Acta Dermato-Venereologica 99-6CompleteContent | Page 20

610 SHORT COMMUNICATION Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass Takahiro KIYOHARA, Kaoru MAKIMURA, Mari MIYAMOTO, Takako SHIJIMAYA, Naoko NAGANO, Sei NAKAMARU and Hirotsugu TANIMURA Department of Dermatology, Kansai Medical University Medical Center, 10–15 Fumizono-cho, Moriguchi, Osaka 570-8507, Japan. E-mail: [email protected] Accepted Sep 18, 2018; E-published Sep 18, 2018 Progressive nodular histiocytosis (PNH) is a rare normolipaemic macrophage disorder first reported by Taunton et al. in 1978 (1). PNH belongs to a subgroup of unifying non-Langerhans cell histiocytoses (NLCHs), or the xanthogranuloma (XG) family (2). It displays a progressive clinical course with no signs of spontaneous resolution, and is characterized by the histological fin- ding of a nodular infiltrate of spindle cells in a storiform arrangement intermingled with foamy cells and Touton giant cells (3, 4). CASE REPORT An 11-year-old boy presented with multiple painless no- dules on his trunk and extremities that started at the age of 9 years as yellow to brown papular lesions. Although spontaneous regression was expected on the basis of the histopathological diagnosis of juvenile XG, new lesions continued to appear over time. At the age of 17 years, the boy had large nodules on his face, trunk, and upper and lower limbs (Fig. 1a–c), most of which were 1–2 cm in diameter, smooth-surfaced, and yellow to brown. The number of lesions was over 200. These nodules tended to be larger on the buttocks, knees, palms and soles (Fig. 1d, b c d Fig. 2. (a) Foamy cells and multinucleated Touton giant cells from a lesion on the patient at 11 years. (b) A dermal nodular infiltrate beneath a thinned epidermis composed of (c) spindle cells in a storiform arrangement, accompanied by (d) foamy cells, Touton giant cells, and foreign body giant cells in the periphery at 17 years. (Haematoxylin and eosin (HE), a: ×400, b: ×12.5, c: ×100, d: ×400). e). In particular, several nodules had coalesced, leading to a size of over 20 cm in largest diameter on the perineum to bilateral inguen (Fig. 1f). A biopsy specimen from a forearm lesion at 11 years demonstrated a dermal nodule containing foamy cells and multinucleated Touton giant cells (Fig. 2a). Histological examination at 17 years revealed a dermal nodular infiltrate of spindle cells in a a d a b e c f Fig. 1. Clinical appearance, 17-year-old boy. Smooth-surfaced, yellow to brown nodules on (a) face, (b) trunk, (c) lower limbs, (d) buttocks, and (e) left palm. A bulky mass over 20 cm in largest diameter, composed of coalesced nodules on (f) the perineum to bilateral inguen. doi: 10.2340/00015555-3045 Acta Derm Venereol 2019; 99: 610–611 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.