The dermatologic disturbances associated with the group of conditions which includes eosinophilic granuloma,* Hand-Schüller-Christian † and Letterer-Siwe ‡ disease have been reviewed thoroughly elsewhere.§
The cutaneous findings in acute Letterer-Siwe disease are generally of a petechial, ecchymotic, or purpuric nature. Eruptions resembling seborrheic dermatitis have been noted, and even a necrotic, ulcerating angina of the oral mucosa has been reported in one case. The histologic picture is essentially the same regardless of the system involved, that is, skin, viscera, or bone.
Approximately one-third of the patients with Hand-Schüller-Christian disease have cutaneous lesions. These consist of papulopustular eruptions, seborrheic and purpuric lesions, bronze discoloration, and occasionally lipoid infiltration about the eyes and xanthomas, especially of the disseminate variety.
Cutaneous alterations are rarely seen in association with eosinophilic granuloma of bone. However, granulomatous lesions with the same histologic structure as eosinophilic lesions of bone have been noted
KIERLAND RB, EPSTEIN JG, WEBER WE. Eosinophilic Granuloma of Skin and Mucous Membrane: Association with Diabetes Insipidus. AMA Arch Derm. 1957;75(1):45–54. doi:10.1001/archderm.1957.01550130047004
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: