Abstract
Congenital self-healing reticulohistiocytosis (CSHRH) is a benign type of Langerhans cell histiocytosis (LCH) also known as Hashimoto-Pritzker disease. Clinically it presents with skin lesions at birth or in neonatal period, usually without any systemic involvement. Lesions often heal spontaneously in period of weeks to months. We report a case of CSHRH presenting with skin lesions at birth, describing need to make an early diagnosis and to have a multidisciplinary approach with regular follow-up, in managing this rare type of LCH.
Highlights
Langerhans cell histiocytosis (LCH) is a clonal proliferative disease of Langerhans cells with broad spectrum of forms
Congenital self-healing reticulohistiocytosis (CSHRH) is a rare benign variant of LCH first described by Hashimoto and Pritzker [3]
A diagnosis of LCH is typically made with a skin biopsy that shows positive immunohistochemical staining of infiltrative cells in the dermis with CD1a and S-100
Summary
Langerhans cell histiocytosis (LCH) is a clonal proliferative disease of Langerhans cells with broad spectrum of forms. Congenital self-healing reticulohistiocytosis (CSHRH) is a rare benign variant of LCH first described by Hashimoto and Pritzker [3]. Multiple lesions are most common but the presence of a solitary skin lesion has been reported. Case Report An 8-month-old female infant born full-term via uncomplicated vaginal delivery with normal birth weight, length and APGAR score, presented with reddish brown papules all over the body, progressively developing since birth.
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