An ‘eruptive’ variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis

This chapter summarizes the clinical spectrum of the histiocytic disorders—Langerhans cell histiocytosis (LCH), haemophagocytic lymphohistiocytosis (HLH), and some uncommon histiocytic disorders, including juvenile xanthogranuloma (JXG) and Rosai–Dorfman disease—as well as the current diagnostic and therapeutic approaches in these diseases. Multiple activating mutations in the RAS–RAF–MEK–ERK pathway have recently been described in LCH. Their role in the pathophysiology of the disorder and in targeted therapy is reviewed. This chapter explains the differences between primary and secondary HLH, and reviews the genetic abnormalities playing a role in both forms of HLH.

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Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases

Clinical and Experimental Dermatology ◽

10.1046/j.1365-2230.2001.00842.x ◽

2001 ◽

Vol 26 (5) ◽

pp. 391-394 ◽

Cited By ~ 36

Author(s):

P. H. Hoeger ◽

C. Diaz ◽

M. Malone ◽

J. Pritchard ◽

J. I. Harper

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma

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Fine-needle cytology of cutaneous juvenile xanthogranuloma and langerhans cell histiocytosis

Cancer Cytopathology ◽

10.1002/cncy.20132 ◽

2010 ◽

Vol 119 (2) ◽

pp. 134-140 ◽

Cited By ~ 7

Author(s):

Ambrogio Fassina ◽

Andrea Olivotto ◽

Rocco Cappellesso ◽

Roberto Vendraminelli ◽

Matteo Fassan

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma ◽

Fine Needle ◽

Fine Needle Cytology

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LANGERHANS' CELL HISTIOCYTOSIS AND JUVENILE XANTHOGRANULOMA OF THE ORBIT

Radiologic Clinics of North America ◽

10.1016/s0033-8389(05)70242-6 ◽

1998 ◽

Vol 36 (6) ◽

pp. 1229-1240 ◽

Cited By ~ 24

Author(s):

Ahmed A. Hidayat ◽

Mahmood F. Mafee ◽

Nora V. Laver ◽

Samir Noujaim

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma

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Langerhans Cell Histiocytosis and Juvenile Xanthogranuloma

Dermatology ◽

10.1159/000078589 ◽

2004 ◽

Vol 209 (1) ◽

pp. 57-61 ◽

Cited By ~ 32

Author(s):

Annalisa Patrizi ◽

Iria Neri ◽

Federica Bianchi ◽

Valentina Guerrini ◽

Cosimo Misciali ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Juvenile Xanthogranuloma

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Langerhans Cell Histiocytosis Preceding the Development of Juvenile Xanthogranuloma: A Case and Review of Recent Developments

Pediatric and Developmental Pathology ◽

10.2350/10-12-0950-cr.1 ◽

2011 ◽

Vol 14 (6) ◽

pp. 480-484 ◽

Cited By ~ 20

Author(s):

Ashish Bains ◽

David M. Parham

Keyword(s):

Stem Cell ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Factor Xiiia ◽

Juvenile Xanthogranuloma ◽

Cell Precursor ◽

Hematopoietic Stem ◽

Birbeck Granules ◽

Cells Of Origin ◽

Recent Developments

Langerhans cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) both belong to the histiocytosis group of disorders, which have varied prognostic and clinical significance. Their normal cellular counterparts share a common CD34-positive hematopoietic stem cell precursor that matures along CD14-negative or -positive pathways. Rare cases of LCH and JXG show overlapping findings, suggesting that this divergent maturation is not irreversible. We report a case of an infant diagnosed with cutaneous LCH shortly after birth. Two years after diagnosis, a recurrent lesion in his external auditory canal contained lipidized cells with CD68 and factor XIIIa expression and lacked Birbeck granules and CD1a, consistent with JXG. Our case and previous reports of this phenomenon raise the question of a histogenic relationship between LCH and JXG, and we speculate that the lesional cells of origin are subjected to therapy-induced modulation that results in a varied differentiation.

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