Congenital Self‐Healing Reticulohistiocytosis with Eye Involvement

Abstract

Congenital self-healing reticulohistiocytosis (CSHR) represents the benign end of the spectrum of Langerhans cell histiocytoses, with spontaneous resolution of lesions within the first year of life. However, involvement of organ systems other than the skin has been described occasionally and recurrence of disease at sites distant from the skin has been documented. We report a case of CSHR with eye involvement that spontaneously resolved concurrent with resolution of skin lesions. Because multiple organ systems can be involved and recurrences are possible, long-term follow-up of these patients is indicated.

Congenital Self-Healing Reticulohistiocytosis Mimicking Diffuse Neonatal Hemangiomatosis

“…CSRH is characterized by (a) congenital or neonatal skin lesions, (b) a healthy infant with no systemic involvement, (c) histopathology showing a Langerhans cell infiltrate and (d) spontaneous involution within 1–3 months or the first year of life without systemic sequelae or recurrence during a long-term follow-up [10, 11]. Occasional extracutaneous involvement or recurrence of disease at sites distant from the skin has been documented [12, 13]. …”

A Singular Case of Congenital Self-healing Histiocytosis with Skin, Liver and Atypical Eye Involvement

“…4 The peculiarity of this case is given by two elements: first, the simultaneous uveal and vitreal involvement; second, the presence of a membrane occluding the iridocorneal angle, with typical histiocytic cells, and secondary glaucoma. This finding is consistent with the reactive formation of an inflammatory pseudo-membrane around some residual histiocytes, rather than with a relapse of the primary disease.…”

Congenital self-healing reticulohistiocytosis: a case report

“…However, there are rare reports of pulmonary and ocular involvement in CSHRH that resolved along with cutaneous findings [6,7]. There have also been reports of disseminated brown or violaceous nonblanching macules and papules raising concerns of congenital infections or other hematological disorders as seen with the "blueberry muffin" lesions [8].…”