Pustular psoriasis palmaris et plantaris L40.3

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.

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.

The pathogenesis of PPP is unclear.

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

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

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

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

Gluten-sensitive enteropathy: a link has been postulated; however, this requires confirmation.

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

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

• On palms and/or soles, there are whitish or white-yellowish opacified, sterile minute pustules on sharply demarcated erythema or red non-scaling plaques (predominantly pustular type).
• In addition to these rather monomorphic clinical pictures, there are variants of PPP with markedly polymorphic changes with a colorful coexistence of erythema, dyshidrotic vesicles, pustules and scaly papules or plaques (mixed pustular-hyperkeratotic type).

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

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.

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)

Recurrent course for years.

• 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.

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 Dermatolog 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 tumor necrosis factor receptor fusion protein (etanercept). J Eur Acad Dermatol Venereol 21:255-257.
7. Königsbeck M (1917) On severely exudative psoriasis with pustule 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. Dermatol 67: 245-253

14. Krieg PH et al (1992) The pustule in palmoplantar psoriasis: transformed vesicle or mature microabscess? A three-dimensional study. Dermatology 185:104-112