Congenital Self-Healing Reticulohistiocytosis Presented
with Multiple Hypopigmented Flat-Topped Papules:
A Case Report and Review of Literatures
Rawipan Uaratanawong MD*,
Tanawatt Kootiratrakarn MD, PhD*, Poonnawis Sudtikoonaseth MD*,
Atjima Issara MD**, Pinnaree Kattipathanapong MD*
Affiliation :
* Institute of Dermatology, Department of Medical Services Ministry of Public Health, Bangkok, Thailand
** Department of Pediatrics, Saraburi Hospital, Sabaruri, Thailand
Congenital self-healing reticulohistiocytosis, also known as Hashimoto-Pritzker disease, is a single system
Langerhans cell histiocytosis that typically presents in healthy newborns and spontaneously regresses. In the present report,
we described a 2-month-old Thai female newborn with multiple hypopigmented flat-topped papules without any internal
organ involvement including normal blood cell count, urinary examination, liver and renal functions, bone scan, chest
X-ray, abdominal ultrasound, and bone marrow biopsy. The histopathology revealed typical findings of Langerhans cell
histiocytosis, which was confirmed by the immunohistochemical staining CD1a and S100. Our patient’s lesions had
spontaneously regressed within a few months, and no new lesion recurred after four months follow-up.
Keywords : Congenital self-healing reticulohistiocytosis, Congenital self-healing Langerhans cell histiocytosis, Langerhans
cell histiocytosis, Hashimoto-Pritzker disease, Birbeck granules
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