Pustular Psoriasis - It is more frequent in the fifth or sixth decade of life, has a female predominance, with a woman relationship: 3:1 man. The exact cause and pathogenesis of the disease remains unknown, and there is debate about whether the PPP is a variant of psoriasis or a different condition. Although 18% of patients have lesions of psoriasis in any part of the body, the genetic characteristics of PPP are different from the vulgar psoriasis. Reported associations with thyroid disease, celiac disease / gluten intolerance, consumption of tobacco and type 2 diabetes. The condition worsens by allergies to metals, psychosocial stress, focal infection like tonsillitis.
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| Pustular Psoriasis - Palmoplantar Pustular Psoriasis |
More recently, case reports point to a potentially paradoxical reaction to the tumor necrosis factor inhibition alfa-terapia, which can worsen an existing PPP or trigger a new beginning of PPP. A review of the literature reveals that various types of treatments have been used in PPP over the years, ranging from topical corticosteroids, Anthralin, phototherapy, Grenz Ray, retinoids, tetracyclines and Cyclosporine to biologic therapies. The lack of well documented clinical studies makes it difficult to select an ideal treatment, and the literature on PPP is restricted to case reports or small case series. The purpose of this review is to discuss the current treatment for PPP therapies, based on the results of randomized clinical trials.
Treatments
Topical corticosteroids and topical retinoids
Topical agents remain the treatment more used in the treatment of PPP, even when the disease is usually tough, and topical steroids are the most commonly prescribed medications in PPP. Corticosteroids prevent the formation of new pustules, especially when applied under occlusion, but prolonged use of corticosteroids is not advisable due to adverse effects well-known and disadvantages. Referrals by the topical corticosteroids are temporary.
Topical retinoids can be used to prevent adverse effects or reinforce the effects of steroids, but there were no controlled studies of the effectiveness of topical retinoids in PPP. Tazarotene is the preferred drug.
Systemic retinoids
Studies investigating the effectiveness of oral retinoids in PPP, most evaluate the efficacy of etretinate. Oral etretinate 0.6 - 1 mg/kg/day produced improvement in nearly two thirds of patients with PPP, in a study Lassus and Geiger reported that the acitretin is as effective as etretinate in the treatment of PPP, and may be used at the same dose. Meta-analysis have shown improvement complete or excellent in 39% of cases after 12 weeks of treatment with oral
retinoids, and acitretin not only decreased the number of pustules, but it also helped control of coexisting hyperkeratosis. During treatment with acitretin, is required dose according to the weight adjustment. The drug can be administered alone or in combination with psoralen more UVA (PUVA) where also required a dose adjustment.
Phototherapy
PUVA, with oral or topical psoralen in the treatment of PPP has been used. Murray and cabbage and Rosen and col reported that oral local PUVA is more effective than placebo and 176-228 J/cm2 UVA dose is required to control the disease, however, were lower percentages of responses by others. The main advantage of topical PUVA on oral PUVA is to avoid the potential adverse effects of systemic therapy, so it is preferred the topical PUVA for selected patients with gastrointestinal or liver disease or patients with cataracts.
Many studies failed to show the effectiveness of topical PUVA in PPP. It showed that oral PUVA, etretinate and PUVA, topical or oral or combinations were superior to topical PUVA only with regard to effectiveness and efficiency in PPP home.
It has investigated the value of PUVA in maintenance therapy. Nielsen and Madsen reported that 3 of 9 patients with PPP receiving PUVA for short time then of the remissions achieved Corticoid under occlusion not relapsed in the coming year. Ettler and Richards reported that 2 weeks relapses occurred in 10 patients after completed acitretin, despite therapy PUVA therapy, so topical PUVA was only unable to maintain remission after therapy with acitretin.
These findings call into question the role of PUVA as maintenance therapy.
Cyclosporine
Many studies have investigated the effectiveness of Cyclosporine in patients with PPP. Low-dose Cyclosporine (1 - 2.5 mg/kg/day) is an effective treatment of choice in 2/3 of patients with PPP. Erkko and col evaluated the dose of Cyclosporine that is sufficient to control the activity of the disease in a study that included 58 patients with PPP, and found that 1 - 2 mg/kg/day of Cyclosporine was effective in the majority of patients. The study began with a dose of 1 - 2 mg/kg/day of Cyclosporine for 4-8 weeks and 4 mg/kg/day dose increased only in patients who did not have a satisfactory response with the lowest dose. In the follow-up period, there was an increase of pustules to 12 months after the suspension of the treatment, but average values not exceeded baseline values at each stage.
The use of low-dose Cyclosporine may prevent the dose-related toxicity (e.g. hypertension, Nephrotoxicity) Cyclosporine, and achieve a greater acceptance of Cyclosporine in the treatment of PPP. The development of Generalised Pustular psoriasis then discontinue Cyclosporine is a cause for concern. Although it is a complication rarely reported, gradual decrease in Cyclosporine strategies are developed to avoid it.
Colchicine
The efficacy of colchicine on PPP has been shown for 30 years. Thestrup-Pedersen and Reymann reported significant improvement with colchicine in 10 of 27 cases, and Takigawa et al. found it effective in 13 of 32 cases in 2-8 weeks of treatment in a non-controlled trial. However, controlled studies not randomised trials did not support this efficiency.
Other treatments
There is only limited experience of radiotherapy Grenz and tetracyclines in PPP. Controlled trials there are not current trials that show some effectiveness of methotrexate, Anthralin or topical retinoids for the treatment of PPP.
The evidence in the treatment of PPP is inadequate and unsatisfactory. So it is needed further investigation to discover better treatments and validate the current therapeutic options.
What does this article to the Dermatologic practice?
Pustulosis palmoplantar (PPP) is a pustular dermatosis chronic and recurrent characterized by multiple sterile pustules and plates erythematous on palms and soles.
The exact cause of the pathogenesis of the disease remains unknown and there is still a debate on whether it is a variant of psoriasis or a different condition. Several treatments exist for this disease. The purpose of this review is to discuss the treatment options for PPP, based on randomized controlled trials.
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