Psoriasis erythema anulare centrifugum-like L40.8

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Franziska Welke

All authors of this article

Last updated on: 14.03.2022

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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 adults, it is the most common pustular psoriasis variant in children.

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

Clinical features
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Chronic, usually relapsing, generalized psoriasis characterized by its prominent anular formations. Depending on the stage of activity, the marginal zones may flake dry or be covered with non-follicular pustules. There is varying intensity of itching. The tendency to heal centrally and a simultaneous peripheral progression leads to the characteristic annular and, when confluent, to garland-like formations. The inward scaling (collerette scaling) of the anular foci is typical. The foci heal with the formation of a coarse lamellar (corneolytic) scaling.

Relapses may be associated with a general feeling of illness, fever and possibly also athralgias.

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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See below Psoriasis.

Systemic therapeutics of 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, medications (NSAIDs in combination with febrile bacterial infections) or hormonal factors. Erythema anulare-like psoriasis can occur in episodes over years. But also the single occurrence is possible, also complicative in case of a previously known psoriasis vulgaris (e.g. by a longer 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: 14.03.2022