Letterer-Siwe disease (histiocytosis X, Langerhans cell histiocytosis) - an acute disseminated form of histiocytosis X occurring in children during the first two years of life and characterized by significant clinical diversity. ICD-10 Code: C96.0
It is twice as common in boys aged 1-3 years, with approximately 2-5 cases per 1 million children annually. Letterer-Siwe disease may be congenital, but more commonly presents between the sixth month and the second year of life with an acute disseminated multisystemic nature. It is extremely rare in adults. Many patients have mutations in the 17p13 locus (p53 gene).
Letterer-Siwe disease is the most severe form of histiocytosis X. The disease begins suddenly and progresses rapidly. The skin is affected with pruritic erythematous-squamous plaques, especially on the head and in large folds, with weeping and erosions resembling severe seborrheic dermatitis with secondary infection, vesiculation, and crusting. In severe cases, the eruptions are generalized, affecting even the palms and soles. Small papular eruptions, 1-2 mm in diameter, flesh-colored or reddish-brown in color, sometimes with a hemorrhagic component, are characteristic.
Disruption of skin barrier function and decreased resistance to infection often lead to bacterial sepsis, accompanied by fever, weight loss, and lymphadenopathy. Hepatosplenomegaly develops. Pulmonary involvement results in cough and shortness of breath, possibly progressing to pneumothorax. Bone marrow involvement results in anemia. Osteolytic lesions are seen in the bones.
- Seborrheic dermatitis
- Atopic dermatitis
- Scabies
- Candidiasis
- Darier disease
- Eczema
- Leiner's disease (erythroderma desquamativum)
- Niemaan-Pick disease
- Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker type)
- Felty syndrome