Von Zumbusch: The worst kind of psoriasis
Three weeks ago MS’s daughter called me about her mom. She sounded anxious and was referring her mom’s condition to me. MS was a psoriasis patient who visited me several years ago until I suddenly lost track of her. According to her son, she went to another dermatologist because she felt that my medicines were quite pricey. According to her daughter, it would be difficult for her mom to see me because she has difficulty walking as her skin is very tender everytime she moves. I told the daughter that I had to see her mom to be able to assess her and treat her properly. Eventually, they brought MS to my clinic. When I saw MS, she was really in severe pain, febrile and in a wheelchair. She was able to stand and walk towards me, but her ambulation was really unusual. It was slow and in very, very short strides, accompanied by a grimace. Upon seeing her skin, I was quite worried because it was very angry red in color, very dry, tight, scaly, and extremely tender. I told her daughter that she needed to be confined because she could be having Von Zumbusch (a life-threatening kind of psoriasis). Good thing the family agreed to have her admitted. During an interview with MS, I was able to gather that she was given isotretinoin, methotrexate, and PUVA treatment (oral psoralen drug plus ultraviolet A light exposure) all at the same time, together with clobetasol mix with petroleum jelly applied on the skin. I was wondering why she was given a very strong drug that is more indicated for acne when her problem is psoriasis. Isotretinoin will make the skin dry, tight, cracked, and sometimes itchy. Then to top it all, she was treated with PUVA. Ultraviolet exposure is contraindicated in patients taking isotretinoin (the efficacy of isotretinoin for psoriasis has not been studied, either singly or in combination; MS was given methotrexate, too). I was not able to get the dose of each drug as the patient does not know it, too. I saw MS in the morning, but she was admitted in the evening. When I saw her that night on the day of admission, very tiny pustules started to appear on the angry red plaques (elevated lesions) and patches (flat lesions). MS was treated as a case of pustular psoriasis (Von Zumbusch). On her first night, the patient was quite agitated due to the many people entering her room, taking her BP and getting blood extraction for her laboratory tests. So, I ordered the nurses to refrain from touching her skin as it was really very painful. After two days, the patient was discharged, feeling comfortable and much better, but the problem here is that the drug isotretinoin stays in the system for a long time so occasionally, if she fails to take care of her skin properly with the lotions and creams given to her, she might have a flare-up anytime.
Psoriasis is an incurable chronic skin condition that causes the buildup of thick silvery scales and itchy dry patches (flat lesions) and, most of the time, plaques (elevated lesions) that can be painful at times. It affects more than 125 million people. On the other hand, generalized pustular psoriasis is a rare and serious skin disorder that presents with flares of widespread sterile pustules on a background of red and tender skin. It is also known as acute generalized pustular psoriasis or Von Zumbusch. About 10% of patients with generalized pustular psoriasis have a preceding history of psoriasis in which there are persistent, circumscribed, red and scaly plaques.
Strong topical or oral corticosteroids are actually dangerous. They do clear up the psoriasis while the patient is taking them, but when the patient stops, the psoriasis often comes back even worse. Any person with psoriasis who is prescribed oral corticosteroids for another condition should discuss their use with a dermatologist before taking them. Emotional stress and pregnancy can lead to localized areas of pustulosis, often associated with existing plaques of psoriasis.
People with Von Zumbusch pustular psoriasis often need to be hospitalized, as this can be a life-threatening condition. The treatment options are as follows:
• Intensive nursing and supportive therapy with attention to hydration, fluid balance, nutrition, and temperature regulation.
• Bland topical compresses and saline or oatmeal baths assist in soothing and debriding affected areas.
• There is a very limited evidence base regarding the efficacy of different systemic therapies separately or in combination, partly due to the rarity of the disease. Treatment can be challenging, with problematic side effects or lack of response. Indeed, case reports have implicated some of the agents used to treat generalized pustular psoriasis aggravate or induce the condition. For example: ciclosporin, etanercept and infliximab.
• Biological therapies — infliximab, etanercept, ustekinumab, adalimumab, and anakinra.
• Psoralen combined with ultraviolet A (UVA) treatment (PUVA) cannot normally be tolerated in the early stage, except perhaps in children who tend to experience a milder disease. Once the patient is stable, usually after several days of acitretin, PUVA may be commenced.
But why wait for pustular psoriasis to occur? A lot of newer and more effective drugs are now available to treat psoriasis before it ends up becoming the pustular type. Take, for example, the first Interleukin 17 A inhibitor (a critical molecule in the immune system called IL-17 that has been linked to the skin inflammation of psoriasis) to be FDA-approved. So far, reviews and studies claimed it to be the best biological agent in the treatment of moderate to severe plaque psoriasis and was also approved by the European Commission as a first-line systemic treatment for moderate to severe plaque psoriasis in adults who are candidates for oral therapy.
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For questions or inquiries, call 09174976261, 09998834802 or 263-4094; email gc_beltran@yahoo.com.
