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Linear‐agminated juvenile xanthogranulomas
Author(s) -
Kiorpelidou Despoina,
Stergiopoulou Christina,
Zioga Aikaterini,
Bassukas Ioannis D.
Publication year - 2008
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4632.2008.03479.x
Subject(s) - medicine , histiocyte , lesion , pathology , physical examination , popliteal fossa , juvenile xanthogranuloma , population , hepatosplenomegaly , biopsy , anatomy , radiology , disease , environmental health
An 8‐month‐old girl presented with an asymptomatic skin lesion on the right popliteal fossa, which had been present for approximately 6 months. The child had a past medical history of a urinary tract infection at the age of 1 month and had been on daily cotrimoxazole since. There was no history of trauma to the site. Examination revealed a solitary, well‐demarcated, plaque‐like lesion on the right popliteal fossa, with multiple agminated papules in an almost linear distribution ( Fig. 1a). The lesion did not follow Blaschko's lines, but was vertical to them. The plaque was slightly indurated, measuring approximately 4 × 1.5 cm, fixed to the overlying skin but movable over the deeper tissue. The papules were yellowish in color and firm to palpation, showing a positive Darier's sign ( Fig. 1b,c). There was no regional adenopathy and no other skin lesions were observed. The physical examination and laboratory investigations were otherwise unremarkable. There was no hepatosplenomegaly, and an ocular examination and chest X‐ray were normal. 1Juvenile xanthogranuloma: agminated nodulopapular lesions on the right popliteal fossa (a) showing positive Darier's sign (b and c; arrows) A biopsy from the lesion ( Fig. 2a) revealed a dermal infiltrate of histiocytes, some of which were foamy, and admixed Touton‐type giant cells, lymphocytes, eosinophils, and mast cells. By immunohistochemistry, the predominant cell population was CD68 (KP‐1, MIB‐1, and PG‐M1, all pursued from Dako) positive, but S‐100 protein and CD1a negative ( Fig. 2b–e). By Giemsa stain, scattered mast cells (< 5% of the total cell number) were detectable within the lesion. The morphology and immunohistochemistry of the lesion were diagnostic for juvenile xanthogranuloma. Eight months later, the lesion was still present but slightly elongated, proportional to the child's growth, and hyperpigmented. 2Juvenile xanthogranuloma: histomorphology of skin lesion showing a cell‐rich histiocytic dermal infiltrate (a) with immunohistochemical characteristics (b–e) of non‐Langerhans dendritic cells (a, hematoxylin and eosin; b, anti‐S‐100 protein; c, anti‐CD‐1a; d, e, anti‐CD68 monocytic markers MIB‐1 and KP‐1, respectively; a–e, initial magnification ×40)