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Letterer-Siwe disease

This is now also known as Langerhans cell Histiocytosis

The classical skin lesions of Letterer-Siwe disease are fine, erythematous papules surmounted by a scale. These small papules are frequently on the scalp, but can be generalized. On cursory examination, the diagnosis of seborrheic dermatitis is suggested, but unlike seborrheic dermatitis, the eruption of histiocytosis X is papular and can be hemorrhagic. The red-brown papules tend to become confluent, forming a greasy scale. Frequently there are erosions, particularly in the flexures. Frank ulcers may occur behind the ears and in the groin. While nearly all patients with Letterer-Siwe disease have these papular skin lesions, only 30% to 50% of those with Hand-Schüller-Christian disease have skin lesions. Skin lesions of eosinophilic granuloma are uncommon, but when they occur, they are usually identical to those seen in Letterer-Siwe disease. Larger papules and nodules may also occur. Xanthomas of the skin can be a manifestation of histiocytosis X.

Treatment

  • Systemic therapy with oncology agents is required.
Histiocytosis


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