Palmoplantar pustulosis

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Palmoplantar pustulosis

Sonja Molin and Thomas Ruzicka

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Palmoplantar pustulosis (PPP) is a skin disease characterized by chronic and relapsing pustular eruptions of palms and soles. Because its clinical and genetic characteristics differ from psoriasis vulgaris, PPP probably must be considered as a separate entity.

Management strategy

PPP is a common disease that is often refractory to treatment and shows a high recurrence rate. Unlike psoriasis, there are no reported associations with candidate genes within the PSORS1 locus or tumor necrosis factor (TNF)-α promoter polymorphisms. Female predominance and high prevalence in smokers are characteristic. Cessation of smoking is essential for the treatment course of PPP. Consideration of comorbidities such as streptococcal or Helicobacter pylori infection, diabetes mellitus, thyroid disease, celiac disease or osteoarthropathy is necessary. Tonsillectomy can be helpful if a streptococcal focus is found. A gluten-free diet is recommended if gluten intolerance is proved. At the beginning of therapy and in patients with milder symptoms of PPP, potent topical corticosteroids are the treatment of choice and are even more effective when used under occlusion. Psoralen and ultraviolet A light (PUVA) therapy is also effective. In more severe or recalcitrant courses of disease systemic medication should be considered. The retinoid acitretin (starting dose 0.3–0.5 mg/kg body weight) is proven, but practical use in women is limited due to teratogenicity. There is evidence that the combination of PUVA with systemic retinoids is superior to the individual treatments. Low-dose cyclosporine (1–4 mg/kg daily) and methotrexate (10–25 mg once weekly) can cause improvement of PPP, but requires careful clinical and laboratory monitoring. Fumaric acid esters have also been reported to be effective.

A multitude of other therapeutic proposals for the treatment of PPP decorate the literature. Among those, the use of the retinoic acid metabolism blocking agent liarozole showed at least modest efficacy.

Reports on tumor necrosis factor (TNF)-α antagonist therapy in PPP show conflicting results, with many reports highlight initiation or aggravation of the disease. Non-TNF-α-inhibiting biologic agents such as alefacept or ustekinumab might be promising new therapeutic options especially in recalcitrant cases. Controlled clinical trials are needed to substantiate the evidence.

Specific investigations

First-line therapies

imageOral retinoids A
imagePUVA B
imageTopical corticosteroids C

Second-line therapies

imageCyclosporine A
imageMethotrexate B
imageFumaric acid esters C
imageExcimer light therapy (308 nm) C

Third-line therapies

imageLiarozole A
imageAlefacept C
imageUstekinumab E

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Palmoplantar pustulosis

Sonja Molin and Thomas Ruzicka

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Palmoplantar pustulosis (PPP) is a skin disease characterized by chronic and relapsing pustular eruptions of palms and soles. Because its clinical and genetic characteristics differ from psoriasis vulgaris, PPP probably must be considered as a separate entity.

Management strategy

PPP is a common disease that is often refractory to treatment and shows a high recurrence rate. Unlike psoriasis, there are no reported associations with candidate genes within the PSORS1 locus or tumor necrosis factor (TNF)-α promoter polymorphisms. Female predominance and high prevalence in smokers are characteristic. Cessation of smoking is essential for the treatment course of PPP. Consideration of comorbidities such as streptococcal or Helicobacter pylori infection, diabetes mellitus, thyroid disease, celiac disease or osteoarthropathy is necessary. Tonsillectomy can be helpful if a streptococcal focus is found. A gluten-free diet is recommended if gluten intolerance is proved. At the beginning of therapy and in patients with milder symptoms of PPP, potent topical corticosteroids are the treatment of choice and are even more effective when used under occlusion. Psoralen and ultraviolet A light (PUVA) therapy is also effective. In more severe or recalcitrant courses of disease systemic medication should be considered. The retinoid acitretin (starting dose 0.3–0.5 mg/kg body weight) is proven, but practical use in women is limited due to teratogenicity. There is evidence that the combination of PUVA with systemic retinoids is superior to the individual treatments. Low-dose cyclosporine (1–4 mg/kg daily) and methotrexate (10–25 mg once weekly) can cause improvement of PPP, but requires careful clinical and laboratory monitoring. Fumaric acid esters have also been reported to be effective.

A multitude of other therapeutic proposals for the treatment of PPP decorate the literature. Among those, the use of the retinoic acid metabolism blocking agent liarozole showed at least modest efficacy.

Reports on tumor necrosis factor (TNF)-α antagonist therapy in PPP show conflicting results, with many reports highlight initiation or aggravation of the disease. Non-TNF-α-inhibiting biologic agents such as alefacept or ustekinumab might be promising new therapeutic options especially in recalcitrant cases. Controlled clinical trials are needed to substantiate the evidence.

Specific investigations

Thyroid disease in pustulosis palmoplantaris.

Agner T, Sindrup JH, Høier-Madsen M, Hegedüs L. Br J Dermatol 1989; 121: 487–91.

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