A 1-month-old otherwise healthy girl was referred to our clinic for evaluation of a congenital asymptomatic lesion on the face. Upon physical exam, a firm, sharply marginated, infiltrative, yellowish plaque measuring 6×4 cms with peau d'orange surface was evident on the right mandibular region (figure 1). A 4 mm punch biopsy was performed and histological exam revealed a diffuse infiltrate of non-foamy mononuclear histiocytes with scattered eosinophils within the papillary dermis, and a scarce amount of Touton cells. Immunohistochemistry was positive for the histiocytic marker, CD68, and negative for CD1a and S-100 protein, indicating an origin of non-Langerhans cells. On this basis, we confirmed the clinical suspicion of giant congenital juvenile xanthogranuloma (JXG).

Berti S, Coronella G, Galeone M, Balestri R, Patrizi A, Neri I (2013). Giant congenital juvenile xanthogranuloma. ARCHIVES OF DISEASE IN CHILDHOOD, 98, 317-317 [10.1136/archdischild-2012-303338].

Giant congenital juvenile xanthogranuloma.

BALESTRI, RICCARDO;PATRIZI, ANNALISA;NERI, IRIA
2013

Abstract

A 1-month-old otherwise healthy girl was referred to our clinic for evaluation of a congenital asymptomatic lesion on the face. Upon physical exam, a firm, sharply marginated, infiltrative, yellowish plaque measuring 6×4 cms with peau d'orange surface was evident on the right mandibular region (figure 1). A 4 mm punch biopsy was performed and histological exam revealed a diffuse infiltrate of non-foamy mononuclear histiocytes with scattered eosinophils within the papillary dermis, and a scarce amount of Touton cells. Immunohistochemistry was positive for the histiocytic marker, CD68, and negative for CD1a and S-100 protein, indicating an origin of non-Langerhans cells. On this basis, we confirmed the clinical suspicion of giant congenital juvenile xanthogranuloma (JXG).
2013
Berti S, Coronella G, Galeone M, Balestri R, Patrizi A, Neri I (2013). Giant congenital juvenile xanthogranuloma. ARCHIVES OF DISEASE IN CHILDHOOD, 98, 317-317 [10.1136/archdischild-2012-303338].
Berti S;Coronella G;Galeone M;Balestri R;Patrizi A;Neri I
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/151653
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