Lupus erythematosus and porphyria. Coexistence in seven patients.
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Biomedical subjects
Publications and source records attributed to D L Cram.
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Four life-threatening dermatoses-Stevens-Johnson syndrome, toxic epidermal necrolysis, Kaposi's varicelliform eruption and purpura fulminans-are unique in their abrupt onset and rapid progress to death, but prompt diagnosis and proper therapy can often cure the condition or prevent undesirable sequelae. Since two of the four conditions can follow the use of a variety of drugs and all may be secondary to an infectious agent, any physician may encounter them in practice and should be aware of their seriousness.
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Ultraviolet irradiation produced an acantholytic phenomenon in normal skin of seven patients with pemphigus foliaceus. In six of the seven patients, this reaction was manifested by a positive Nikolsky sign and was confirmed by biopsy in four. Friction alone, infrared irradiation, occlusive dressing, and a rubefacient failed to produce a similar reaction. The thermal burn in the skin of patients having pemphigus was of subepidermal type. Ultraviolet-induced acantholysis was inhibited by chloroquine phosphate, taken orally, in the two patients tested. Prednisone had no such effect. It is concluded that ultraviolet light is a major aggravating stimulus for pemphigus foliaceus.