Congenital self-healing reticulohistiocytosis with spontaneous regression

Abstract

Congenital self-healing reticulohistiocytosis is a rare, benign, self-limiting variant of Langerhans cell histiocytosis (LCH). LCH encompasses a group of idiopathic disorders characterized by the clonal proliferation of Langerhans cells. Congenital self-healing reticulohistiocytosis typically appears at birth or in the neonatal period as isolated cutaneous lesions, often appearing as multiple crusted papules with no systemic findings. Although clinical features seem aggressive, the lesions tend to involute spontaneously within weeks to a few months leaving residual hypo or hyperpigmented macules. Timely diagnosis with histology, immunocytochemistry, and electron microscopic studies will eliminate unnecessary therapeutic interventions. Although mostly self-resolving, it carries a variable clinical course in some patients with cases of extracutaneous involvement and/or recurrences. Hence, reassurance and long-term follow-up play key roles in the management of this disease.

Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature

“…In 1 case report, hypopigmented lesions were seen during an episode of recurrence of congenital self‐healing reticulohistiocytosis . They may also coexist with other types of LCH lesions, as reported by Parimi and colleagues and seen in our second case . Histopathology of all reported cases was consistent with LCH, with the Langerhans cell infiltrate expressing CD1a, S‐100, or CD207.…”

“…They have been described after resolution of active lesions in congenital self‐healing reticulohistiocytosis . Few case reports have described hypopigmented macules as an initial presentation of the disease . We report 2 cases of the hypopigmented variant of LCH and review the literature on this rare clinical presentation.…”

A congenital ulcerated nodule with spontaneous resolution

“…LCH is a heterogeneous group of clonal proliferation diseases of Langerhans cells derived from myeloid progenitor cells from the bone marrow (1,2). Formal diagnosis requires a skin biopsy for histologic/immunohistochemical examination.…”

“…Watchful waiting is recommended for patients with skin-only LCH (5,8,11). Typically, the papules/nodules resolve spontaneously within a few weeks to months, occasionally, leaving residual hyper-or hypopigmented macules or scars (1,2,6,8,10). Ulceration or necrosis on the surface of the nodule is an indicator of good prognosis (7).…”

A rare case of multisystemic Langerhans cell histiocytosis mimicking diffuse neonatal hemangiomatosis

“…In the last two decades, DNH-like eruptions have been described in CSHR [4][5][6] ; however, the condition is rarely reported in multisystemic LCH and demonstrated diffuse hemangiomas as a skin manifestation. [7][8][9] In conclusion, we present herein the fourth case of diffuse hemangioma-like lesions in a patient with multisystemic involvement of LCH.…”