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Cutaneous porphyrialike photosensitivity after liver transplantation
A P Sheth1, N B Esterly, L G Rabinowitz
1Department of Pediatrics, Medical College of Wisconsin, Milwaukee.
Background:
Liver disease and cholestasis are often associated with abnormal coproporphyrin levels, but photosensitivity eruptions do not usually occur with these conditions. We present a case of a cutaneous porphyrialike photosensitivity in a liver transplant patient with only mildly elevated coproporphyrin levels.
Observations:
A 5-year-old girl developed a cutaneous porphyria cutanea tarda-like photosensitivity after liver transplantation for congenital biliary atresia. Her cutaneous eruption consisted of fluid-filled vesicles and crusted erosions involving her face and arms. These lesions eventually healed with atrophic scarring and milia. The patient was in a state of chronic hepatic rejection, but her serum and 24-hour urine specimens contained only mildly elevated levels of coproporphyrin. Results of histopathologic examination and direct immunofluorescence of a skin biopsy specimen resembled those of porphyria.
Conclusions:
Although clinically porphyria cutanea tarda was suspected, her porphyrin profile was not consistent with that diagnosis. Liver transplantation has become relatively common, but porphyria cutanea tarda-like clinical and histopathologic findings associated with coproporphyrinemia in a liver transplant patient have not been reported to date.