Congenital self-healing histiocytosis
In solitary self-healing reticulohistiocytosis, a red-brown painless papulonodule manifests at birth or in the neonatal period, commonly on the head, neck, or distal extremities. Mucous membrane lesions are rare. The papule may become crusted over the following weeks, with spontaneous involution over a 3- to 4-month period. Some lesions may resolve with hypopigmented, hyperpigmented, or atrophic scarring. Typically, there are no recurrences, no systemic symptoms, and no visceral involvement. Since relapses in skin lesions or at extracutaneous sites have been reported up to 4 years after resolution of the initial lesion, and because it is difficult to differentiate the condition from LCH, a skin biopsy, systemic evaluation, and close follow-up should be performed. Overall, however, the prognosis is good.
Reports of other findings in relation to solitary self-healing reticulohistiocytosis include:
- Lesion(s) urticating from physical manipulation (pseudo-Darier sign).
- Ocular involvement that resolves concurrently with cutaneous lesions.
- Multiple lung cysts that resolve within 1 year.
- Hepatosplenomegaly of unknown clinical significance.
D76.3 – Other histiocytosis syndromes
404160007 – Congenital self-healing histiocytosis
- Neonatal herpes simplex virus (HSV)
- Langerhans cell histiocytosis
- Seborrheic dermatitis– Also erythematous and scaly, but not papular or accompanied by systemic symptoms.
- Psoriasis – Also erythematous and scaly, but not papular or accompanied by systemic symptoms, and usually there is a family history of psoriasis.
- Benign cephalic histiocytosis – Occurs between 2 months and 2 years and is on the face and other portions of the head. It is caused by a non-Langerhans cell histiocytosis. Lesions regress spontaneously.