Evan R. Farmer

Neonatal citrullinemia associated with cutaneous manifestations and arginine deficiency

A patient with neonatal citrullinemia (argininosuccinic acid synthetase deficiency), a heritable disorder of the urea cycle, developed a generalized cutaneous eruption at 35 days of age. The skin lesions consisted of erosive, erythematous, scaling patches and plaques. The plasma arginine concentration at that time was low. After treatment with oral arginine supplements, the cutaneous lesions rapidly resolved and the plasma arginine concentration normalized. Histologic features of pretreated lesions included parakeratosis, crust formation, absence of a granular cell layer, pallor of the upper epidermal cells, and a mild, superficial, perivascular, mononuclear-cell infiltrate. Since the patients skin lesions responded to arginine supplements, and since arginine is a component of keratin, we postulate that the skin lesions are the result of arginine deficiency.

Nonmelanoma skin cancer in Japanese residents of Kauai, Hawaii

A prospective 3-year survey of nonmelanoma skin cancer in Japanese-American residents of the Hawaiian island of Kauai was conducted beginning in January 1983. The crude rate for basal cell and squamous cell carcinoma was 123/100,000, 88 times higher than the corresponding figure from Japan. This discrepancy may be attributed to an increase in ultraviolet exposure due to lifestyle and latitude, but arsenic exposure may also have played a role. Nonmelanoma skin cancer in Japanese in Hawaii appears to be a significant problem that has not been appreciated because these malignancies are not reportable to tumor registries.