Porphyria With Cutaneous Manifestations

Observations on Porphyria Cutanea Tarda

“…Stains for amyloid were negative. This is the pic¬ ture of the change in skin after chronic light exposure, including the so-called senile skin, which was emphasized in a previous paper 6 (Fig. 5b).…”

Cutaneous Manifestations of Porphyria

“…Porphyrin may be excreted in the urine of subjects who are symptom-free, although so far this has been detected only in the sibs of patients with porphyric bullous dermatosis (Templeton and Lunsford, 1932;Brunsting andMason, 1946, 1949;Calvy et al, 1951). We are thus led to postulate a syndrome in which porphyrin may be excreted without exciting any symptoms; whereas in some patients who excrete porphyrin the symptoms of a porphyric bullous dermatosis may appear.…”

“…In such patients no dermatosis occurs, but if the liver is damaged or production of porphyrin is excessive the blood porphyrin rises above the critical level at which dermatosis occurs. Urbach (1936), Szodoray and Suimegi (1944), Brunsting and Mason (1946, 1949), Zeligman and Baum (1948, and Calvy et al (1951) record altogether 32 cases in which chronic alcoholism (with or without syphilis) seems to have been the principal factor in determining the onset of dermatosis; and of - Barnes's (1945Barnes's ( , 1951 11 cases, 7 were among Bantus, almost all of whom suffer from a nutritional cirrhosis of the liver. Bolgert et al (1952) record the onset of the disease at age 65 in a sufferer from haemochromatosis.…”

“…The combination of exposure to sunlight and liver damage may account for the high frequency of the disease found in South Africa by Barnes. Conigenital Nature of the Disease.-Porphyric bullous dermatosis is a familial disease. Urbach (1937, Case 1) notes the disease in father and son; Gross (1910) and Hudelo and Montlaur (1919) describe cases in brothers and sisters, while Anderson (1898), Templeton and Lunsford (1932), and Brunsting andMason (1946, 1949) record cases in which the patient's mother or sister was symptom-free though excreting porphyrin. Borzow (1933) and Calvy et al (1951) describe families with symptoms in sibs of four or five generations.…”

Uroporphyrin I Stimulation of Collagen Biosynthesis in Human Skin Fibroblasts

“…Furthermore, in -25% of patients with PCT, indurated hypopigmented plaques often indistinquishable from the cutaneous lesions of scleroderma are seen. Unlike the blistering lesions of PCT, which are due to cutaneous photosensitivity, these sclerodermatous plaques are found in both light-exposed and light-protected body areas (13,14). This suggests that photosensitivity is not an absolute requirement for this pathologic reaction to occur.…”