Nevo-Xantho-Endotheuoma

Lamb, John H.; Lain, Everett S.

doi:10.1097/00007611-193706000-00006

Cited by 25 publications

(3 citation statements)

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“…Of all 22 patients with extracutaneous JXG, a solitary or multiple nodule(s) were clearly identified in the affected site 5. Table I shows 16 infants who suffered “multisystem‐visceral JXG” within 3 months of age 6–11. All but one extracutaneous case (No.…”

Section: Discussionmentioning

confidence: 99%

Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma

Hara

Ohga

Hattori

et al. 2005

Pediatric Blood & Cancer

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We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.

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Section: Discussionmentioning

confidence: 99%

Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma

Hara

Ohga

Hattori

et al. 2005

Pediatric Blood & Cancer

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show abstract

“…In this case of juvenile xanthoma there is evidence of systemic involvement, as shown by the hepatosplenomegaly. Lamb and Lain (1937) described a case of juvenile xanthoma with pulmonary infiltration and Blank et al (1949) a similar case with ocular involvement. Most writers now agree with Tannhauser (1950) that juvenile xanthoma should be grouped between the hypercholesterolkmic disseminated xanthom-atoses and the normocholesterolkmic "Histiocytosis X" of Lichtenstein (1953) (Table I).…”

Section: Figmentioning

confidence: 99%