Congenital self-healing reticulohistiocytosis
Synopsis

While the vast majority of patients have disease limited to the skin, rare pulmonary or ocular involvement has been described.
CSHR is usually a benign, self-limited disease that does not require any specific treatment, as the skin and pulmonary / eye lesions spontaneously involute; however, relapses in skin lesions or lesions at extracutaneous sites (lung or eye) have been reported up to 4 years after resolution of the initial lesions. Because it is difficult to differentiate this condition from classic LCH, a skin biopsy, systemic evaluation (laboratory testing / imaging), and close follow-up should be performed. Overall, the prognosis is good.
Codes
C96.6 – Unifocal Langerhans-cell histiocytosis
SNOMEDCT:
404160007 – Congenital self-healing histiocytosis
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Last Updated:02/28/2024