Pustulose acute generalized exanthematic L27.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.02.2021

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Synonym(s)

Acute generalized exanthematic pustular dermatitis; Acute generalized exanthematic pustulosis; Acute generalized exanthematous pustulosis; Acute generalized pustular bacterium; Acute generalized pustulosis; AGEP; Dermatitis acute generalized exanthematic pustular; PEAG; Pustular drug rush; pustulodermic toxin; Pustuloses exanthématique aiguës généralisés; Toxic Pustuloderm

History
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MacMillian, 1973; Tan, 1974; Beylot, 1980

Definition
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Acute, generalized, often febrile, non-follicular, sterile, pustular exanthema, occurring in connection with infections and/or medication, which is always accompanied by marked leukocytosis and neutrophilia and has a tendency to heal spontaneously.

Occurrence/Epidemiology
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The incidences are given as 0.1-0.5/100,000 inhabitants/year.

Women seem to be affected slightly more often than men.

Etiopathogenesis
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Manifestation
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A special age disposition does not exist. Children are affected less often than adults.

Localization
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Typical is the infestation of the large flexures (axillary, submammary, inguinal region); but generalized infestation is also possible. AGEP can be accompanied by oedema of the facial skin.

Clinical features
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Beginning of the disease with significant impairment of the general condition; often feverish course with temperatures > 38°C.

Typical is the onset in the face and the large joint bends with disseminated, synchronous and relapsing laminar erythema or red swellings on which multiple, constricted, about 0.1 cm large non follicular pustules form. These flow together to form large pustule lakes, whereby the tender cover of pustules can be pushed away from their lower surface (Nikolski phenomenon) and lie on top like a crumpled damp cloth. The pustular exanthema is accompanied by itching or even stinging or burning pain (like a needle prick).

In some cases erythema exudativum may develop multiforme-like cocard-like patterns in which the pustules are usually arranged edge-on, either disseminated or grouped. The pustules exist for a few days; they heal with a racy, raised scaling of thin horny films (see figure).

Involvement of mucous membranes close to the skin (oral mucosa, genitals) is rather rare; if present, clinically mild exfoliating enanthema can be observed.

Notice! The coarse lamellar corneolytic desquamation is diagnostically indicative of a subcorneal pustulation!

In case of an atypical course, a clinically evident pustular formation may be missing in this clinical picture. In this case it is only visible in the histological preparation; but even in these cases a lesional corneolytic scaly ruffle is found when healed.

Laboratory
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ESR elevation, CRP elevation, always marked leukocytosis (leukocytes > 10,000-12,000/mm³; sine qua non), marked neutrophilia, usually mild to marked relative lymphopenia, possibly eosinophilia. Elevations of transaminases and alk. phosphatase.

Histology
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  • Exocytosis with intraepidermal, directly subcorneal accumulations of neutrophilic granulocytes.
  • Formation of spongiform to single-chambered pustules.
  • Focal loss of the str. granulosum. Mostly orthokeratotic epithelium. Occasionally there are also admixtures of eosinophilic granulocytes and erythrocyte extravasations.
  • Pattern: Superficial perivascular and diffuse neutrophilic dermatitis with subcorneal pustular pustular formation
  • In contrast to TEN (subepidermal cleft formation with necrotic epidermis), the sample biopsy shows subcorneal pustules in pustulosis acuta generalisata.

Direct Immunofluorescence
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Immunohistology: Immunoglobulins (IgG) and complement factors (C3) in the vessel walls of the capillaries of the stratum papillare and in the basement membrane zone

Diagnosis
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The "AGEP validation score" from the EuroSCAR study group (Sidoroff et al. 2001) is helpful for the validation of the diagnosis. A score of 8-12 pts speaks for the diagnosis:

AGEP validation score of EuroSCAR study group (Sidoroff A et al. 2001)

Symptom Score

  • Pustule
    • Typical +2
    • Compatible +1
    • Non-compatible 0
  • Erythema
    • Typical +2
    • Compatible +1
    • Non-compatible 0
  • Distribution patterns
    • Typical +2
    • Compatible +1
    • Non-compatible 0
  • Postpustular desquamation
    • Typical +1
    • Non-compatible 0
  • Mucous membrane infestation
    • Yes +1
    • No 0
  • Acute course (<10 days)
    • Yes +1
    • No 0
  • Healing (<15 days)
    • Yes +1
    • No 0
  • Fever
    • Yes +1
    • No 0
  • Neutrophil leukocytosis (>7,000)
    • Yes +1
    • No 0

Differential diagnosis
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  • Clinical differential diagnoses:
  • Histological differential diagnoses:
    • Psoriasis pustulosa generalisata (no reliable histological differentiation; medical history)
    • Pustular tinea (subcorneal pustular formation possible; detection of hyphae in the PAS preparation; in the upper dermis dense diffuse, sometimes perivasally accentuated lymphocytic infiltrate with few neutrophils and eosinophil granulocytes)
    • Impetigo contagiosa (subcorneal pustule; detection of bacteria)
    • Impetiginized eczema (eczema picture with broad-based acanthosis and extensive parakeratosis, broad spongiosis; focal penetration of the epithelium with neutrophil granulocytes).

General therapy
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Stopping the triggering medication.

External therapy
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Drying measures with Lotio alba, in case of superinfection possibly addition of 3-5% Clioquinol R050, baths with addition of potassium permanganate (light pink), Betaisodona wound gauze or antibiotic treatment with e.g. Clioquinol cream(Linola-Sept). If necessary, storage on Metalline foil. In case of a pronounced inflammatory note, topical glucocorticoids such as betamethasone valerate (e.g. Betnesol V, R030, R029 ) or triamcinolone acetonide (Triamgalen, R259 ) may be necessary.

Internal therapy
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  • Healing is usually described 10-14 days after discontinuation of the triggering drug. Until then symptomatic therapy, e.g. with dimetinden (Fenistil Drg.),
  • Important: protein and electrolyte balancing.
  • An internal steroid medication can be initiated in the eruption phase of the disease: initial 250-150 mg prednisolon/day i.v.; depending on the acuity, reduce daily by 50 mg, oralise from 50 mg and reduce in 5 mg steps.

Progression/forecast
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Spontaneous healing after 10-28 days

Note(s)
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  • The disease also heals spontaneously after discontinuing the triggering medication. The extent to which glucocorticoid therapy is useful remains open at present.
  • In individual cases, a positive epicutaneous test against causative drugs can be obtained after the clinical symptoms have subsided (the non-irritative concentration of the drug in epicutaneous testing of drugs varies).

Case report(s)
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The 63-year-old, always skin-healthy patient had been suffering for 4 days from a trunk and limb seizing exanthema accompanied by malaise, loss of appetite and moderate fever (38.5 °C). This was preceded by a 2-week oral administration of Terbinafine due to Tinea unguium.
  • Findings: Patients in clearly reduced, feverish (38.7 °C) AZ. On the trunk and extremities in diffuse distribution dense exanthema of 0.2-0.5 cm large, red, partly isolated papules and plaques, partly confluent to larger areas. Particularly on the confluent plaques, isolated pustules of barely 0.1 cm in size, which in places have confluated to form larger pus puddles, can hardly be seen. Oral mucosa o.B. No hepatosplenomegaly, no lymph node swelling.
  • Histology: Intraepidermal, superficial, spongiform to single chambered pustules. Laboratory: CRP 65 mg/l; leucocytosis (16.400/µl), neutrophilia (87%), lymphopenia (1.200/ul), GGT(54 U/l).
  • Therapy: Systemic glucocorticoids (prednisolone 250 mg/day i.v. with dose reduction of 50 mg in decreasing doses. Additional sedative antihistamine ( Dimetinden 2 times/day 4 mg i.v.). Local therapy with thin application of Lotio alba or Lotio alba aquosa skin-coloured (NRF 11.22.).
  • Course: After 2 days of defibrillation, after 4 days of paling of the lesions, coarse lamellar desquamation first on the trunk and significantly later on the lower extremity. After 7 days CRP 35 mg/l, leucocytosis at 11,400 µ/l. After 14 days extensive restitutio. The patient was allowed to take lukewarm showers in each phase of the disease.

Literature
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  1. Beylot C et al (1980) Acute generalized exanthematous pustulosis. Semin Cutan Med Surg 15: 244-249
  2. Langner D et al (2011) Systemic therapy with Infliximab for severe pustular psoriasis pustulosa generalisata and psoriatic arthritis. Act Dermatol 35: 185-186
  3. Lin JH et al (2002) Acute generalized exanthematous pustulosis with erythema multiforme-like lesions. Eur J Dermatol 12: 475-478
  4. Macmillan AL (1973) Generalised pustular drug rash. Dermatologica 146: 285-291
  5. Nigen S et al (2003) Drug eruptions: approaching the diagnosis of drug-induced skin diseases. J Drugs Dermatol 2: 278-299
  6. Paulmann M, Mockenhaupt M (2015) Severe drug-induced skin reactions: clinical, diagnostic, etiology and therapy. JDDG 13: 625-643
  7. Pichler WJ et al (2002) Cellular and molecular pathophysiology of cutaneous drug reactions. At J Clin Dermatol 3: 229-238
  8. Rouchouse B et al (1986) Acute Generalized Exanthematous Pustular Dermatitis and Viral Infection. Dermatologica 173: 180-184
  9. Sidoroff A et al (2001) Acute generalized exanthematous pustulosis (AGEP)--a clinical reaction pattern. J Cutan Pathol 28:113-119.
    Sidoroff A et al. (2007) Risk factors for acute generalized exanthematous pustulosis (AGEP) - results of a multinational case -control study (EuroSCAR). Br J Dermatol 157: 989-996
  10. Sidoroff A et al. (2014) Acute generalized exanthematic pustulose. dermatologist 65:430-435
  11. Tan RS (1974) Acute generalized pustular bacterid. An unusual manifestation of leukocytoclastic vasculitis. Br J Dermatol 91: 209-215

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Last updated on: 19.02.2021