Palmoplantar Pustulosis (PPP)
Last Updated: 2022-02-25
Author(s): Navarini A.
ICD11: -
Last Updated: 2022-02-25
Author(s): Navarini A.
ICD11: -
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.
Acral
If only the terminal limbs are affected, then it is not PPP but acrodermatitis continua suppurativa Hallopeau.
Chronic course, waxing and waning.
Topical therapy
Phototherapy
System therapies