PMC9937778_01
Photo panel representing appearance of lesions at birth.
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Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. At the time of delivery, infant was noted to have multiple petechiae, non-blanching purple nodules, and macules ranging from 1 mm to 2 cm in diameter ( Fig. 1 ).
Platelet counts from birth to 4 months of life. T-(
n
) represents platelet transfusions and IVIG-(
n
) represents intravenous immunoglobulin.
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The infant was transfused 15 mL/kg of platelets ( Fig. 2 ) and pediatric hematology and infectious disease specialists were consulted.  Neonatal alloimmune thrombocytopenia testing was negative and platelet counts did not respond to intravenous immunoglobulin treatment on two separate occasions ( Fig. 2 ).  During the hospital stay, the lesions remained stable in size and the platelet count remained low, but stable, despite multiple platelet transfusions ( Fig. 2 ). He was followed closely by a hematologist oncologist outpatient and his thrombocytopenia gradually improved by 4 months of age ( Fig. 2 ). 
Top panel represents appearance of hand and foot lesions at 2 months of age and bottom panel represents appearance of lesions at 6 months of age.
Arrow
points to the site of the biopsy of a large 3-cm diameter lesion on the right distal thigh which revealed the diagnosis.
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 At this time, decision was made to biopsy a large 3-cm diameter lesion on the right distal thigh ( Fig. 3 ) which revealed the diagnosis of congenital disseminated JXG.  The lesions slowly started fading in color and their growth was negligible compared with the relative growth of the patient ( Fig. 3 ).
(
A
) High power view with hematoxylin and eosin (H&E) stain demonstrating dense infiltrate of mononuclear histiocytic cells, some with a vacuolated cytoplasm, with extension into subcutaneous fat (
black arrow
). (
B
) Lesion was uniformly positive for CD163 immunostain confirming its histiocytic origin. The lesion was also positive for factor XIIIa and CD68 stains (not displayed).
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Immunostaining showed diffuse expression of factor XIIIa, CD163, and CD68 stains, but the lesion was negative for CD1a stain ( Fig. 4 ).