Hydroa Vacciniforme

This is a vesicular and bullous disease which tends to recur each summer in childhood on uncovered parts of the body. Although both sexes are affected, the disease occurs more often in boys, beginning during the first three or four years of life and usually disappearing soon after puberty. Mild types of the disorder are characterized by papules or small vesicles (summer prurigo of Hutchinson or hydroa aestivale ), but when the eruption is severe there are bullae, impetiginous crusts, and pitted scars (hydroa vacciniforme). The lesions appear mostly on the face and the extensor aspects of the extremities and are arranged symmetrically chiefly over the nose, cheeks, ears, and dorsal surfaces of the hands. They may arise on other areas, including the cornea, and in this location cause scars that interfere with vision. Itching and burning, as well as mild constitutional symptoms, may precede the outbreak of skin lesions, which appear in crops. These begin as erythematous macules that rapidly develop into vesicles and bullae, which in the course of three or four days dry up with the formation of adherent brown crusts. Some of the bullae become umbilicated, forming a central punctum about which are the remains of the bulla; the whole surrounded by a mildly inflammatory zone, so that the appearance simulates a vaccination vesicle. Fresh crops of lesions appear at various intervals, which in summer may be so short that they are in-determinate. The itching may lead to lichenification that persists through the winter. In some cases of hydroa vacciniforme, porphyrinuria occurs so that the urine fluoresces under Wood's light. The disease in many respects resembles the congenital type of porphyria. The treatment of hydroa vacciniforme is the avoidance of exposure to sunlight and the elements and the use of nicotinic acid, quinacrine hydrochloride, and estrogens. Locally sun screens and hydrocortisone ointment are useful.