Congenital self-healing reticulohistiocytosis mimicking diffuse neonatal hemangiomatosis.
Congenital self-healing reticulohistiocytosis (CSRH), a rare benign variant of Langerhans cell histiocytosis (LCH), is characterized by (a). congenital skin lesions, (b). a healthy infant with no systemic involvement, (c). the histopathologic finding of a Langerhans cell infiltrate and (d). spontaneous involution within the first year of life without sequelae. We report a Taiwanese girl born with widespread hemangioma-like lesions. The diagnosis of LCH was confirmed by finding a diffuse dermal infiltrate of S-100-protein- and OKT6 (CD1a)-positive mononuclear cells and the presence of Birbeck granules in 10% of the mononuclear cells ultrastructurally. The diagnosis of CSRH was further established by rapid and complete involution of the lesions in 3 months. No recurrence was noted for 7 years. Our case illustrates that CSRH can mimic diffuse neonatal hemangiomatosis clinically; thus, it is important to include CSRH in the differential diagnosis of congenital or neonatal hemangiomatosis.
Huang
Ching-Yuang
CY
Departments of Dermatology and Pediatrics, Sin-Lau Christian Hospital, Tainan, Taiwan, ROC.
Chao
Sheau-Chiou
SC
Ho
Shae-Fang
SF
Lee
Julia Yu-Yun
JY
D001707:Biopsy, Needle; D003937:Diagnosis, Differential; D005260:Female; D006391:Hemangioma / Q000175:diagnosis / Q000473:pathology*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D011379:Prognosis; D012075:Remission, Spontaneous; D018570:Risk Assessment; D012720:Severity of Illness Index; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology*