Spontaneous Remission Of Multi-system Histiocytosis X
Lancet 1(8371): Feb 4 1984; 253-254
View Original Source →Abstract
Four neonates had solitary, congenital, rapidly growing, spontaneously ulcerating tumors of the face, trunk, and extremities. No extracutaneous involvement was found, and all lesions spontaneously involuted. Mononuclear cells of the cutaneous infiltrate were Langerhans’ cells. These findings expand the spectrum of congenital self-healing reticulohistiocytosis.
Case Details
Personal Characteristics
A 3-month-old girl, born to healthy unrelated pakistani parents
Clinical Characteristics
A rash had been present for 2 weeks. A generalised brown-red papular eruption over the trunk and scaly erythema of the scalp resembling cradle-cap were noted, but there was no lymphadenopathy, hepatosplenomegaly, or respiratory difficulty.
Remission Characteristics
Over the next 5 months the disease underwent spontaneous and complete remission. At age 2 years clinical examination, chest x-ray, and the t4:t8 ratio (1:1) were normal, and the child continued to make good general progress.
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Additional Notes
The diagnosis of HX was established by skin biopsy. After recovering rapidly from gastroenteritis, the baby thrived. Analysis of blood mononuclear cells showed a raised T4: T8 ratio of 3:1.