Case Based Expert Perspective on the Management of Generalized Pustular Psoriasis - Episode 2

Case 1: Treatment and Disease Management Challenges

Dr Mark Lebwohl comments on the treatment and management considerations for a patient suffering from severe generalized pustular psoriasis, highlighting the inefficiency of systemic steroids in the management of this condition.

Mark G. Lebwohl, MD: The case gives us a real lesson. Systemic steroids have indeed been used to treat pustular psoriasis. The outcomes have been awful. In fact, in 1969 to 1971, 2 dermatologists named Ryan and Baker studied large numbers of patients. In fact, over a hundred, in which they did a trial comparing folic acid analog—so a drug similar to methotrexate—and compared that to systemic steroids for the treatment of pustular psoriasis. Nearly a quarter of the patients in the systemic steroid arm died and they died because of what I just described to you. Yes, steroids work quickly for pustular psoriasis, but as soon as you try to lower the dose, the condition recurs, and then when you try to put the steroid dose higher, the patients require higher and higher doses to control their pustular psoriasis. That's why the patient became cushingoid. That happens to the high proportion of patients whom you treat with systemic steroids. We recently reviewed a large number of patients at Mount Sinai who had received systemic steroids for psoriasis. On average, when you lowered the dose, most of them flared. The lesson to be learned here is avoid the use of systemic steroids in psoriasis, but especially in pustular psoriasis where their use can be deadly. Now, there are a number of other drugs that have been known to trigger pustular psoriasis. Nonsteroidal anti-inflammatories [NSAIDs] are reported to exacerbate psoriasis. The mnemonic that I use here is nails. Non-steroidal anti-inflammatories, but not pustular psoriasis. The exacerbation that NSAIDs like ibuprofen or naproxen create in psoriasis are very minor. Antimalarials, on the other hand, have been associated with severe exacerbation of psoriasis. There are numerous case reports of this. I have an anecdote here related to a patient of mine who had business in Southeast Asia, and the first time he went, he went on an antimalarial and developed pustular psoriasis. That was treated. He returned. He continued to do business in Southeast Asia but the next time he went, he didn't take an antimalarial, and indeed he came down with malaria. A true story of a patient of mine. Another anecdote related to antimalarials. There was a short period when antimalarials were used to treat Crohn's disease. During that short period, we had multiple cases of generalized pustular psoriasis seen at Mount Sinai. Undoubtedly related to the antimalarial triggering GPP so we do avoid antimalarials in patients with psoriasis in general, but specifically with GPP. The mnemonic I said it was nails, Inderal, or beta-blockers have been known to trigger psoriasis. Again, not pustular psoriasis. Lithium has been associated with the development of pustular psoriasis so we avoid that in these patients. There are some substitutes that psychiatrists can come up with. Then the most common cause of pustular psoriasis is the withdrawal of systemic steroids. That's why we generally try to not use systemic steroids in psoriasis patients in general, but specifically in patients with pustular psoriasis. That's one lesson that we learned from this case.

Transcript edited for clarity