions, solitary and multiple forms, with or without other skin tumors. In the literature BHD syndrome has been associated with internal malignancy. We describe a patient with multiple firm, skin-colored papules in which the three types of lesion are documented. No signs of systemic disease were present.  1998 Elsevier Science B.V. All rights reserved Keywords: Fibrofolliculoma; Trichodiscoma; Acrochordon; Birt-Hogg-Dube ` syndrome 1. Introduction Multiple fibrofolliculomas (FF), trichodiscomas (TD) and acrochordons (AC) are associated with the Birt-Hogg-Dube ` (BHD) syndrome, a rare condition described in 1977 [1]. BHD syndrome is clinically characterized by an asymptomatic eruption of dome shaped papules in- volving the head, neck, chest, back and arms fre- quently associated with acrochordons; and histologi- cally, by multiple follicular tumors, namely fibrofolliculomas and trichodiscomas, representing benign proliferations of mesodermal and ectodermal components of the pilar apparatus; the syndrome appears to have an autosomal dominant mode of inheritance [2]. 2. Case report A 52-year-old male presented several asymp- tomatic dome-shaped papules on the head, neck and back which had first appeared 25 years earlier (Figs. 1 and 2). In addition, numerous pedunculated tags were present in the axillae. The patient’s mother, a sister and a brother have clinically identical lesions. No signs of systemic disease were clinically appre- ciated. Histopathologic examination of two dome-shaped lesions showed a distorted hair follicle in the cen- ter surrounded by a thick mantle of basophilic, mucoid stroma. Numerous thin, anastomosing bands Journal of the European Academy of Dermatology and Venereology 11 (1998) 45–47 0926-9959/98/$19.00  1998 Elsevier Science B.V. All rights reserved PII S0926-9959(98)00018-X * Corresponding author. Tel.: +39 81 5466062; fax: +39 81 7462442.