Direct immunofluorescence studies show deposition of immunoglobulins and complement in and around upper dermal vessel walls and, to a lesser extent, at the dermoepidermal junction. Biopsies of livers of individuals with protoporphyria typically reveal brown pigment in hepatocytes, Kupffer cells, portal macrophages, and small biliary structures. The brown pigments are deposits of protoporphyrin that can be appreciated by electron microscopy to contain crystals and are birefringent when examined with polarization microscopy. For protoporphyria uncomplicated by hepatobiliary disease, the major problem is lifelong cutaneous photosensitivity. Anemia, if present, typically is mild and rarely requires specific therapy.
Cholelithiasis is managed surgically.
Liver dysfunction is an ominous development for which medical remedies are not consistently effective.