HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, acute, rarely chronic, neutrophilic pustulosis on palmae and/or plantae of unexplained etiology (infectious allergic?)
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Occurrence/EpidemiologyThis section has been translated automatically.
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.
EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
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).
LocalizationThis section has been translated automatically.
Palmae and Plantae, rarely also disseminated on the stem.
Clinical featuresThis section has been translated automatically.
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.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
General therapyThis section has been translated automatically.
Smoking cessation can improve the course of the disease and the therapeutic response.
External therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
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.
Progression/forecastThis section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Andrews GC, Machacek GF (1935) Pustular bacterids of hands and feet. Arch Dermatol Syphil 32: 837-847
- Bacharach-Buhles M et al (1993) The Pustular Bacteride (Andrews). Dermatologist 44: 221-224
- 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 .
- Takahara M (2011) Clinical outcome of tonsillectomy for palmoplantar pustulosis and etiological relationship between palmoplantar pustulosis and tonsils. Adv Otorhinolaryngol 72:86-88
- 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
- Terui T et al (2018) Efficacy and Safety of Guselkumab, an Anti-interleukin 23 Monoclonal Antibody, for Palmoplantar Pustulosis: A Randomized Clinical Trial. JAMA Dermatol 154:309-316.
- Uzun G et al (2013) Pustulosis palmaris et plantaris. BMJ Case Rep doi: 10.1136/bcr-2013-009400
- Weißenseel P (2016) Pustular psoriasis. Dermatologist 67: 445 - 453.
- Yoshida Y et al (2003) A case of pustulosis palmaris et plantaris that began as generalized pustular eruption. J Dermatol 30: 141-145
- Yamamoto T (2013) Pustulotic arthro-osteitis associated with palmoplantar pustulosis. J Dermatol 40: 857-863
- 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
Incoming links (19)Acrodermatitis continua suppurativa; Acropustuloses; Andrews bacterium; Andrews syndrome; Ap1s3 gene; Bacteride andrews; Betamethasone valerate cream hydrophilic 0.025/0.05 or 0.1% (nrf 11.37.); Betamethasone valerate emulsion hydrophilic 0,025/0,05 or 0,1 % (nrf 11.47.); Chronic non-bacterial osteomyelitis; Deficiency of IL-1 Receptor Antagonist; DIRA; ... Show all
Outgoing links (21)Acitretin; Acrodermatitis continua suppurativa; Betamethasone valerate cream hydrophilic 0.025/0.05 or 0.1% (nrf 11.37.); Betamethasone valerate emulsion hydrophilic 0,025/0,05 or 0,1 % (nrf 11.47.); Ciclosporin a; Dyshidrotic dermatitis; Glucocorticosteroids systemic; Glucorticosteroids topical; Guselkumab; Hydrocortisone cream 0.5-2.0% (w/o); ... Show all
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