Peer-reviewed veterinary case report
Dog with inherited skin and liver disease causing pigment changes
By Kunz, Brittany C et al.·Published in Journal of the American Veterinary Medical Association·2020·View original on PubMed →
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Original publication title: Congenital erythropoietic protoporphyria and protoporphyric hepatopathy in a dog.
- Species:
- dog
Plain-English summary
A 6-month-old Clumber Spaniel was brought to the vet because he was small for his age and had recurring skin problems on his head. Tests showed liver issues and a buildup of certain pigments in his blood and liver, leading to a diagnosis of congenital erythropoietic protoporphyria, which can cause sensitivity to sunlight and liver damage. To manage his condition, the vet recommended avoiding sunlight, along with medications like ursodeoxycholic acid and antioxidants. At a follow-up when he was 4 years old, the dog was stable and did not show signs of jaundice, although he still had some skin issues related to his condition.
People also search for: Clumber Spaniel skin problems · dog liver disease treatment · congenital erythropoietic protoporphyria in dogs
Abstract
CASE DESCRIPTION: A 6-month-old sexually intact male Clumber Spaniel was evaluated because of small stature, recurrent dermatitis of the head, and progressive pigmentary hepatopathy. CLINICAL FINDINGS: Clinicopathologic findings included nonanemic hypochromic microcytosis, hypocholesterolemia, persistently high serum liver enzyme activities, and anicteric hyperbilirubinemia. Histologic examination of liver biopsy specimens collected when the dog was 6 months and 2 years of age revealed expansion and bridging of portal tracts, occasional centrilobular parenchymal collapse, scattered lymphoplasmacytic infiltrates, and dark red to brown pigment within large aggregates of macrophages, engorged bile canaliculi, and hepatocytes. The pigment failed to stain for the presence of iron, copper, bile, and glycoprotein and, when examined with polarized microscopy, emitted a yellow to green birefringence with occasional Maltese cross configurations. Further analyses confirmed marked porphyrin accumulation in blood, urine, feces, and liver tissue; protoporphyrin accumulation in RBCs and liver tissue; and a signature porphyrin profile and fluorescence peak consistent with erythropoietic protoporphyria. Advanced protoporphyric hepatopathy was diagnosed. The chronic dermatopathy was presumed to reflect protoporphyric photosensitivity. TREATMENT AND OUTCOME: Management was focused on avoiding conditions known to induce heme synthesis and catabolism, administrating ursodeoxycholic acid and antioxidants-adenosylmethionine and vitamin E, and avoiding sunlight exposure. At follow-up at 4 years of age, the dog was stable without evidence of jaundice but with probable persistent erythropoietic protoporphyria-related solar dermatopathy. CLINICAL RELEVANCE: Clinical and histologic features of congenital erythropoietic protoporphyria and resultant protoporphyric hepatopathy, the diagnosis, and the successful management of a dog with these conditions over 4 years were described. Veterinarians should consider porphyric syndromes when unusual pigmentary hepatopathies are encountered.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/33226294/