Erythema gyratum repens L53.3

Rare clinical picture, now controversial as an entity, originally described as "obligate cutaneous paraneoplasia" in carcinomas of the mamma (6%), the female genital, the pharynx, the bronchi (in 50% of cases), the oesophagus or the stomach. The exanthematic skin changes can precede a tumor disease by 4-8 months (see below notes). In the meantime, the erythema gyratum repens has also been described in non-tumorous diseases (e.g. rheumatoid arthritis).

Unclear. Immunological reactions to various antigens (tumor products 70% of patients, infection-associated antigens, drugs/viral infections), which lead to the characteristic, but not specific, morphological pattern of the skin, are being discussed. Recently, cases have been associated with COVID-19 infection (Peres G et al. 2021; Castro Silva R et al. 2021) and SARS-CoV-2 vaccination (Chiquito D et al. 2022).

Occurring mainly in adults, usually 40th-60th l yr; m:f=2:1;

Annular, garland-shaped or spirally intertwined, slightly infiltrated erythema and plaques, always with accentuated edges. Possibly also urticarial, 1-2 cm wide, rapidly changing erythema strips with marginal, somewhat raised scaling.

The skin changes are often described as "wood grain-like" or as "zebra stripes (red and white zebra)", which would distinguish them from the clinically comparable changes of erythema anulare centrifugum . The extent to which this morphological peculiarity can be considered a specific feature of this disease remains questionable.

Furthermore, extensive, scaly erythema on the face, neck, hands and feet. Small patchy recurrences in the healed areas.

Some of the patients also had palmoplantar keratoses or acquired ichthyosis.

Hematoeosinophilia is detectable in 50% of cases.

Uncharacteristic perivascular infiltrates of lymphocytes, monocytes, histiocytes and sometimes also eosinophilic leukocytes. Apoptotic (dyskeratotic) epithelia can be detected in the surface epithelium. The keratinization is orthofocal parakeratotic. The histological picture corresponds to that of erythema anulare centrifugum.

In direct immunofluorescence, C3, C4, or IgG can occasionally be detected at the dermo-epidermal junction.

In a summary evaluation of the relevant literature with 112 cases of EGR, malignant internal neoplasms were detected in only 52% of the cases according to Rongioletti F et al (2014). 48% of the cases were evaluated as non-paraneoplastic. Thus, the classification of erythema gyratum repens as a morphologic entity and as an obligate paraneoplasia must be doubted. Rather, it has to be classified as a "non-specific réaction cutanée" to different systemic processes or as a particular morphological pattern of different figured skin diseases (intimal changes in cutaneous T-cell lymphoma or in pityriasis rubra pilaris - Almaani N et al. 2011) or as a drug reaction. Furthermore, EGR has been described in association with rheumatoid arthritis or also as para- or post-infectious (e.g. in pulmonary tuberculosis and leprosy).

The 65-year-old woman had noticed barely itchy, red plaques for 6 months, which developed within weeks from 0.5-1.0 cm large solid plaques to large anular or circinate, slightly infiltrated, linear plaques.

Based on the clinical picture, a suspected diagnosis of erythema gyratum repens was made. A subtle examination revealed no evidence of an internal malignancy. The laboratory was completely unremarkable.

Histology: Uncharacteristic perivascular infiltrates of lymphocytes, monocytes, histiocytes and scattered eosinophilic leukocytes. Focal parakeratosis.

Course: The HV regressed completely after 6 months of local therapy. A further clinical examination did not reveal any evidence of an internal tumor.

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