Clin Pediatr Hematol Oncol.  2009 Apr;16(1):32-37.

Langerhans Cell Histiocytosis in a Two-month-old Infant

• Baek JM
• Sohn SY
• Yang JH
• Woo CW
• Kim YS
• Kim IH
• Lee J
• Lee K

Abstract

Langerhans cell histiocytosis (LCH) is a rare condition characterized by the proliferation of distinctive histiocytes. The clinical manifestations and outcomes of LCH vary from a skin-only self-healing condition to a malignant multi-organ systemic disease. We report a case of LCH in a two-month-old boy. He initially presented only with multiple erythematous eruptions infiltrated with histiocytes that were S100-positive, but CD1a-negative. These eruptions regressed spontaneously after 9 days, after which the boy was in a good condition. Two months later, however, the patient returned with vesicular skin eruptions and melena. A skin biopsy showed the eruptions were infiltrated with histiocytes that expressed both S100 and CD1a. There was a marked increase in the number of mononuclear cells in the peripheral blood of the patient. This case shows that the self-healing type of LCH can only be diagnosed retrospectively and that patients suspected of having LCH should be thoroughly evaluated systemically at the time of diagnosis. They should also be followed-up on a long-term basis to inspect for relapses or any disease progression.

Keyword

Langerhans cell histiocytosis; Self-healing type; Infant

MeSH Terms

Biopsy
Histiocytes
Histiocytosis
Histiocytosis, Langerhans-Cell
Humans
Infant
Melena
Recurrence
Retrospective Studies
Skin