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824 SHORT COMMUNICATION Agminated Spitzoid Naevi after Remission of Langerhans Cell Histiocytosis: First Italian Case and Literature Review Roberta ROTUNNO 1 , Andrea DIOCIAIUTI 1 , Rita DE VITO 2 , Stefania GASPARI 3 , Claudia CARNEVALE 1 , Simona GIANCRISTOFORO 1 and May EL HACHEM 1 1 Dermatology Unit, 2 Department of Pathology, and 3 Department of Paediatric Haematology-Oncology, Bambino Gesù Children’s Hospital, Piazza Sant’Onofrio, 4, IT-00165 Rome, Italy. E-mail: [email protected] Accepted Feb 26, 2019; E-published Feb 27, 2019 Langerhans cell histiocytosis (LCH) is a rare childhood disease of the monocyte-macrophage system characterized by a clonal, uncontrolled proliferation and accumulation of CD1a + / CD207 + dendritic cells (DCs). LCH is stratified into single system disease (unifocal or multifocal), multi­ organ disease without organ dysfunction, and multiorgan disease with organ dysfunction. The clinical presentation ranges from isolated, self-healing skin and bone lesions, to life-threatening multi-system disease, and the sympto­ matology is extremely wide. Thus, the course, treatment and prognosis vary according to LCH type. Skin lesions represent the second most-common clinical manifestation of LCH (30–60%). The eruption may involve the scalp, intertriginous area, face, trunk and buttocks. Cutaneous presentations are polymorphic, from erythematous, yellow scaly or crusted papules, to macerated patches, pustules, vesicles, or petechiae and purpura (1, 2). with fever, hepatosplenomegaly and anaemia, and presented with diffuse papular and purpuric lesions on the trunk and inguinal and axillary folds (Fig. 1 A–C), and crusted yellow papules on the scalp. No bone marrow involvement was detected. She was treated with vinblastine (6 mg/m 2 i.v.), oral prednisone (40 mg/m 2 daily) and methotrexate (500 mg/m 2 24 h-infusion with folinic acid rescue), because of skin, liver and spleen involvement with complete regression within two years. At 5 years of age, she began to develop freckling on the neck and bilateral axillary and inguinal regions. Physical examination revealed several clustered brown macules, 2 to 4 mm in diameter, on the neck, bilateral ax­ illary and inguinal folds, and external genitalia (Fig. 1 D, E). The differential diagnosis included lentigines, naevi or postinflamma­ tory melanosis. On dermoscopy these pigmented lesions showed a reticular pattern with sporadic globules. Finally, a biopsy of two macules was performed and histologic examination revealed junctional spitzoid naevi consisting of nests of melanocytes with epithelioid morphology at the dermo–epidermal junction; immu­ nohistochemical staining for B-RAF V600E mutant protein was negative in naevus cells (Fig. S1 C, D 1 ). CASE REPORT DISCUSSION We report here the case of a girl, in remission from LCH, with subsequent appearance of agminated spitzoid naevi on the neck, and in the axillae, inguinal folds and vulva. This 6-year-old Caucasian girl, followed for LCH in remission, was referred to the Dermatology Department for the annual skin check and for evaluation of her flexure freckling. LCH had been diagnosed by skin biopsy when the child was 2 months old (Fig. S1 A, B 1 ). On the first admission, she arrived To our knowledge, a correlation between LCH and erup­ tive agminated naevi has been described in 4 other child­ ren (3–5). Our case is the first Italian patient reported in the literature. There are several similarities between the cases suggesting a potential causal and not coincidental association between LCH and eruptive naevi. LCH skin involvement was described in 4 of 5 patients; however, all patients presented pigmented lesions in the folds, especi­ ally the groin and axillae. None of the patients developed naevi on the scalp, the area mostly involved in LCH (3–5). In 3 cases the lesions were diagnosed as Spitz naevi or junctional nae­ vi with spitzoid features (3, 4); Feldstein et al. (5) reported a diagnosis consistent with agmi­ nated junctional nae­ vi. In another case no biopsy was performed due to clinical features Fig. 1. Numerous diffuse crusted of benign melanocytic or scaly papules and purpuric lesions (4). Our his­ lesions on the trunk (A), axillary folds (B) and inguinal (C). Several tological examination clustered brown macules, 2 to 4 mm revealed features of in diameter, were present on the neck junctional naevi, with (D) and bilaterally on axillary (E) and spitzoid morphology. inguinal folds (not shown). https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3156 1 doi: 10.2340/00015555-3156 Acta Derm Venereol 2019; 99: 824–825 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.