CONGENITAL SELF-HEALING RETICULOHISTIOCYTOSIS

By Gary M. White, MD

Congenital self-healing reticulohistiocytosis


Congenital self-healing reticulohistiocytosis (CSHR), aka congenital self-healing Langerhans cell histiocytosis, is a rare, congenital, benign, self-healing variant of Langerhans cell histiocytosis (LCH).

Clinical

The newborn presents with a solitary or multiple asymptomatic red-brown papulonodules which resolve spontaneously within a few weeks to months. The lesions may be present at birth or arise typically in the first 2 months of life. Later onset (e.g., 6 months) has been reported. Occasionally, pseudo-Darier sign has been reported (urtication after physical manipulation).

Diagnosis and Workup

Diagnosis of Langerhans cell process is established by clinical exam and skin biopsy. There is no way at this juncture to distinguish CSHR from the disseminated forms of LCH. Thus, a thorough workup and evaluation for systemic disease to exclude systemic/disseminated LCH is in order. This may include baseline laboratory and radiographic evaluation, thorough history and physical examination, a complete blood count with differential, liver function tests, coagulation studies, a skeletal survey, and chest radiography should be completed. Further studies, such as urine osmolality (to evaluate for diabetes insipidus) or chest CT can be done if indicated by these studies or symptoms.

Treatment

No treatment is needed. Lesions usually resolve within 2-4 months of onset, often leaving hypopigmented or hyperpigmented macules. Close followup for expectant resolution should be done. Even after resolution, the child needs further monitoring as recurrence and even death has rarely occurred [Int J Dermatol. 2003;42:245–246]. In another report, diabetes insipidus occurred two years after the skin findings resolved [J Am Acad Dermatol. 1985;13:481].

References

Photos of lesions initially and two months later. Ann Dermatol. 2011 Sep; 23(Suppl 1): S4–S7

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