Palmoplantar Pustulosis

Background

• Palmoplantar Pustulosis (PPP) is an uncommon, chronic inflammatory disease, characterized by recurrent, and usually symmetrical, sterile pustules on the palms and/or soles that typically present on an erythemato-squamous background.
• The pathogenesis of PPP is not fully understood. The development of PPP may involve genetic, immunologic, and environmental triggering or exacerbating factors such as smoking and allergens, certain drugs, and underlying infections or conditions.
• There is controversy among dermatology experts whether PPP should be classified as its own entity or as a variant of psoriasis.

Evaluation

• Palmoplantar pustulosis (PPP) is usually diagnosed by identification of the typical clinical presentation, which includes eruptions of sterile pustules on the palms and/or soles on an erythemato-squamous base.
• PPP tends to begin on single palm and/or sole and progress to both palms and soles.
• Nail changes, such as hyperkeratosis and pustulation under nails, onycholysis, and discoloration are commonly present with skin lesions
• Concomitant plaque psoriasis is reportedly seen in up to 60% of patients.
• Some patients may report persistent lesions, while others may report periods of remission and relapse.

Management

• The management of palmoplantar pustulosis (PPP) is generally considered to be a challenging , with no curative treatment and high rates of treatment resistance reported. There is limited evidence of efficacy for most drug therapies.
• Advise smoking cessation (Strong recommendation) (see also Treatment for Tobacco Use)
• Consider topical medication, such as a topical vitamin D analogue or topical corticosteroid as first-line treatment to skin lesions.
• Systemic treatment options are available as second-line therapies, particularly following unsatisfactory results with topical treatments.
• Consider acitretin as a commonly preferred primary systemic retinoid treatment. Acitretin can be combined with psoralen plus ultraviolet A (PUVA) photochemotherapy.
• Other systemic nonbiologic treatment options may include: apremilast, cyclosporine or methotrexate.
• For patients with severe of refractory PPP, consider systemic interleukin inhibitor options, such as including guselkumab, secukinumab or ustekinumab, which each have shown some evidence of efficacy for reducing the severity of PPP in adults.
• The tumor necrosis factor (TNF) inhibitors, such as infliximab, adalimumab or etanercept, may yield some clinical improvement in adults with PPP, but they have also been associated with the development of pustular psoriatic lesions.
• Phototherapy is an option with some evidence of efficacy in resolving PPP lesions however, frequent relapses are common.
  • Consider use of psoralen plus ultraviolet A photochemotherapy (PUVA) or ultraviolet A1 phototherapy, which may be more effective than narrow-band ultraviolet B phototherapy.
  • Phototherapy is contraindicated in persons with history of skin cancer.
• Older medication options with very limited evidence of efficacy include tetracycline antibiotics, hydroxyurea and colchicine.
• Consider treatment of underlying conditions or addressing risk factors such as local bacterial infections and contact allergies which have been reported to reduce palmoplantar pustulosis symptoms.