Abt-letterer-siwe disease C96.0

Acute, generalized, malignant course of Langerhans cell histiocytosis in early childhood.

The incidence of LCH in childhood is 2.0-5/1,000,000 children. f:m=1.8:1.0

Unknown; familial cases with autosomal recessive inheritance have been described.

Pathogenetic: Proliferation of dendritic cells (Langerhans cells) with expression of CD1 antigen, S-100 protein and neuron-specific endase (NSE).

Integument: Eruption of disseminated small, flat, yellow-brownish papules with scaly to crusty surface. Tendency to necrotic disintegration and petechial hemorrhages, hemorrhagic-eczematoid, sometimes ulcerative skin lesions, especially capillitium, intertriginous spaces, articular folds affected; aphthous lesions of oral mucosa; thrombocytopenic purpura.

Extracutaneous manifestation: Destructive bone foci (extremities, ribs, skull, iliac blades), scarring. Obligate lymph node swelling, hepatosplenomegaly, fever, increasing anemia. Optional pulmonary involvement (cough, dyspnea, radiographic miliary mottling), osteolysis.

Leukocytosis or leukopenia, thrombopenia, hypochromic anemia.

Dense subepidermal infiltrate of large, atypical histiocytoid cells with renal nuclei as well as lymphocytes and possibly eosinophilic granulocytes with marked epidermotropism. Immunohistochemically, the cells react with S100, CD1a, and langerin-CD207.

Electron microscopy: detection of Langerhans cell granules (tennis racket-like Birbeck granules).

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