Erythema gyratum repens

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Erythema gyratum repens
SymptomsExpanding swirly wavy red lines, scale, itch[1]
Usual onsetOlder adults (60s)[1]
CausesCancer: lung cancer, oesophageal cancer, breast cancer[1]
Diagnostic method
Differential diagnosisNecrolytic migratory erythema, erythema migrans, erythrokeratodermia variabilis, subacute cutaneous lupus erythematosus, tinea corporis[1]
TreatmentTreat underlying cause[1]
MedicationAntihistamines[2]
PrognosisResolves with successful cancer treatment[1]
FrequencyRare, male:females (2:1)[1]

Erythema gyratum repens is a skin rash that has a strong association with internal cancers.[1] It characteristically presents with red wavy lines, generally in older adults.[1] These regular whirly rings rapidly and repetitively appear within existing ones, expanding outward at a rate of up to 1cm a day, giving the impression that the rash is moving.[3] The resulting pattern is similar to wood grain.[1] There is often an intense itch and scale over the leading edge, which may be slightly raised.[2] Around one in 10 affected individuals have thick skin of the palms of the hands.[1] In some, the skin may become extremely dry.[3]

The cause is believed to have an immunological base.[4] 80% of cases have an underlying cancer, of which almost half have lung cancer.[1] Other cancers reported to be associated include cancers of the oesophagus and breast, and less frequently gastric cancer, uterine cancer, throat cancer, pancreas cancer and lymphoma.[1] The rash generally precedes the cancer diagnosis by around 9-months.[2] Non-cancer associations include tuberculosis of the lung.[3] Sometimes no cause is found.[3]

Diagnosis is generally by its appearance, although tests may be required to exclude other conditions.[3] These tests may include blood tests such as a complete blood count, PSA, and antinuclear antibodies.[1] A cancer may be located using chest X-ray, mammogram, CT scan, and endoscopy.[1] Necrolytic migratory erythema, erythema migrans, tinea corporis, erythrokeratodermia variabilis, and subacute cutaneous lupus erythematosus are some of many other skin conditions that may appear similar.[1]

Treatment and outlook depend on the underlying cause.[1] Antihistamines may help to reduce the itch, although the role of applying a steroid cream is unclear.[2][5] The rash typically resolves with successful cancer treatment.[3]

The condition is rare.[1] Males are affected twice as frequently as females.[1] J. A. Gammel first described the condition in 1952, in an individual who was later found to have breast cancer.[6][7]

Signs and symptoms

Erythema gyratum repens characteristically presents as wavy red lines on the skin.[1] These regular whirly rings rapidly and repetitively appear within existing ones, expanding outward at a rate of up to 1cm a day, giving the impression that the rash is moving.[3] The resulting pattern is similar to wood grain.[1] There is typically an intense itch and scale over the leading edge, which may be slightly raised.[2] The trunk and limbs are most frequently affected.[5] Thickening of the skin of the palms co-exists in around 10% of affected individuals, whatever the underlying cause.[1] The skin may become extremely dry.[3] Onset is generally in older adults; after the age of 60-years.[1]

Cause

The cause is believed to have an immunological base.[4] 80% of cases have an underlying cancer, of which almost half have lung cancer.[1] Other cancers reported to be associated include cancers of the oesophagus, breast, stomach, uterus, throat, pancreas and blood.[1] The rash generally precedes the cancer diagnosis by around 9-months.[2] Less frequently, the cause may be tuberculosis of the lung, or no cause is found.[3] Other rare reported associations have included cryptogenic organizing pneumonia and rheumatoid arthritis.[2]

Diagnosis

Diagnosis is generally by its appearance.[3] Tests include blood tests such as a complete blood count which may reveal raised eosinophils.[2] Other blood tests include PSA, antinuclear antibodies and biochemistry.[1] Medical imaging may locate a cancer; chest X-ray, mammogram, CT scan of pelvis and abdomen.[1] If indicated then an endoscopy may be required; colonoscopy, gastroscopy.[1]

Differential diagnosis

Necrolytic migratory erythema, erythema migrans, tinea corporis, erythrokeratodermia variabilis, and subacute cutaneous lupus erythematosus are some of many other skin conditions that may appear similar.[1]

Treatment

Treatment and outlook depend on the underlying cause.[1] Antihistamines may help to reduce the itch, although the role of applying a steroid cream is unclear.[2][5] The rash typically resolves with successful cancer treatment.[3]

Epidemiology

The condition is rare.[1] Males are affected twice as frequently as females.[1]

History

J. A. Gammel first described the condition in 1952, in an individual who was later found to have breast cancer.[6][7]

See also

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 1.20 1.21 1.22 1.23 1.24 1.25 1.26 1.27 1.28 1.29 1.30 1.31 1.32 1.33 1.34 Griffiths, Christopher E. M.; Bleiker, Tanya O.; Creamer, Daniel; Ingram, John R.; Simpson, Rosalind C. (2022). "19. Reactive inflammatory erythemas". Rook's Dermatology Handbook. Hoboken: Wiley-Blackwell. p. 259. ISBN 978-1-119-42819-0. Archived from the original on 2023-08-11. Retrieved 2023-08-05.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "7. Erythema and urticaria". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. p. 144. ISBN 978-0-323-54753-6. Archived from the original on 2023-08-11. Retrieved 2023-08-07.
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 Bolognia, Jean L.; Schaffer, Julie V.; Duncan, Karynne O.; Ko, Christine (2021). "15. Figurate erythemas". Dermatology Essentials (2nd ed.). Elsevier. pp. 142–148. ISBN 978-0-323-70971-2. Archived from the original on 2023-08-08. Retrieved 2023-08-05.
  4. 4.0 4.1 Motta, Adriana; González, Luis Fernando; García, Gonzalo; Guzmán, Jennifer; Prada, Lorena; Herrera, Hugo; Rolon, Mariam (2022). "11. Inflammatory skin diseases presenting with erythema, urticaria and purpura". Atlas of Dermatology: Inflammatory, Infectious and Tumoral Skin Diseases. Springer. pp. 310–311. ISBN 978-3-030-84106-5. Archived from the original on 2023-08-08. Retrieved 2023-08-06.
  5. 5.0 5.1 5.2 "Erythema gyratum repens | DermNet". dermnetnz.org. Archived from the original on 8 August 2023. Retrieved 6 August 2023.
  6. 6.0 6.1 Boehner, Alexander; Neuhauser, Ruth; Zink, Alexander; Ring, Johannes (July 2021). "Figurate erythemas - update and diagnostic approach". Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology: JDDG. 19 (7): 963–972. doi:10.1111/ddg.14450. ISSN 1610-0387. PMID 34046996. Archived from the original on 2023-08-08. Retrieved 2023-08-05.
  7. 7.0 7.1 Gammel, J. A. (October 1952). "Erythema gyratum repens; skin manifestations in patient with carcinoma of breast". A.M.A. Archives of Dermatology and Syphilology. 66 (4): 494–505. doi:10.1001/archderm.1952.01530290070010. ISSN 0096-5979. PMID 12975861. Archived from the original on 2023-08-08. Retrieved 2023-08-05.

External links

Classification