Histiocytosis x (hand-schuller-christian syndrome; lipoid granuloma; xanthomatous granuloma syndrome; schuller-christian-hand syndrome; letterer-siwe syndrome; acute histiocytosis x; eosinophilic granuloma; reticuloendotheliosis syndrome) 5

General: The term histiocytosis X has been proposed to include Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma of bone; there are sufficient grounds to treat Hand-Schuller-Christian and Letterer-Siwe together as different phases of the same disease process; eosinophilic granuloma most likely represents a reaction pattern, sharing some histologic features with the first two but nonetheless carrying a more benign prognosis; Letterer-Siwe disease is referred to as acute differentiated histiocytosis; Hand-Schuller-Christian disease is referred to as subacute differentiated or chronic differentiated histiocytosis; Letterer-Siwe etiology is unknown, onset is in infancy and early childhood, and prognosis is generally poor; Hand-Schuller-Christian etiology is unknown, onset is in childhood, male preponderance is 2:1, and prognosis is chronic with remissions; eosinophil may playa contributory pathophysiologic role.

Ocular: Exophthalmos; ocular pulsations; orbital roof defects; xanthelasma; blepharitis; internal ophthalmoplegia; nystagmus; retinal hemorrhages; papilledema; optic atrophy; uveitis; hypopyon; pannus; bullous keratopathy; corneal ulcer; hypochromic heterochromia; retinal detachment; cataract; scleritis.

Clinical: Hepatosplenomegaly; lymphadenopathy; skin lesions with papular eruptions; ecchymosis; purpura; bone lesions; anemia; fatigue; anorexia; fever; xanthoma of the skin; diabetes insipidus; skull defects; lung fibrosis; cardiac insufficiency.

Christian HA. Defects in membranous bones, exophthalmos and diabetes insipidus, vol. I. New York: Paul B. Hoeber, 1919:390.

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Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Hand A. Polyuria and tuberculosis. Arch Pediatr 1893; 10:673.

Mittelman D, et al. Ocular involvement in Letterer-Siwe disease. Am J Ophthalmol 1973; 75:261.

Pearlstone AD, Flom L. Letterer-Siwe's disease. J Pediatr Ophthalmol 1970; 77: 103-l05.

Petersen RA, Kuwabara T. Ocular manifestations of familial lymphohistiocytosis. Arch Ophthalmol 1968; 79:413.

Trocme SD, Aldave AJ. The eye and the eosinophil. Surv Ophthalmol 1994; 39:241-252.