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).

Not clear. Discussed are, among other things, immunological reactions to various antigens (tumor products, infection-associated antigens, drugs), which lead to the described (not specific, but indicative) morphological pattern of the skin.

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

Anular, garland-shaped or spirally intertwined, slightly infiltrated, always borderline erythema and plaques. Possibly also urticarial, 1-2 cm wide erythema stripes rapidly changing their shape with marginal, somewhat raised scaling.

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

Furthermore flat, scaly erythema on face, neck, hands and feet. Small spotty recurrences in the cured areas.

According to Rongioletti F et al (2014), in a summary evaluation of the relevant literature with 112 cases of EGR, malignant internal neoplasms were detected in only 52% of the cases. 48% of the cases were evaluated as non-paraneoplastic. Thus, the classification of erythema gyratum repens as a morphological 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, the 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 hardly noticed any itchy, red plaques for 6 months, which developed within weeks from 0.5-1.0 cm large solid plaques to large-area anular or circulatory, 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 internal malignancy. The laboratory was completely unremarkable.
• Histology: Uncharacteristic perivascular infiltrates of lymphocytes, monocytes, histiocytes and isolated eosinophilic leukocytes. Focal parakeratosis.
• Course: After 6 months of blande local therapy the HV regressed completely. A new clinical examination did not show any evidence of an internal tumor.

1. Almaani N et al (2011) Erythema gyratum repens associated with pityriasis rubra pilaris. Clin Exp Dermatol 36:161-164.

2. Bryan ME et al (2003) Erythema gyratum repens associated with pityriasis rubra pilaris. J Drugs Dermatol 2: 315-317

3. Endo Y et al (2013) Erythema gyratum repens preceding the onset of rheumatoid arthritis. Eur J Dermatol 23:399-400

4. Galán-Gutiérrez M, Martínez-Peinado CM, Ruiz-Villaverde R. Erythema gyratum repens: Not always a paraneoplastic disease. Rev Clin Esp 214:425-427
5. Gammel JA (1953) Erythema gyratum repens. Arch Derm Syph 66: 494-505
6. High WA et al (2003) Superficial gyrate erythema as a cutaneous reaction to alendronate for osteoporosis. J Am Acad Dermatol 48: 945-946
7. Holcomb M et al (2012) Erythema gyratum repens-like eruptions with large cell transformation in a patient with mycosis fungoides. Int J Dermatol 51:1231-1233
8. Lo Schiavo A et al (2012) Erythema gyratum repens and rheumatoid arthritis: an unrecognized association? Indian J Dermatol Venereol Leprol 78:122
9. Nagase K et al (2014) CD4/CD8 double-negative mycosis fungoides mimicking erythema gyratum repens in a patient with underlying lung cancer. Acta Derm Venereol 94:89-90
10. Marchetti MA et al (2013) Pityriasis rubra pilaris treated with methotrexate resolving with an erythema gyratum repens-like appearance. J Am Acad Dermatol 69:e32-3

11. Rongioletti F et al (2014) Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience. J Eur Acad Dermatol Venereol 28:112-115.

12. Rongioletti F et al (2012) Erythema gyratum repens induced by pegylated interferon alfa for chronic hepatitis C. Arch Dermatol 148:1213-1214
13. Sohl S et al. (2009) Woodgrain-like exanthema with central fading at the lower extremity. SDDG 7: 261-262