NCIt definition : A cutaneous form of the genetic photosensitive disease, porphyria, that is characterized
by onset in adult life and the presence of scarring bullae, hyperpigmentation, facial
hypertrichosis, and sometimes sclerodermatous thickenings and alopecia. Uroporphyrins
are found in the urine due to a deficiency of uroporphyrinogen decarboxylase, an enzyme
required for the synthesis of heme.;