Palmoplantar Pustulosis (PPP)
Last Updated: 2021-12-20
Königsbeck 1917; Barber; Andrews and Machacek 1935
Pustular palmoplantar psoriasis, acral PP, acral pustular psoriasis, pustular psoriasis of the palms, pustular psoriasis of the soles, pustular bacterid Andrews
Palmoplantar pustulosis (PPP) produces primary, chronically persistent (>3 months), sterile, macroscopically visible pustules on the palms and/or soles of the feet and may occur with or without psoriasis vulgaris.
0.01% for Europeans, 0.1% in Japan
Primary pustular form: Barber-Königsbeck
Secondary pustular form: Andrews. Vesicles form initially, which then become cloudy. Commonly observed especially in Japan.
The IL-36 signalling pathway is central. However, high rates of mutations in the IL36RN gene have not been described as in generalised pustular psoriasis (there >50%), in PPP it is about 5% who carry this mutation.
White-yellow pustules, which are initially very small and can be detected with the dermoscope. Surrounding erythema, often swelling. Pain like tiny cuts. After a few days, the fluid in the pustules is absorbed and brown macules form, which can persist for weeks and disappear again due to exfoliation during skin regeneration. The pustules can be loosely distributed in the palmoplantar area or concentrated in individual oval-round areas. They are not infrequently transgredient; the affected areas can, for example, reach straight up the medial arch of the foot. Therefore, purely palmoplantar photographs are often not sufficient for quantification.
Typical are different stages of efflorescences. If there is a simultaneous, abrupt appearance of pustules, one thinks of the form of pustular bacterid Andrews.
- ERASPEN criteria (see definition), typical clinic
- Exclude secondary pustules in the sense of superinfected vesicles in the context of dyshidrotic eczema
- Search SAPHO syndrome (joint complaints, acne)
If only the terminal limbs are affected, then it is not PPP but acrodermatitis continua suppurativa Hallopeau.
- Stop smoking, also no e-cigarettes, if possible complete nicotine abstinence.
- Skin care, to reduce irritation of the skin.
Chronic course, waxing and waning.
- Skin care: Excipial Repair and Protect
- Topical steroids: In acute cases Dermovate ointment 1-0-0, then try to downgrade to Betnovate ointment. Elocom less atrophogenic, on the other hand it not infrequently burns a little.
- If possible, then switch to calcineurin inhibitor Protopic 0.1% ointment, apply 2x per day
- UVBnb, bath PUVA
- Acitretin, combine with bath PUVA if necessary
- Cyclosporine, combine with bath PUVA if necessary. Not ideal in terms of NMSC risk, but often done
- IL-17 antagonists, IL-23 antagonists registered in Japan for treatment of PPP