Pustular psoriasis L40.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

generalized pustular psoriasis; GPP; PPG; Psoriasis pustulosa gravis Zumbusch; Pustular psoriasis of the Zumbusch type; severe pustular psoriasis

History
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by Zumbusch, 1910

Definition
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Rare, acquired, occasionally also familial, severe, potentially life-threatening, pustular maximum variant of psoriasis characterized by a severe disturbance of the general condition. It is clinically characterized by countless, disseminated, sterile, white pustules on flat erythema, often confluent to pus lakes.

Classification
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The disease occurs in 3 subtypes:

  1. Sporadic PPG without symptoms of psoriasis vulgaris
  2. Sporadic PPG with symptoms of psoriasis vulgaris (30% of cases)
  3. Family PPG

Occurrence/Epidemiology
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Rare; prevalence: 0.1-0.9/100,000 inhabitants. Highly concentrated in Japan (incidence: about 0.7/100,000 inhabitants).

Etiopathogenesis
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Pustular psoriasis embodies a reaction type different from psoriasis vulgaris, which can occur with or without the precursor stage of psoriasis vulgaris. However, there are patients in whom both manifestations change. Psoriasis vulgaris can also suddenly acquire a pustular component (psoriasis cum pustulatione).

Infections: Trigger factors of pustular psoriasis generalisata pustulosa are bacterial and viral(Epstein-Barr virus infections) infections, drugs (antihypertensives, terbinafine, chloroquine), pregnancy (s. impetigo herpetiformis) or a sudden withdrawal of systemic glucocorticoid therapy. Rarely is there a paradoxical reaction under therapy with biologicals (P Weisenseel 2016).

Genetics: Rare cases of familial PPG are caused by mutations of the interleukin-36 receptor gene(IL-36RN), which encodes interleukin-36a. IL-36a is an antagonist of 3 proinflammatory interleukins of the IL-1 family (IL-36alpha, IL-36beta, IL-36gamma). Its dysfunction leads to a predominance of proinflammatory cytokines. Such mutations are also found in about 80% of sporadic cases without symptoms of psoriasis vulgaris. IL-36RN mutations are found in only 10% of PPG with psoriasis vulgaris. Another mutation associated with pustular psoriasis generalisata is the adaptor protein complex 1 (AP1S3). This variant leads to a destabilization and malfunction of the pattern recognition receptor TLR-3(toll-like receptor 3). Toll-like receptors are used for the recognition of so-called "Pathogen Associated Molecular Patterns" ( PAMPs ).

Manifestation
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Adults appearing after the 40th LJ. Rarely children.

Localization
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The entire integument with preference of the trunk and extremities, also palmae and plantae, oral mucosa, mucous membranes of the upper respiratory tract and genital mucous membranes can be affected.

In the area of the oral mucosa, acute stomatitis with fine-lamellar, wipeable zircine or oval plaques (stomatitis geographica, stomatitis areata migrans) is found, in the tongue area findings which can be characterized as exfoliatio areata linguae.

Clinical features
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Acute, "overnight" occurring complete or incomplete erythroderma accompanied by high fever and a severe feeling of illness with countless white or yellow sterile pustules, either standing alone or confluent to pus puddles. The pustules burst within a short time, dry out or leave behind extensive wetting areas.

If left untreated, a relapsing course is typical, which can last for weeks or months. The relapse frequencies vary in length and last for hours or days.

In the area of the oral mucosa there may be whitish or grey, veil-like, exfoliating plaques.

Laboratory
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BSG > 40 mm/hour; CRP > 50mg/l; leukocytosis mostly > 12,000/ul; neutrophilia often >80%; rarely hypoparathyroidism.

Histology
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Superficial perivascular and interstitial lymphocytic and leukocytic dermatitis with epidermotropy and spongiform, subcorneal or highly intraepithelial pustules, see also Kogoj pustules.

Differential diagnosis
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Complication(s)
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Bronchopneumonia, liver metabolic disorders, iron deficiency, renal insufficiency

General therapy
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  • The range of the clinical appearance is wide.
  • PPG must be classified as a life-threatening disease.
  • In the case of severe PPG, intensive care treatments in appropriately equipped therapy units apply.
  • Discontinuation of possible provocative medication.
  • Treatment of bacterial infections, focus search and remediation.
  • General measures: isolation of the patient, protective clothing / mouthguards for medical/nursing staff, monitoring, sufficient volume supply, fluid balancing, heat supply, exact temperature control, supply of humidity in the room air, special bed for decubitus prophylaxis / positioning on Metalline foil, bladder catheter, documentation of the findings.

Notice! Check intravenous accesses regularly and change daily (high risk of contamination)!

External therapy
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  • Apply a thin layer ofsulfadiazine-silver cream(e.g. Flammazine) to affected areas (erosions). Wound debridement, remove necrotic skin areas; puncture and open blisters sterilely. Gazette on erosive wound surfaces, if necessary with added antibiotics (e.g. Bactigras). Oral hygiene with astringent liquids (e.g. rp. 255, Hexoral Lsg.). Several times a day eye hygiene with disinfecting and astringent eye drops (e.g. Solan eye drops). In addition, apply a thick layer of eye ointment containing dexpanthenol. Loosen adhesions with a swab.
  • Treat pustulous skin with potent glucocorticoid for a short time like 0.1% betamethasone rp. 029 or 0.05% clobetasol cream rp. 054 . In groins and axillae 0.5% hydrocortisone zinc cream rp. 127 . After the pustular shearing activity has subsided, transition to a bland local therapy with hydrophilic ointments (e.g. Ungt. emulsif. aq.).

Radiation therapy
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After the acute phase has subsided, cautious PUVA therapy has proven to be effective, also as PUVA bath therapy. If necessary, experiment with selective UVB narrow band therapy (e.g. 311 nm).

Internal therapy
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  • In severe cases, fully balanced parenteral nutrition in the first days. Scheme with daily application of colloidal solution (1 ml/kg KG x affected KO), electrolyte solution (physiological saline solution 1 ml/kg KG x affected KO).
  • Antibiosis after antibiogram.
  • If the situation is stable, transition to high-calorie liquid food (e.g. meritene). Later diet with passed food; no spices, no fruit acids.
  • Successes with Infliximab (off-label use!) are described in individual cases. S.a.u. Psoriasis vulgaris.

    Notice!

    For this indication Infliximab is already included in the guidelines of the British Association of Dermatology! However, as a quick and reliable response to the therapy is absolutely essential, Infliximab is currently considered to be a "safe" therapy of the first choice.
  • Successes with acitretin (neotigason) 0.5-1.0 mg/kg bw/day p.o. have been reported (Remark: the therapeutic principle has not been sufficiently proven!) After the initiation phase, reduction to the lowest possible maintenance dose according to clinical practice. In case of therapy failure methotrexate (e.g. MTX) or fumaric acid ester (Fumaderm). Priority is given to methotrexate 15 - 25 mg/week i.v. because of faster onset of action!
  • Alternative: If necessary, a combination of methotrexate and fumaric acid esters is possible. After the acute attack has been intercepted, the therapy can be continued with fumaric acid esters alone if necessary.
  • Experimental and alternative: The use of interleukin-1-inhibitors (Gevokizumab) led in individual cases (2 patients) to a prompt response within a 4-week period!
  • Accompanying sedative antihistamine like promethazine 75-100 mg/day p.o. (e.g. Atosil Drg.) or Dimetinden 2 times/day 4 mg i.v. (e.g. Fenistil)

Comment: Internal glucocorticoids may be contraindicated from the etiological understanding of the disease. However, they are effective in a very acute initial period, both externally and internally (200-250mg prednisolone i.v.) and are suitable to bridge an acute initial phase!

Case report(s)
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See illustration above

Literature
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  1. Cassandra M et al (2003) Childhood pustular psoriasis elicited by the streptococcal antigen: a case report and review of the literature. Pediatric Dermatol 20: 506-510
  2. Hussain S et al (2015) IL36RN mutations define a severe autoinflammatory phenotype of generalized pustular psoriasis. J Allergy Clin Immunol 135:1067-1070
  3. Jiyad Z et al (2014) Generalized pustular psoriasis associated with Epstein-Barr virus. Clin Exp Dermatol 40:146-148
  4. Kim HS et al (2014) Two cases of generalized pustular psoriasis: successful treatment with infliximab. Ann Dermatol 26:787-788
  5. Mansouri B et al (2014) Treatment of two patients with generalized pustular psoriasis with the interleukin-1β inhibitor gevokizumab. Br J Dermatol 173: 239-241.
  6. Setta-Kaffetzi N ET AL: (2014) AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. At J Hum Genet 94:790-797.
  7. Shiratori T et al (2015) IL36RN gene analysis of two Japanese patients with generalized pustular psoriasis. Int J Dermatol 54:e60-62
  8. Sugiura K et al (2013) The majority of generalized pustular psoriasis without psoriasis vulgaris is caused by deficiency of interleukin-36 receptor antagonist. J Invest Dermatol 133:2514-2521
  9. Tholen S et al (1987) Oral mucosal changes in pustular psoriasis generalisata. Dermatologist 38: 419-421
  10. Vogelgsang L et al (2015) Generalized pustular psoriasis pustulosa: a rare entity. JDDG 13 (Suppl 1): 103
  11. Weißenseel P et al. (2016) Pustulous psoriasis. Dermatologist 67: 445-453
  12. by Zumbusch LR (1910) Psoriasis and pustular exanthema. Arch Dermatol Syphilol (Berlin) 99: 335-346

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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: 29.10.2020