2017
DOI: 10.1111/pde.13073
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A Case with Juvenile Xanthogranuloma and Progressive Nodular Histiocytosis Overlap

Abstract: A patient with overlap between juvenile xanthogranuloma and progressive nodular histiocytosis is presented. He had progressive, superficial, small, yellow-reddish papules and large dermal nodules, consistent with progressive nodular histiocytosis. Histopathology showed distinctive features of juvenile xanthogranuloma. Our patient reinforces the view that progressive nodular histiocytosis is not a distinct clinical entity, but an evolutionary form of juvenile xanthogranuloma.

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Cited by 6 publications
(8 citation statements)
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“…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 finding of a nodular infiltrate of spindle cells in a storiform arrangement intermingled with foamy cells and Touton giant cells (3,4).…”
mentioning
confidence: 99%
“…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 finding of a nodular infiltrate of spindle cells in a storiform arrangement intermingled with foamy cells and Touton giant cells (3,4).…”
mentioning
confidence: 99%
“…Lesions with overlapping features have been described. Entities with JXG and PNH overlap are on record [9,35], as are lesions with clinicopathologic features that bridge PNH and multiple AXG [12]. There have also been well-documented cases of transformation of one entity into another, e.g., BCH evolving to JXG [36] and GEH morphing into XD, JXG, or PNH [37].…”
Section: Discussionmentioning
confidence: 99%
“…Corticosteroid treatment as intralesional injection, pulse therapy, and continuous daily treatment (10-70mg/day oral prednisone) has been reported [5][6][7]. Electrodesiccation and curettage of disfiguring lesions has been reported in a pediatric patient with satisfactory cosmetic results [2]. Systemic chemotherapeutic agents like vincristine, cyclophosphamide, and prednisolone have been tried without success [4,13].…”
Section: Case Discussionmentioning
confidence: 99%
“…Progressive nodular histiocytosis (PNH) is an infrequent form of non-Langerhans cell histiocytosis (LCH) of the xanthogranuloma group, which includes juvenile xanthogranuloma, xanthoma disseminatum, benign cephalic histiocytosis, multiple adult xanthogranuloma, and generalized eruptive histiocytosis. It was first described in 1978 by Taunton [1][2][3]. Currently, fewer than 20 cases can be found on a PubMed search of reports indexed for MEDLINE.…”
Section: Introductionmentioning
confidence: 99%