Alves, Francisca

Cardoso, José

Ramos, Leonor

Relvas, Maria

English

Journal of the Portuguese Society of Dermatology and Venereology

281Qual o Seu Diagnóstico?Pápulas Castanhas Aplanadas num Menino de 4 Anos Francisca Alves1, M. Relvas1, J. Cardoso2, L. Ramos2 1Interna de Dermatovenereologia, Serviço de Dermatologia, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal2Assistente Hospitalar de Dermatovenereologia, Serviço de Dermatologia, Centro Hospitalar Universitário de Coimbra, Coimbra, PortugalPALAVRAS-CHAVE –  Criança; Face; Histiocitose de Células não Langerhans. Flat Topped Brown Papules on a Four-Year-Old MaleKEY WORDS – Child; Face; Histiocytosis, Non-Langerhans-Cell.Dermatology QuizRevista SPDV 78(3) 2020; Pápulas castanhas aplanadas num menino de 4 anos; Francisca Alves, M. Relvas, J. Cardoso, L. Ramos.CLINICAL CASE We report the case of a four-year-old male, who presen-ted with a one-year history of asymptomatic facial lesions. Weeks later, these lesions progressed a few lesions similar lesions occurred on the proximal upper and lower extremi-ties. The patient was healthy and had no associated extra-cutaneous symptoms.On physical examination, we observed several brown flat-topped papules on the face, favouring the malar and zygomatic areas bilaterally, and on the proximal upper and lower extremities (Fig.s 1 and 2). Darier sign was negative. The patient had no other cutaneous or mucosal lesions. Lesional biopsy was performed, revealing a mild infil-trate in the papillary and superficial portion of the reticular dermis, consisting predominantly of large oval cells (Fig. 3). These cells were slightly pleomorphic and presented abun-dant pale cytoplasm, with poorly defined limits. Scattered lymphocytes and occasional eosinophils were also present. The immunohistochemical study revealed a predominance of histiocytic cells in the infiltrate, with strong diffuse positi-vity for CD163. Only a few scattered cells were positive for S100 protein, CD1a, Langerin and CD117 (c-kit) (Fig. 4).Correspondência: Francisca AlvesServiço de Dermatologia - Centro Hospitalar de CoimbraPraceta Prof. Mota Pinto3000-075, Coimbra, PortugalE-mail: francisca.alves37@gmail.comDOI: https://dx.doi.org/10.29021/spdv.78.3.1176Recebido/Received2020/03/20Aceite/Accepted2020/07/21Publicado/Published2020/09/30© Autor (es) (ou seu (s) empregador (es)) 2020 Revista SPDV. Reutilização permitida de acordo com CC BY-NC. Nenhuma reutilização comercial.© Author(s) (or their employer(s)) 2020 SPDV Journal. Re-use permitted under CC BY-NC. No commercial re-use.Figure 1 - Flat-topped brown papules, located at the malar and zygomatic areas.  282Qual o Seu Diagnóstico?Revista SPDV 78(3) 2020; Pápulas castanhas aplanadas num menino de 4 anos; Francisca Alves, M. Relvas, J. Cardoso, L. Ramos.Figure 2 - The patient also had similar lesions at the proximal upper and lower extremities (A,B). A BFigure 3 - Histopathological findings. A mild infiltrate in the papillary and in the superficial portion of the reticular dermis consisting predominantly of relatively large oval cells. These cells are slightly pleomorphic and presented abundant pale cytoplasm, with poorly defined limits. Figure 4 - The immunohistochemical study revealed a predominance of histiocytic cells in the infiltrate, with strong diffuse positivity for CD163.  283Qual o Seu Diagnóstico?WHAT IS YOUR DIAGNOSIS? BENIGN CEPHALIC HISTYOCITOSISThese histopathological findings favour a non-Lan-gerhans cell histiocytosis. Taking into account the clinical presentation, namely the patient’s age and the topogra-phic distribution of the lesions, this case can be interpreted as a benign cephalic histiocytosis.Benign cephalic histiocytosis (BCH) is a rare histiocytic proliferative disorder occurring in young children, prima-rily affecting the face. It usually presents by the age of one year and is more common in males.1 Systemic involvement in the form of diabetes insipidus has been reported, but is uncommon.2 No treatment is usually needed, as it is gene-rally a self-limiting disorder. The pathogenesis of BCH is not fully understood. It is intriguing to notice that juvenile xanthogranuloma and ge-neralized eruptive histiocytoma share similar histopatholo-gy, ultrastructural appearance, and immunohistochemical profile with BCH (3). In light of these findings, several au-thors have suggested that these entities should be viewed as a single disease with a spectrum of clinical features.Regarding histopathological features, there are 3 di-fferent histologic patterns described: a papillary dermal pattern, a diffuse pattern, and a lichenoid pattern.3 Our case conforms to the papillary dermal pattern. Histiocytes are often admixed with lymphocytes and a few eosinophils and cells typically express non-Langerhans cell histiocytic markers including CD11b, CD11c, CD14b, CD68 and HAM56.4,5 The differential diagnosis includes the non-Langerhans cell histiocytosis (non-LCH) disorders, particularly erupti-ve histiocytoma and juvenile xanthogranuloma, as well as urticaria pigmentosa. Frequently, clinical differentiation is possible, as the number of the lesions as well their distribu-tion pattern mainly in the cephalic region usually allow for distinction of benign cephalic histiocytosis from other non--LCH disorders. Immunohistochemical findings are vital for excluding other entities, namely indeterminate cell histio-cytosis and Langerhans cell histiocytosis (LCH).Even though no treatment is usually needed given the benign and self-limiting nature of BCH, regular exami-nations of all patients are recommended, since periodic exacerbations of the disease and diabetes insipidus are possible.Regarding the clinical course, the patient presented with stable lesions, without progression of the disease. Given the benign nature of the disease, no additional in-vestigation was performed but he remains under observa-tion, twice annually. To this moment, he has a follow-up time of 18 months.The present case is a reminder that occasional cases of benign cephalic histiocytosis may present with extracepha-lic lesions, in addition to the cephalic lesions that tend to predominate.Conflitos de interesse: Os autores declaram a inexistência de conflitos de interesse na realização do presente trabalho. Fontes de financiamento: Não existiram fontes externas de financiamento para a realização deste artigo. Confidencialidade dos dados: Os autores declaram ter segui-do os protocolos da sua instituição acerca da publicação dos dados de doentes.Consentimento: Consentimento do doente para publicação obtido. Proveniência e revisão por pares: Não comissionado; revisão externa por pares.Conflicts of interest: The authors have no conflicts of interest to declare. Financing support: This work has not received any contribution, grant or scholarship. Confidentiality of data: The authors declare that they have followed the protocols of their work center on the publication of data from patients.Patient Consent: Consent for publication was obtained. Provenance and peer review: Not commissioned; externally peer reviewed  ORCIDJosé Carlos Cardoso https://orcid.org/0000-0001-7113-7061Maria Relvas https://orcid.org/0000-0002-2639-7494REFERENCES1. Patsatsi A, Kyriakou A, Sotiriadis D. Benign cephalic histiocytosis: case report and review of the literature. Pediatr Dermatol. 2014;31:547-50. doi: 10.1111/pde.12135.2. Weston WL, Travers SH, Mierau GW, Heasley D, Fit-zpatrick J. Benign cephalic histiocytosis with diabe-tes insipidus. Pediatr Dermatol. 2000;17:296-8. doi: 10.1046/j.1525-1470.2000.01779.x.3.	 Koca	 R,	 Bektaş	 S,	 Altinyazar	 HC,	 Sezer	 T.	 Benign	 ce-phalic histiocytosis: a case report. Ann Dermatol. 2011;23:508-11. doi: 10.5021/ad.2011.23.4.508. E 4. Samson JF, Libu GK, Philip M, Simi PS. Benign cephalic histiocytosis. Indian Dermatol Online J. 2013;4:300-1. 5. Zelger BG, Zelger B, Steiner H, Mikuz G. Solitary giant xanthogranuloma and benign cephalic histiocytosis---variants of juvenile xanthogranuloma. Br J Dermatol. 1995;133:598-604. Revista SPDV 78(3) 2020; Pápulas castanhas aplanadas num menino de 4 anos; Francisca Alves, M. Relvas, J. Cardoso, L. Ramos.

Pápulas Castanhas Aplanadas num Menino de 4 Anos

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Pápulas Castanhas Aplanadas num Menino de 4 Anos

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Journal of the Portuguese Society of Dermatology and Venereology