Langerhans cell histiocytosis in a newborn

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Langerhans cell histiocytosis (LCH) is rare and results from the clonal proliferation of cells, which are similar to Langerhans cells (LC) [1].  In the neonatal period, the incidence is one to two cases per million [2].

We present the case of a male full-term newborn, in good health conditions, that upon birth presented disseminated cutaneous lesions consisting of hemorrhagic papules and vesicles on the scalp, face, trunk, scalp and extremities (Fig. 1-2). At birth, he began to show generalized skin lesions, which were initially papules, followed by central vesicles, which finally became crateriform after crust formation.

At four days old, our medical team decided to perform an incisional biopsy of the trunk lesion. Microscopically, the epidermis showed focal spongiosis with eosinophil exocytosis. In addition, loosely aggregated large cells, some of which showed infolded/vesicular nuclei, were seen in the epidermis and papillary dermis. In the upper dermis, there was mild perivascular infiltration of inflammatory cells composed of eosinophils and lymphohistiocytosis.

The immunohistochemical study was positive for Cd1a in mononuclear cells from the high dermis and S100 in the epidermis, reaching a diagnosis of LCH (Fig. 3).

The treatment was with prednisolone and vincristine for the first course. The regimen was shifted to vincristine and methotrexate (MTX) plus prednisolone (high-risk group) due to lung involvement. At 7 months old, the skin lesions regressed after the second course of chemotherapy.