Psoriasis erythema anulare centrifugum-like L40.8

Last updated on: 29.10.2020

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Synonym(s)

Anular pustular psoriasis; Circinar generalized pustular psoriasis; EACP; Erythema annulare centrifugum-like psoriasis cum pustulatione; Erythema anulare centrifugum Degos; Erythema anulare centrifugum-like psoriasis; Erythema circinatum recidivans; pustular psoriasis

History
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Milan and Katchoura, 1932

Definition
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Rare, mild special form of pustular psoriasis generalisata (Zumbusch type) with the formation of characteristic garland-shaped (circulatory) skin patterns that are anular and caused by confluence. In EACP, the general symptoms of pustular psoriasis are missing.

Manifestation
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While EACP is rare in adulthood, it is the most common pustular psoriasis variant in childhood.

Localization
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Extremities, gluteal inguinal and abdominal region. Face, palmae and plantae always remain free. Localized solitary variants have been described.

Clinical features
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Chronic generalized psoriasis, usually in relapses, characterized by its prominent anular formations. Depending on the stage of activity, the peripheral zones may be dry peeling or may be covered with pustules which are not bound to the follicle. The itching varies in intensity. The tendency towards central healing and a simultaneous peripheral progression leads to the characteristic ring-shaped and, in case of confluence, garland-like formations. Typical is the inward-directed scaling (Collerette scaling) of the anular foci. The foci heal with the formation of coarse lamellar (corneolytic) scaling.

Episodes can be associated with a general feeling of illness, fever and possibly athralgia.

Laboratory
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CRP and BSG increased. In the blood count leukocytosis with neutrophilia.

Histology
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Superficial dermatitis with subcorneal pustular formation

Therapy
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S.u. Psoriasis.

Systemic therapeutic agents of the first choice are REtinoids in the usual dosages.

Alternatively: Ciclosporin A, Methotrexate, Infliximab, Etanercept and Adalimumab

External: Glucocorticoids, PUVA therapy

Progression/forecast
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The disease can be triggered by infections, drugs (NSAIDs in combination with febrile bacterial infection) or hormonal factors. The erythema of anulare-like psoriasis can progress in phases over years. However, a single occurrence is also possible, even in the case of complications in previously known psoriasis vulgaris (e.g. through prolonged internal administration of glucocorticoids and their discontinuation - steroid withdrawal).

Literature
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  1. Albert A et al (2007) Erythema annulare centrifugum-like psoriasis cum pustulatione. dermatologist 58:769-773
  2. Casper U et al (1998) Exfoliatio areata linguae et mucosae oris: a mucous membrane manifestation of psoriasis pustulosa? dermatologist 49:850-854
  3. Milan G, Katchoura F(1932) Psoriasis pustuleux generalisé. Bull Soc Fr Dermatol Syph 40: 851-852
  4. Pfohler C et al (2013) Psoriasis vulgaris and psoriasis pustulosa - epidemiology, quality of life, comorbidities and treatment. Curr Rheumatol Rev 9:2-7
  5. Rotstein H (1996) Psoriasis: changing clinical patterns. Australas J Dermatol37 Suppl 1: 27-29
  6. Schaefer W et al. (2015) Erythematous Ülaques with centrifugal spreading, collerette-like scaling and pustules. J Dtsch Dermatol Ges 13:464-467
  7. Soto Lopes MS et al (2013) Paradoxical Reaction to Golimumab: Tumor Necrosis Factor α Inhibitor Inducing Psoriasis Pustulosa. Case Rep Dermatol 7:326-331

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Last updated on: 29.10.2020