Pustulosis palmaris et plantaris L30.2

Rare, acute, rarely chronic, neutrophilic pustulosis on palmae and/or plantae of unexplained etiology (infectious allergic?)

Worldwide distribution. The estimated prevalence is 0.01-0.05%. As the main literature comes from the Japanese language area, it is assumed that incidence and prevalence are higher in Japan than in the Central European population.

Focal disease; streptococcal infections may trigger relapsing activity.

The relationship between PPP and psoriasis pustulosa palmo-plantaris is the subject of research. Research findings point to a possible common genetic and immunological basis of both diseases. By definition, clinical evidence of psoriasis excludes the diagnosis of "pustulosis palmaris et plantaris".

One hypothesis is that certain T-cells primed against streptococci in the tonsils cross-react with epitopes on human keratin and thus trigger an exacerbation of the disease.

Smoking appears to be an important cofactor in disease development.

In a subgroup of PPP patients, antibodies against gliadin or the presence of a gluten-sensitive enteropathy could be detected in Sweden. In a larger American study (n=215 patients), the detected gluten sensitivity was 1% (Olazagasti JM et al 2017). Chronic thyroid disease was found in 8% of patients.

Women fall ill more frequently than men (3-7:1); the average age in larger collectives was 45.3 years (Olazagasti JM et al. 2017).

Palmae and Plantae, rarely also disseminated on the stem.

Palms and/or soles: Yellow-white, 0,1- 0,5 cm large pustules, some of them confluent to large pus ponds. The pustules dry out in the further course of the treatment. This results in flat brown or yellow papules with or without scaly ruffs. Polymorphic image due to the coexistence of different developmental stages. Moderate itching possible.

General: The general condition may be impaired: fever, signs of an acute streptococcal infection, more rarely signs of a viral infection.

The clinical picture may be accompanied by sterile bone inflammation. This possible co-morbidity results in various clinical names ( SAPHO syndrome, pustulous arthroosteitis, anterior chest wall syndrome (Sonozaki), chronic recurrent multifocal osteomyelitis, pustulotic arthro-osteiitis) which in various variations denote an identical systemic constellation.

Smoking cessation can improve the course of the disease and the therapeutic response.

If necessary, antibiotics according to an antibiogram (throat swab), if necessary short-term glucocorticoids in low to medium dosage such as prednisone (e.g. Decortin) 50 mg/day.

In chronic forms of the disease or resistance to therapy, the use of methotrexate (e.g. MTX) or Ciclosporin A (e.g. Sandimmun®) 2.5-5.0 mg/kg bw/day is described in addition to acitretin (neotigason) 0.5-1.0 mg/kg bw/day. Successes have also been recorded with dapsone (Dapson Fatol) 50-150 mg/day or with the combination of acitretin and local PUVA therapy. The therapeutic risk and possible side effects should be taken into account for all mentioned therapies.

Guselkumab: in a smaller, double-blind, randomized study (n=49) over 24 weeks, patients who had not responded to conventional therapy were successfully treated with Guselkumab (Terui et al. 2017). The interleukin-23 inhibitor thus represents an (off-label) therapy option.

Acute pustulosis palmaris et plantaris is by definition self-limiting. A chronic course lasting for years would define the disease as pustulosa palmoplantaris psoriasis.

Healing after focus repair.

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2. Bacharach-Buhles M et al (1993) The Pustular Bacteride (Andrews). Dermatologist 44: 221-224
3. Olazagasti JM et al (2017) Clinical Features,Etiologic Factors, Associated Disorders, and Treatment of Palmoplantar Pustulosis: The Mayo Clinic Experience, 1996-2013 Mayo Clinic proceedings 92: 1351-1358 .
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5. Tanimoto Yet al. (2014) Presence of keratin-specific antibody-forming cells in palatine tonsils of patients with pustulosis palmaris et plantaris (PPP) and its correlation with prognosis after tonsillectomy. Acta Otolaryngol 134:79-87
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11. Yamamoto T (2011) Triggering role of focal infection in the induction of extra-palmoplantar lesions and pustulotic arthro-osteitis associated with palmoplantar pustulosis. Adv Otorhinolaryngol 72:89-92