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Pseudoporphyria in a hemodialysis patient successfully treated with oral glutamine

Dear Editor, Pseudoporphyria is a blistering disease, which is similar to porphyria cutanea tarda clinically and pathologically. However, porphyrin levels of pseudoporphyria are normal or nearly within normal range in serum, urine, and stool samples. A 54-year-old male patient who was under hemodialysis treatment for 5 years referred to our clinic with the complaints of skin fragility with a recurrent bullous eruption on the face and the dorsum of the hands for 3 months. Examination of the hands revealed multiple vesicles and tense bullae of 5–15 mm in diameter showed on the dorsum associated with multiple crusted erosions, atrophic scars, and multiple milia (Figure 1). Mucosal membranes did not show any pathological signs. Histological examination of a cutaneous biopsy showed crusted and eroded epidermis without inflammatory infiltration, only slight thickening of the vessel walls have been noted. Patient’s serum porfirin level was measured in normal range; his urine examination could not be performed due to anuria. Skin lesions have been diagnosed as pseudoporphyria based on the clinical and laboratory findings. Oral glutathione therapy has been initiated. Two weeks after starting oral glutamine (Resource Glutamine; Nestle, Turkey), 10 g daily and avoiding sun exposure, the formation of blisters ceased and ulcers healed, with leaving scars. The therapy was continued for 2 months without significant side effect. The pathophysiology of pseudoporphyria is not exactly understood. Ultraviolet light-induced free radicals may be the reason causing tissue injury and leading blister formation. It is well known that the patients with chronic renal failure have impaired antioxidant. Skin lesions in hemodialysis patients include a wide spectrum of diseases. Pseudoporphyria is relatively more frequent in hemodialysis patients and it has been reported that administration of acetyl cysteine and glutathione has beneficial effects. Our findings point that oral glutamine administration may be an alternative instead of acetyl cysteine and glutathione therapies.