Baby with neonatal systemic juvenile xanthogranuloma born within a cross-cousin marriage

Abstract

a b s t r a c tJuvenile xanthogranuloma is a non-Langerhans cell histiocytosis seen most commonly in childhood and adolescence. Extracutaneous involvement is rare. We report an interesting and extremely rare case of systemic (skin, lung, spleen, and colon) "juvenile xanthogranuloma" in the neonatal period. Our case was the first ever reported case born to a cross-cousin marriage.

Neonatal systemic juvenile Xanthogranuloma with Hydrops diagnosed by Purpura skin biopsy: a case report and literature review

“…Eight cases had atypical skin lesions; one case had purpura (as in our case) [ 23 ], and the others had bluish nodules, ecchymoses, petechiae, blueberry muffin rash, and a mass [ 4 , 6 , 13 , 23 – 27 ]. Six cases had unclear in the details [ 2 , 7 , 28 – 30 , 32 ]. There were nine cases [ 5 , 11 , 12 , 20 – 22 , 25 , 27 , 28 ] in which the color and/or shape changed (excluding flattening, regression and disappearance) or new skin findings appeared over time, although there is a possibility that the change was not recognized because the patient was treated before the diagnosis.…”

“…Six cases had unclear in the details [ 2 , 7 , 28 – 30 , 32 ]. There were nine cases [ 5 , 11 , 12 , 20 – 22 , 25 , 27 , 28 ] in which the color and/or shape changed (excluding flattening, regression and disappearance) or new skin findings appeared over time, although there is a possibility that the change was not recognized because the patient was treated before the diagnosis. Change over time has been reported as a feature of congenital JXG [ 3 ].…”