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Pathogenesis

Mauricio Goihman-Yahr
Dermanities September 28, 2011; 7(1)

Joshua Cohen, M.D., tossed in his bed. The sheets hurt his skin, there was a burning sensation. He had felt itching for the past two or three days. There were red raised welts on his back and abdomen, sometimes crisscrossing as if there had been flagellation. This improved after taking desloratadine. Now it was different… he stood up, went to the medicine chest and took a tablet of gabapentin, walked a few steps, returned and swallowed a second one. He turned back to his bed, lied down, and slumbered.

Dr Cohen was a dermatologist. He had been an outstanding student throughout his career. Thus, he entered first in an excellent internal medicine program with special training in immunology, and then into a top residence in dermatology. He had long had a great interest in the blistering diseases, so after taking his boards, he went into a fellowship with John Stanley. He had participated in the research that determined the antigenic determinants that reacted with pemphigus and pemphigoid antibodies. After the fellowship he climbed the academic ladder and directed a special clinic and laboratory on bullous diseases. He became almost obsessed with finding the true pathogenesis of these conditions, particularly pemphigus and pemphigoid. What was the cause of the rupture of immunological tolerance?. Why were antibodies directed to components of the basal membrane or of the keratinocyte outer covering?

The dermatologist woke from his slumber. His skin burned and hurt. He touched himself; there were bumps in the surface. He ran to the mirror. He was covered with blisters. Some were relatively tiny vesicles. Many were bigger bullae surrounded by erythema. They were tense. He could not elicit a Nikolsky sign by pressing on their surface.

Four hundred years before in this exact day and hour his homonymous ancestor Rabbi and Doctor Joshua Cohen (Jesús Calatrava) was burning at the stake in an auto de fé in Valladolid. He had been accused and convicted of returning to the faith of his ancestors. This he had done after sending his wife and child to his brother´s home in Amsterdam. He had planned to follow suit, but had tarried because of his clandestine congregation and a very sick patient. The Santo Oficio caught him and as there was no recanting he was sentenced to burn. His skin hurt very much and tense blisters formed where flames touched it.