Congenital self-healing histiocytosis. Report of two cases with histochemical and ultrastructural studies.
Congenital self-healing histiocytosis (CSHH) is a rare primary skin disorder. Of the two cases in newborn infants reported here, one had numerous widespread lesions while the other had a solitary ulcerating scalp nodule. Both neonates were otherwise healthy; neither exhibited either systemic involvement or involvement of mucous membranes. The findings drawn from the skin biopsies, including histology, S-100 positivity in the majority of the cells, and the presence of Birbeck granules, were indistinguishable from those described in infantile Letterer-Siwe disease (histiocytosis X). However, the benign clinical course, with rapid regression of the nodules in both cases, was diagnostic of CSHH.
Divaris
D X
DX
Department of Pathology, Queen's University, Kingston, Ontario, Canada.
Ling
F C
FC
Prentice
R S
RS
D015614:Histiocytosis / Q000151:congenital* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008297:Male; D012075:Remission, Spontaneous; D009418:S100 Proteins / Q000032:analysis; D012867:Skin / Q000737:chemistry / Q000648:ultrastructure; D012871:Skin Diseases / Q000151:congenital* / Q000378:metabolism / Q000473:pathology
D009418:S100 Proteins