Cutaneous Manifestations of Porphyria

Clinical Aspects of Importance in the Porphyrias

“…A similar case has been described by Dean 43 following the use of ‘Lyndiol’ while McGregor et al 44 ,. Discombe & Treip 45 and Rimingtom 46 have also reported a reciprocal relationship in cases of porphyria complicated by jaundice. However, our studies in uncomplicated variegate porphyria have not substantiated Rimington's claim 21 of reciprocity in the route of excretion in protocoproporphyria (variegate porphyria) whereby in remission the main route of excretion is via the faeces and in the acute attack via the urine.…”

The porphyrias: a review.

“…Subsequent reports established that 'porphyria cutanea tarda' was predominantly a disease of men over the age of 40, who frequently gave a history of alcoholism and showed evidence of liver dysfunction and that both attacks of acute porphyria and a family history of porphyria were uncommon in this group (Szodoray and Sumegi, 1944;Brunsting, Mason, and Aldrich, 1951;Brunsting, 1954). However, there were also accounts of a less common cutaneous porphyria in which both photosensitivity and acute attacks occurred (Gray, Rimington, and Thomson, 1948;Watson, 1951 ;MacGregor, Nicholas, and Rimington, 1952;Rimington, 1952;Calvert and Rimington, 1953;Discombe and Treip, 1953;Wells and Rimington, 1953;Holti, Rimington, Tate, and Thomas, 1958). In general these patients were younger than the former group and in some there was evidence of porphyria in other members of the family although there was no difference between the skin lesions in the two groups.…”

Porphyria Cutanea Tarda

“…In the nonbullous cases the dermatological diag¬ nosis should rely on other features, such as the thickening or sclerodermizing of the skin, which may be pronounced (6,7,8,11,14, and 15) and may thus cause an older appearance of the patient (5, 6, 8, 14, and 15) and a rather immobile, mask-like face (6, 7, 8, 11, 14, and 15); the thickening may also be slight (10 and 13) or practically unnoticeable (9). In these latter cases the pigmentation may be also less and suspicion then could only be aroused by some scratcheffects on the face, superficial minute pearly atrophie spots (best observed under tangen¬ tial Wood-light), especially on a rim along the hairline (area most exposed to the sun) and on the backs of the hands; furthermore, the facial hypertrichosis (7, 9, and 13) and milia may be helpful.…”