Erythema gyratum repens L53.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Gammel`s disease; gyrate erythema; wood-grain erythema

History
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Gammell, 1953

Definition
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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).

Etiopathogenesis
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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.

Manifestation
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Occurs mainly in adults, mostly 40 to 60 LJ.

Localization
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Trunk, proximal extremities.

Clinical features
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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.

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

Differential diagnosis
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Erythema anulare centrifugum.

Therapy
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Tumor search and cleanup.

External therapy
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If necessary, topical glucocorticoids, e.g. glucocorticoid lotio such as 0.05% betamethasone valerate (e.g. Betnesol V, R030 ) or 0.1% triamcinolone acetonide cream(e.g. Triamgalen, R259 ).

Note(s)
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According to Rongioletti F et al. (2014), a summary evaluation of the relevant literature with 112 cases of EGR revealed malignant internal neoplasias in only 52% of cases. 48% of the cases were evaluated as non-paraneoplastic. The classification of the erythema gyratum repens as morphological entity and as obligatory paraneoplasia must therefore be doubted. Rather, it is to be classified as a "non-specific réaction cutanée" on different system processes or as a special morphological pattern of different figured skin diseases (intimate changes in cutaneous T-cell lymphoma or in pityriasis rubra pilaris - Almaani N et al. 2011) or as drug reaction. Furthermore, the EGR was described in association with rheumatoid arthritis or as para- or post-infectious (e.g. in pulmonary tuberculosis and leprosy).

Case report(s)
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  • 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.

Literature
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  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

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Last updated on: 29.10.2020