Pustular psoriasis palmaris et plantaris L40.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.12.2020

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Synonym(s)

Psoriasis palmaris et plantaris (pustulöser Typ); Pustular psoriasis of the Königsbeck-Barber type; pustular psoriasis palmoplantaris

History
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Königsbeck, 1917; Barber, 1936

Definition
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Chronic pustular variant of (non-pustular) psoriasis palmaris et plantaris, restricted to the palms of the hands and/or soles of the feet. The disease can be permanent or chronically recurrent over years.

Occurrence/Epidemiology
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The prevalence of PPP is estimated to be 0.01-0.05% (L. Hellgren 1971). In Europe, women are 4 times more likely than men to contract the disease.

Etiopathogenesis
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The pathogenesis of PPP is unclear.

Genetics: In larger clinical study groups only 2-19% (in other studies about 25%) could be shown to be associated with chronic plaque psoriasis. An association of PPP with variants in the IL-36RN gene (detected in pustulosa generalisata psoriasis ) was found in one study but not in a second study. This was also the case with the CARD14 gene.

Dyshidrotic vesicle: The initial efflorescence is an intraepidermal dyshidroticvesicle in the acrosyringium area of the eccrine sweat gland. Pustular formation begins when there is direct contact between the vesicle and the stratum corneum.

Infections: Infections (especially streptococcal infections - Remark: The M-protein of streptococci has a sequence homology to keratin 17) seem to trigger the clinical picture (see also the explanations on acute pustulosis palmaris et plantaris).

Smoking: worsening is observed with smoking (80% of patients smoke at first manifestation). The cause may be the expression of nicotinic acetylcholine receptors on the acrosyringium. The agonist nicotine secreted by the sweat glands could initiate an inflammatory cascade.

Gluten Sensitive Enteropathy: a relationship has been postulated, but needs to be confirmed.

Manifestation
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w>m; first manifestation at the age between 40-60 years (median 48 years).

Localization
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Palms and soles of the feet, especially in the area of the arch of the foot, are affected.

Clinical features
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A relapsing course of the disease is characteristic. Clinically, 2 basic types can be distinguished:

  • On the palms of the hands and/or soles of the feet there are whitish or whitish-yellowish cloudy, sterile tiny pustules on sharply defined erythema or red non-scaly plaques (predominantly pustular type).
  • In addition to these rather monmorphic clinical pictures, variants of PPP with pronounced polymorphic changes with a colourful juxtaposition of erythema, dyshidrotic vesicles, pustules and scaly papules or plaques (mixed pustular-hyperkeratotic type) are found.

Histology
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Superficial dermatitis with epidermotropy and the formation of unilocular pustules in the upper parts of the epidermis.

Differential diagnosis
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Pustulosis palmaris et plantaris: Diffuse distribution of larger pustules over palms and/or soles, possibly general symptoms with bone involvement.

Psoriasis palmaris et plantaris (plaque type): Classic form of psoriatic involvement on palms and soles; no pustule formation

Acrodermatitis continua suppurativa: Involvement of the terminal phalanges. Mostly with nail involvement.

Tinea palmaris/plantaris: Scaling, enlarging plaques, subacute course; fungal evidence, no other psoriatic foci.

Complication(s)
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Rare infection.

Therapy
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General therapy
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Iontophoresis treatment can be successful. The hands or soles of the feet are bathed in tubs of tap water and then an alternating current of defined strength is passed through (device: e.g. Pso-Ionto).

Radiation therapy
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Locally applied PUVA therapies have proven to be effective, especially PUVA cream therapy or PUVA bath therapy.

Internal therapy
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In case of resistance to external therapy including local UV-radiation, systemic therapy with retinoids, in this case with neotigason, has proven to be effective.

Alternatively, treatment with fumaric acid esters (see there) according to the usual dosage scheme is recommended.

Alternative: Use of biologicals (see below psoriasis vulgaris)

Progression/forecast
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Recurrent course for years.

Naturopathy
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In addition to the conventional medicine therapy options, a desiccative therapy, e.g. with oak bark extracts or witch hazel, can be used. Occasionally a clear improvement is described under acupuncture treatment.

Note(s)
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  • The term"Pustulosis palmaris et plantaris" is often used synonymously with Pustulosa palmo-plantaris psoriasis.
  • To what extent the so-called pustular bacteride Andrews (see below,bacteride Andrews pustulöses) is also to be considered as a pustular variant of pustulosa palmaris et plantaris remains to be investigated further.
  • Pustulosis palmo-plantaris shows an increased association with arthritis. See SAPHO syndrome. The criteria of this "umbrella syndrome - SAPHO" are defined as sterile osteitis (blanc osteomyelitis), spondylitis and/or arthritis, which can occur with or without skin changes. Within SAPHO, various associations and overlaps are possible, whereby the cutaneous-osseous association is not an obligatory but a characteristic phenomenon.

Literature
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  1. Bacharach-Buhles M et al (1993) The pustular bacterid (Andrews).
    Are there clinical criteria for differentiating from psoriasis pustulosa palmariset
    plantaris? dermatologist 44:221-224
  2. Barber HW (1936) Pustular psoriasis of the extremities. Guy's Hospital Reports (London) 86: 108-119
  3. de Waal AC, van de Kerkhof PC (2011) Pustulosis palmoplantaris is a disease distinctfrom
    psoriasis. J Dermatologist Treat 22:102-105
  4. Hayashi M et al (2009) Pathogenic role of tonsillar lymphocytes in associated with HSP60/65 in Pustulosis palmaris et plantaris. Auris nasus larynx 36:578-585
  5. Hellgren L et al (1971) Pustulosis palmaris et plantaris. Prevalecne, clinical obervations and prognosis. Acta Derm Venereol 51:284-288
  6. Kasche A et al (2007) Severe psoriasis pustulosa palmaris et plantaris (Barber-Königsbeck) treated successfully with soluble tumour necrosis factor receptor fusion protein (etanercept). J Eur Acad Dermatol Venereol 21:255-257
  7. Königsbeck M (1917) About strongly exudative psoriasis with pustular formation, especially of the hands, feet and nails. Inaugural dissertation, Munich
  8. Mössner R et al (2015) Palmoplantar Pustular Psoriasis Is Associated with Missense Variants in CARD14, but Not with Loss-of-Function Mutations in IL36RN in European Patients. J Invest Dermatol 135:2538-2541.
  9. Murakami M et al (2011) Patients with palmoplantar pustulosis have increased IL-17 and IL-22 levels both in the lesion and serum. Exp Dermatol 20:845-847
  10. Touma Z et al (2007) Long-delayed onset of chest wall pain defining a patient with SAPHO syndrome. J Clin Rheumatol 13:338-340

  11. Uzun G et al (2013) Pustulosis palmaris et plantaris. BMJ Case Rep doi: 10.1136/bcr-2013-009400

  12. Watanabe T et al (2014) Pustulosis palmaris et plantaris successfully treated with leukotriene antagonist. Eur J Dermatol 24:695-696

  13. Weißenseel P et al (2016) Psoriasis pustulosa. Dermatologist 67: 245-253

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 18.12.2020