Spontaneous Remission Of Multi-system Histiocytosis X
Lancet 1(8371): Feb 4 1984; 253-254
View Original Source →Abstract
Two infants presented with biopsy-proven histiocytosis X affecting multiple sites. Since neither showed evidence of organ failure or of constitutional upset, no specific therapy was given. In each case there was long-lasting spontaneous regression of disease. Analysis of blood mononuclear cells revealed a raised T4:T8 (helper:suppressor T lymphocyte) ratio at diagnosis but a normal ratio during remission. These observations support the argument that multi-system histiocytosis X, even in infants (Letterer-Siwe disease), is not a malignancy and that an ‘expectant’ treatment policy may be indicated in selected patients.
Case Details
Personal Characteristics
A first baby was born to healthy unrelated parents, each with healthy children by a previous marriage
Clinical Characteristics
A rash appeared on the babys cheeks within 24 hours of birth. At age 5 days the rash spread to the groins and buttocks and later, with fluctuating severity, to the trunk and proximal limbs. The baby was tachypnoeic (30- 40 /minute at rest) and had some rib recession. There was no anaemia, lymphadenopathy, or hepatosplenomegaly; the baby was not distressed, and his nutrition was excellent.
Remission Characteristics
By age 5 months the skin had cleared, the mouth had healed, and both chest x-ray and lung function had returned to normal. At age 2 years the child was well and chest x-ray was normal. All the earlier skin lesions had disappeared apart from minor intertrigo deep in the folds of the groins.
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Clinical Treatment
Biopsy-confirmed gingival involvement, treated with curettage
Additional Notes
The diagnosis of HX was made by skin biopsy, and the baby was referred to the Hospital for Sick Children. The T4:T8 lymphocyte ratio was very high at 5.4:1 (normal for age 2:1) owing to a deficiency of T8 positive cells.