Erythema anulare centrifugum L53.1

Colcott-Fox 1881; Darier 1916

Chronic, polyetiologic, inflammatory skin disease that has its clinical significance as a "reaction cutanée" or "indicator" dermatosis. Slowly centrifugally growing, annular, less frequently circular, red "palpable" plaques (no erythema) are characteristic. The entity of the clinical picture is controversial, especially since the view of this clinical picture has changed several times since it was first described by Darier. Jean Darier originally described the disease as a "self-limited, etiologically unexplained dermatosis, with anular foci spreading rapidly and resolving spontaneously after 1-2 weeks." Subsequent literature describes inconsistent, apparently reactive clinical pictures with and without scaling, some urticarial, some eczematous, some vesicular, with different histologic patterns presenting with superficial and deep lymphocytic (eosinophilic?) infiltrate.

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.

Ultimately, the etiopathogenesis of erythema anulare centrifugum (EAC) is unclear.

The association with:

• Malignant primary diseases (lymphoma, leukemia (especially chronic lymphocytic leukemia), breast carcinoma, ovarian carcinoma, bronchial carcinoma). The acronym "PEACE = paraneoplastic erythema anulare centrifugum" was coined for the paraneoplastic form of EAC (Chodkiewicz HM et al. 2012) .
• Infections (esp. candidiasis of the gastrointestinal tract, HIV, zoster),
• Autoimmunediseases (autoimmune hepatitis, polygandular autoimmune syndrome, Hashimoto's thyroiditis, Crohn's disease; sarcoidosis, polychondritis, lupus erythematosus).
• Medications (including amitriptyline, salicylates, finasteride, chloroquine, penicillin, diuretics, cimetidine)
• Food allergies(tomatoes, fish proteins, moulds contained in cheese).

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).

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

Multiple, initially homogeneous 1.0-2.0 cm red plaques that heal centrally and expand centrifugally. This results in ring formations up to 6.0 cm in diameter within a few weeks. These may coalesce in multiple arches.Typically, the plaques are smooth on the surface. In some cases, a finely lamellar scaly ruff is found at the inner edge of the ring.

Almost pathognomonic is the palpatory finding: when stroking from the center to the periphery of a focal area, the marginal rim feels like a "wet woolen thread" under the skin.

Notice. The term "erythema" in the naming is misleading, because it is about anular or also multi-arched narrow-banded (always palpable) plaques.

Furthermore, scaly - eczema-like, vesicular or teleangiectatic-purpuric, or pseudolymphoma-like special forms are described. The profound type lacks any epidermal involvement. The surface is smooth and scale-free. Here, a distinction must be made from both lupus erythematosus tumidus and pseudolymphoma.

A superficial type is distinguished from a profound type, and it is increasingly considered that these are 2 distinct entities.

• The superficial type shows a superficial perivascular and interstitial lymphocyte infiltrate with eosinophilic leukocytes in 1/3 of the cases , occasionally also neutrophilic granulocytes. Histoeosinophilia may also dominate the histological picture, and erythrocyte extravasations may be found in isolated cases. Exocytosis with focal spongiosis and parakeratosis is regularly detectable. Intraepidermal blistering is rare.
• The profound type shows dense, usually strictly perivascular infiltrative pods of lymphocytes in the mid and/or deep dermis; eosinophilic leukocytes in varying admixture in 1/3 of cases. The epithelium shows occasional vacuolar degeneration and dyskeratosis. Not infrequently, melanophages are encountered in the upper dermis. Spongiosis and parakeratosis are completely absent in this type. There are some authors who classify the profound type as an independent (deep figurate erythema) clinical picture.

• 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.

Focus search and remediation is in the foreground with elimination of gastric and intestinal disorders (including intestinal candidiasis, worm infections), foci of tonsils, teeth, gallbladder and adnexa. Thorough examination for visceral neoplasms (breast, larynx, lung, pancreas, ovary; see also paraneoplasia, cutaneous) and exclusion of myeloid diseases.

Possibly triggering drugs (see above) should be discontinued and foods such as fish or blue cheese should be avoided.

Typical, however, is a long-lasting, possibly years-long relapse activity. Spontaneous regression of a single foci is possible. Rarer are seasonal courses with occurrence in the summer months and spontaneous healing in the cold season (Haiges 2016).

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.

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