Spontaneous Regression Of Congenital Cutaneous Histiocytosis X; Report Of A Case With Discussion Of Nosology And Pathogenesis
Pediatric Pathology 1(1): Jan-March 1983; 99-106
View Original Source →Abstract
Spontaneous regression of histiocytosis X is a rare biologic event as judged by the extensive recorded experience in the literature. We present a case of congenital cutaneous histiocytosis X that resolved without specific therapy in the first week of life. Histologic and ultrastructural studies confirmed the interpretation. This child is an example of so-called pure cutaneous histiocytosis, which, like the other unisystem forms of the disorders, has an excellent prognosis.
Case Details
Personal Characteristics
2,570 gram female infant, product of a pregnancy complicated by rh incompatibility in gravida 2, para 2, abo mother
Clinical Characteristics
Eight discrete oval cutaneous lesions measuring 0.5 centimeters in diameter, slightly raised, intensely erythematous, and hemorrhagic. Vesicles were noted on the flattened surface. Three new lesions developed in the first 24 hours of life. The liver was palpable 2 centimeters below the right costal margin and the spleen 0.5 centimeters below the left costal margin. Hemoglobin, 12.5 gm/dl; hematocrit, 36.2%; mean corpuscular volume (mcv), 102; mean corpuscular hemoglobin (mch), 34.7; mean corpuscular hemoglobin concentration (mchc), 34.6; white cell count, 13,500/mm3; coombs test, 4+ positive; and total bilirubin, 8.6 mg/dl (direct 1.3 mg/dl) to a high of 14.4 mg/dl
Remission Characteristics
Rapid resolution of the skin lesions, no residual skin changes and extracutaneous manifestations of histiocytosis x are not evident by physical examination or skeletal survey
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Clinical Treatment
An exchange transfusion was performed and phototherapy was instituted. A skin biopsy was also performed
Additional Notes
Spontaneous regression of histiocytosis X is a rare biologic event. This child is an example of so-called pure cutaneous histiocytosis, which, like the other unisystem forms of the disorders, has an excellent prognosis