Erythema anulare centrifugum L53.1

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. med. Gerret Paulsen

Our authors

Last updated on: 29.10.2020

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Synonym(s)

EAC; Erythema annulare centrifugum; Erythema figuratum perstans; Erythema gyratum perstans; Erythema perstans; Superficial or deep gyrate erythema

History
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Colcott-Fox 1881; Darier 1916

Definition
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Chronic, polyätiological, inflammatory skin disease, which has its clinical significance as a "reaction cutanée" or "indicative" dermatosis. Characteristic are slowly centrifugally growing, annular, more rarely circulatory, red "palpable" plaques (no erythema). The entity of the clinical picture is controversial, particularly as the understanding of this clinical picture has changed several times since Darier's first description. Jean Darier originally described the disease as "self-limited dermatosis, with anular foci which spread rapidly and regress spontaneously after 1-2 weeks". The following literature describes inconsistent, apparently reactive clinical pictures with and without scaling, partly urticarial, partly eczematous, partly vesicular with different histological patterns, which would have to present with superficial and deep lymphocytic infiltrate.

Classification
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Both from a clinical and histological point of view,:

  • superficial type
  • and
  • profound type.

Remark: It is doubtful whether the profound type is an independent entity. It is more likely that variants of other diseases have been described under this term (e.g. lupus erythematodes tumidus or pseudolymphomas). The descriptive term "deep gyriated erythema" chosen by some authors for this clinical picture is not very helpful for its etiopathic classification.

Etiopathogenesis
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Ultimately, the aetiopathogenesis of erythema anulare centrifugum is not clear.

Discussed is the association with:

  • Malignant underlying diseases (lymphomas, leukemias, breast carcinoma, ovarian carcinoma, bronchial carcinoma)
  • Infections (especially candidosis of the gastrointestinal tract, HIV, zoster),
  • Autoimmune diseases (autoimmune hepatitis, polygandular autoimmune syndrome, Hashimoto's thyroiditis, Crohn's disease; sarcoidosis, polychondritis, lupus erythematosus)
  • Medicines (including amitriptyline, salicylates, finasteride, chloroquine, penicillin, diuretics, cimetidine)
  • Food allergies(tomatoes, fish proteins, moulds contained in cheese).

Manifestation
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On average, patients in middle age fall ill (50.-55. LJ). Less frequently occurring in adolescents. Women are slightly more frequently affected (w:m = 5:4).

Localization
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The main areas affected are the trunk (50-60%), proximal limbs and the glutaeal region. The mucous membranes remain free of appearance!

Clinical features
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Multiple, initially homogeneous 1.0-2.0 cm red plaques, which heal centrally and expand centrifugally. This results in ring formations with a diameter of up to 6.0 cm within a few weeks. Typically, the plaques are smooth on the surface. In some cases, a fine-lamellar scaly ruff is found on the inner edge of the ring.

The palpation finding is almost pathognomonic: when stroking from the centre to the periphery of a stove, the wall at the edge feels like a "wet wool thread" under the skin.

Notice! The term "erythema" in the name is misleading, since it refers to anular or even multi-curve narrow-band (always palpable) plaques.

Scaly - eczema-like, vesicular or teleangiectatic-purple, or pseudo-lymphoma-like special forms are also described. The profound type lacks any epidermal involvement. The surface is smooth and free of scales. Here a distinction is to be made between lupus erythematosus tumidus and pseudolymphomas.

Histology
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A distinction is made between a superficial type and a profound type, with the increasing view that these are 2 different entities.

  • The superficial type shows a superficial perivascular and interstitial lymphocyte infiltrate with eosinophilic leukocytes in 1/3 of the cases, occasionally also neutrophil granulocytes. Occasionally also erythrocyte extravasates. Exocytosis with focal spongiosis and parakeratosis is regularly detectable. Intraepidermal blistering is rare.
  • The profound type shows dense, mostly strictly perivascularly arranged infiltrate sheaths of lymphocytes in the middle and/or deep dermis; in 1/3 of the cases eosinophilic leukocytes in different admixtures. The epithelium shows isolated vacuolar degeneration and dyskeratoses. Melanophages are often found in the upper dermis. Spongiosis and parakeratosis are completely absent in this type. There are some authors who classify the deep figurate erythema as an independent clinical picture.

Diagnosis
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Typical clinical picture with anular or figured plaques. Histology can be pioneering.

Differential diagnosis
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  • Clinical Differential Diagnosis:
    • Tinea corporis: Itchy, border-emphasized plaques; if not pretreated distinct scaling, no prominent marginal induration on palpation. Clinical and histological pathogen detection (e.g. PAS staining).
    • Anular urticaria: Large migration velocity of the urticae (the changes of the localization are detectable by pen marking and exclusion criterion for erythema anulare centrifugum); prominent to severe itching; light red color. Histology excludes the erythema anulare centrifugum.
    • Nummular eczema: Eczematous plaques without prominent marginal markings; mostly disseminated, no mycelia in the PAS preparation.
    • Seborrheic eczema: Difficult differential diagnosis in marginal plaques! The recurrent course of the disease is typical with aggravation in the winter months and possibly complete healing under summery, maritime climate.
    • Pityriasis rosea: It is assumed that a part of the so-called spongiotic type of Eac belongs to this disease pattern. Pityriasis rosea is not prone to anular configuration. Distribution pattern and analogous (stem emphasized). The erythema anulare centrifugum is not characterized by cleavage lines. Psoriasis vulgaris: Typical (annular) plaque psoriasis is a very important DD. The peak-out phenomenon is always negative in erythema anulare centrifugum! Anular (non-pustular) psoriasis has a significantly lower rate of progression (week-months) than the erythema anulare centrifugum (days to weeks).
    • Erythema-anulare centrifugum-like psoriasis: As the erythema anulare centrifugum anular configured, always detection of pustules (the Eac never shows pustules; exclusion criterion); mostly psoriasis history! Histology is diagnostic.
    • Parapsoriasis en plaques: No marginalization, usually no scaling, pseudoatrophy! No itching!
    • Mycosis fungoides (especially type pagetoid reticulosis): No stress on the edges; very slow growth over months or years! Little or no itching; histology is diagnostic!
    • Erythema gyratum repens (extremely rare!): Anular, garland-shaped or spirally intertwined (untypical for erythema anulare centrifugum), non-itching, slightly indurated plaques. Rapid change of the foci.
    • Erythema exsudativum multiforme: Initially high rate of progression (hours and days); speaks against erythema anulare centrifugum; shooting target configuration (absolutely untypical for erythema anulare centrifugum); often infestation of the mucous membranes (exclusion criterion for erythema anulare centrifugum).
    • Bullous pemphigoid: Initially high rate of progression (hours and days); speaks against eac; usually marked itching; laboratory (pemphigoid antibodies), histology and immunohistology are conclusive.
    • Granuloma anulare: reddish-brown in colour, plaques usually polycyclically limited (untypical for erythema anulare centrifugum), never scaly, histology is conclusive.
    • Eosinophilic cellulitis (rare): stage-dependent variability of the clinical picture (the erythema anulare centrifugum is morphologically stable).
    • Lupus erythematodes tumidus: Solid, homogeneous plaques, no anular structures, never scaly. DD less necessary from a clinical than from a histological point of view!
    • Erythema migrans (anamnesis, usually only single focus, serology...)
  • Histological differential diagnoses (often difficult and only to be made in connection with the clinical picture. Good clinical information is important).:
    • Acute and subacute eczema: Spongiosis, extensive parakeratosis. No strict perivascular accentuation.
    • Urticaria (acute or chronic): Only sparse (never prominent) perivascular oriented, mixed cell infiltrate of eosinophils, neutrophils and few lymphocytes. No epidermotropy; occasionally few perivascular erythrocytes.
    • Pityriasis rosea: Although clinically clearly different, the histopathological changes are largely identical. Differentiation only in connection with clinical picture!
    • Lupus erythematodes tumidus: Superficial and deep lymphocyte infiltrate, also in the vessel walls. No epidermal changes (also missing in the profound type of erythematosus anulare centrifugum). Usually no prominent eosinophilia! Histologically, profound type of erythema anulare centrifugum and lupus erythemaodes tumidus are often not reliably differentiable, but clinically almost always reliably!
    • Borrelia-associated pseudolymphoma: superficial and deep lymphocytic infiltrates, often with characteristic admixture of plasma cells. Possible arragenments of lymph follicles. No eosinophilia.
    • Psoriasis vulgaris: Mostly prominent acanthosis, extensive hyper- and parakeratosis with neutrophil inclusions. No eosinophilia!
    • Parapsoriasis en plaques: Fibrosis of the papillary stratum; rather atrophic surface epithelium; the perivascular accentuation of the infiltrate, typical for the erythema anulare centrifugum, is missing.
    • Early syphilis: interface dermatitis with psoriasiform epidermal reaction. Dense, band-shaped infiltrate in the upper and middle dermis consisting of lymphocytes, histiocytes and plasma cells). Extension of the infiltrate to the deep vascular plexus.

General therapy
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  • Focus search and rehabilitation is in the foreground with elimination of gastrointestinal disorders (including intestinal candidiasis, worm infections), foci on tonsils, teeth, gall bladder and adnexes. Thorough examination for visceral neoplasm (mamma, larynx, lung, pancreas, ovary; see also paraneoplasia, cutaneous) and exclusion of myeloid diseases.
  • Possibly triggering medication (see above) should be discontinued and food such as fish or blue cheese should be avoided.

External therapy
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Glucocorticoids usually show a low response, but cosmetically disturbing foci can be treated with glucocorticoid-containing creams or ointments like mometasone furoate (e.g. Ecural Fatty Ointment), if necessary under occlusion.

Internal therapy
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Glucocorticoids are only effective in higher doses and usually show recurrence after discontinuation.

Progression/forecast
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Typical, however, is a long lasting, possibly years lasting push activity. Spontaneous regression of a single nucleus is possible. More rare are seasonal processes with occurrence in the summer months and spontaneous healing in the cold season (Haiges 2016).

Case report(s)
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The 65-year-old patient noticed for about 3 weeks, first on the neck, later on the décolleté and the upper extremities, at first filled, slightly itchy, red plaques, which increased centrifugally and faded centrally, resulting in the formation of annular and, by confluence, also polycyclic formations. At the edges a parchment-like scaling was detectable.

The medical history was not very productive (no previous infections, no newly administered drugs in the last 3 months, no known tumor diseases). Considerable nicotine abuse.

Laboratory: Orientation laboratory o.B.

Histology: superficial perivascular and interstitial lymphocyte infiltrate with eosinophilic leukocytes; occasionally neutrophil granulocytes and erythrocyte extravasations. Focal exocytosis with spongiosis and parakeratosis.

Further findings: striking CT-thorax findings with a suspicious mass in the left lobe of the lung.

Ther: The patient strictly refused further therapeutic measures.

Literature
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  1. Batycka-Baran A et al (2015) Erythema annulare centrifugum associated with ovarian cancer. Acta Derm Venereol 95:1032-1033
  2. Bottoni U (2002) Erythema annulare centrifugum: report of a case with neonatal onset. J Eur Acad Dermatol Venereol 16: 500-503
  3. Chander R et al (2014) Systemic lupus erythematosus presenting as erythema annulare centrifugum. Lupus 23:1197-200
  4. Chodkiewicz HM et al(2012) Paraneoplastic erythema annulare centrifugumeruption: PEACE. At J Clin Dermatol 13:239-246.

  5. Darier (1916) De l'érythème annulaire centrifuge (érythème papulo-circineé migrateuse et chronique) et de quelques éruptions analogues. Annales de dermatologie et de syphilographie (Paris) 5: 57-58

  6. Gniadecki R (2002) Calcipotriol for erythema annulare centrifugum. Br J Dermatol 146: 317-319
  7. Haiges D et al. (2016) Erythema anuare centrifugum recurrently occurring or excercising in the summer months. Skin 16: 12-14
  8. Halevy S (2002) Autoimmune progesterone dermatitis manifested as erythema annulare centrifugum: Confirmation of progesterone sensitivity by in vitro interferon-gamma release. J Am Acad Dermatol 47: 311-313
  9. Ilkit M et al(2012)Cutaneous id reactions: a comprehensive review of clinical manifestations, epidemiology, etiology, and management. Crit Rev Microbiol 38:191-202.

  10. Imafuku K et al (2015) Erythema annulare centrifugum-like mycosis fungoides after unrelated bone marrow transplantation. Br J Haematol 170:140
  11. Ohmori S et al (2012) Erythema annularecentrifugum
    associated with herpes zoster. J UOEH 34:225-229.

  12. Vocks E (2003) Erythema annulare centrifugum-type psoriasis: a particular variant of acute-eruptive psoriasis. J Eur Acad Dermatol Venereol 17: 446-448

  13. Yaniv R et al (1993) Erythema annulare centrifugum as the presenting sign of Hodgkin's disease. Int J Dermatol 32: 59-61
  14. Weyers, W et al (2003) Erythema anulare centrifugum. At J Dermatopathol 25: 451-462
  15. Ziemer M et al (2010) Erythema anulare centrifugum. dermatologist 61: 967-972

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Last updated on: 29.10.2020