Bullous disease of dialysis

Bullous disease of dialysis Bullous disease of dialysis is a skin fragility syndrome and veziculizare. The skin lesions are clinically and histologically similar to porphyria cutanea tarda. Lesions typically occur on sun-exposed skin on the dorsal hands in individuals who are treated with continuous dialysis for chronic renal failure. Mecanobuloasa disease was observed in patients with end stage renal disease treated by chronic ambulatory peritoneal dialysis and hemodialysis.
The level of porphyrins in urine and stool is normal. The level is slightly elevated plasma porphyrins excluding true that porphyria patients anefrici coarse determine abnormal values.
These skin lesions cause cosmetic discomfort and interfere with the use of hands. May become painful and can scar secondarily infected but are not life threatening.
Causes and Risk Factors Bullous dermatosis of dialysis etiology remains unclear, although the propensity worsening lesions and fragility of sun exposure suggests a phototoxic mechanism.
Since the level of plasma porphyrins in people with chronic renal failure is a little high, photosensitisation the porphyrins may play an important role in some cases. However photosensitisation porphyrins seems to be the main cause because most dialysis patients with similar levels of porphyrins and serum fotocutanate not develop lesions. Speculation that fotosensibilizantii during dialysis (produced emanate from pipes) are responsible remains unproven. Concomitant use of phototoxic potential therapeutic agents (furosemide) can not be identified in most cases. Effects of high concentrations of aluminum in therapeutic sources or external sources of heme biosynthesis enzymes, leading to overproduction of porphyrins has been suggested as possible etiology, but still unproven.
Signs and symptoms People with chronic renal dialysis bullous dermatosis presenting these lesions develop after months or years of maintenance dialysis regimes. The lesions are more florid after exposure to sunlight, patients still do not realize the role of sunlight in causing damage than when they start to become painful.
Physical Exam: Vesicles and hemorrhagic fluid-filled blisters or erosions exuding clear and mostly occur on the dorsal of the hands, scalp, face and neck. Healing of crusted erosions leave atrophic scars. Milia, pigmentation and hypertrichosis occurred more frequently.
Diagnosis Laboratory studies: To exclude porphyria true will evaluate plasma porphyrins. The test may be abnormal coarse a person with compromised renal function.
Histological examination: Microscopic examination of biopsy samples from a vesicle showed subepidermice bubbles can not be distinguished from those of true porphyria, with oedematous dermal papillae and perivascular lymphocytic infiltrate. Ultrastructural studies show thickening of the walls of dermal venules and basal lamina junction due dermoepidermice replicated to degranulate mast cells and hyaline masses granulofilamentos secreted by fibroblasts. Examination by direct immunofluorescence shows the presence of immunoglobulin G and immunoglobulin A versatile, M, complement and fibrin around dermal venules.
The differential diagnosis is made with the following disorders: bullous pemphigoid, epidermolysis bullosa, bullous lupus erythematosus, porphyria cutanea tarda.
Treatment Any photosensitiser drug that the patient could use it to be identified and stopped. Oral N-acetylcysteine used as a healing vesicle determine radiation protection studies in patient groups. Avoiding sunlight, using sunscreens and avoiding mechanical trauma may help reduce the severity of injuries. For bubbles secondarily infected may need treatment with antibiotics.