Erythema gyratum repens

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Huda A. Karman, M.D.

Synonyms and Keywords: Gammel's disease


Overview

Erythema gyratum repens is a rare highly specific and characteristic paraneoplastic syndrome that usually affect older people. It is characterized by wood-grain scaly skin eruption with intense pruritus. The cause of erythema gyratum repens is unknown but many theories suggest immunologic etiology or toxicologic products that are released by the associated tumor. The first case of erythema gyratum repens was described by a dermatologist named Gammel in the year 1952. For many years after erythema gyratum repens original description, there was little progress in defining the pathogenesis of erythema gyratum repens. Erythema gyratum repens has no specific classification but we can classify it based on its association with an internal malignancy into para-neoplastic and non-para-neoplastic erythema gyratum repens. The most common malignancies associated with erythema gyratum repens are lung or bronchogenic cancer, esophageal cancer, and breast cancer. Erythema gyratum repens can also be associated with non-neoplastic diseases as tuberculosis, autoimmune disorders, or CREST syndrome. Erythema gyratum repens is characterized by its pathogonomic figurate, gyrate, or annular erythematous skin eruptions. The intense pruritus can be debilitating and usually urges the patient to go to the emergency department. The microscopic histopathological features of erythema gyratum repens consist of acanthosis, focal parakeratotic, and spongiosis of the epidermis with perivascular mononuclear, lymphocytic, and histiocytic infiltrate in the superficial plexus of the dermis. Erythema gyratum repens is very rare and it mainly affects people in their seventieth decade, the male to female ratio is 2:1. Erythema gyratum repens is diagnosed clinically by its characteristic skin eruption and an urgent thorough paraneoplastic workup should be initiated to look for internal malignancies. Patients with erythema gyratum repens presents with intensely pruritic, gradually progressive, skin lesions that crawl rather than migrate from one body region to the other. It can start in the upper trunk or upper back and extends to involve the extremities sparing the face. The mainstay of the treatment of erythema gyratum repens is finding and treating the underlying malignancy. Symptomatic treatment is not very effective in relieving the pruritus and its associated pain. The management can be surgical removal of the tumor, chemotherapy, or palliative conservative management. The skin eruptions can improve completely after the removal of the underlying tumor, or can recur especially if the tumor recurred or metastasized. Patients can live a few weeks, months or up to five years depending on when and at what stage the malignancy was detected.

Historical Perspective

Classification

Types of Erythema gyratum repens Characterestics
Paraneoplastic EGR
Non-paraneoplastic EGR Idiopathic EGR
  • Erythema gyratum repens with no underlying malignancy, associated conditions, or precipitating cause
|EGR-like eruptions [8]
EGR with concomitant skin disease
Drug-induced EGR

Pathophysiology


Causes

Differentiating Erythema Gyratum Repens from Other Diseases

Types examples
With underlying malignancy [14][15]
  • Erythema gyratum repens (EGR)
Without underlying malignancy
  • Erythema marginatum rheumaticum


Disease Erythema Characteristics Signs and Symptoms Associated Conditions Histopathology Lab finding

& Other evaluation

Prognosis
Erythema gyratum repens (EGR)
  • Migratory annular and configurate erythematous bands that form concentric rings
  • Wood grain scaly appearance
  • scales follows the leading edge of the bands
  • Eruption migrates more rapidly, 1cm/d

(Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)


  • Skin manifestations can be improved within 48 hours of the resection of the underlying tumor with on of the following:
    • Complete cure of the skin eruption and pruritus
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis)
    • No effect of the tumor treatment on the course of EGR
      • Death can occur few weeks after the detection of the malignancy, few months, or four years as in Gammel's patient.
Erythema annulare centrifugum (EAC) [14]
  • Migratory annular and configurate erythematous

or polycyclic lesions

  • Eruption migrate at a slower rate (2 -3 mm/d) reaching up to 10 cm in diameter with central clearing
  • Cover only a small percentage of the total body surface   
  • Annular or polycyclic lesions which may begin as urticaria-like papule
  • Eventually old lesions can spontaneously resolve in several days to a few weeks while new eruptions develop.
  • The deep form of erythema annulare centrifugum has a firm, indurated border, is rarely pruritic, and has no scale
  • The superficial type of erythema annulare centrifugum has an indistinct scaly border and is usually pruritic  
  • No specific laboratory changes
  • Lesions disappear after the underlying etiology is managed (allergy, infection, malignancy)
  • if no underlying cause, lesions can recur after discontinuation of the supportive treatment
Necrolytic migratory erythema (NME)
  • Due to the difficulty of necrolytic migratory erythema recognition, and its association with glucagonoma, diagnosis is usually delayed
  • Necrolytic migratory erythema usually resolved after the resection and treatment of the pancreatic tumor, eg.10 days after tumor resection
  • Early recognition is crucial for better diagnosis and prognosis

Epidemiology and Demographics

Age

  • The average age of onset of erythema gyratum repens i is in the seventh decade of life (65 years old).

Gender

  • The male to female ratio is 2:1.

Race

  • EGR commonly affects Caucasians.

Risk Factors

Screening

Natural History, Complications, and Prognosis

  • The majority of patients with erythema gyratum repens presents with severely pruritic erythematous skin lesions that appear several months prior to the malignancy diagnosis[13]
  • If the underlying malignancy left untreated, the debilitating pruritus could persist until the patient dies[13]
  • Prognosis depends on the type of the underlying tumor and the probability of its treatment. It depends on the time of the erythema gyratum repens onset and the neoplasm discovery. The course and prognosis of erythema gyratum repens can be one of the following:
    • Complete cure of the skin eruption and pruritus after removal and treatment of the internal neoplasm.
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis).
    • No effect of the tumor treatment on the course of erythema gyratum repens.
    • Death can occur few weeks after the discovery of the malignancy, few months, or four years as in Gammel's patient.

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. Rothman, Stephan (1925). "Über Hauterscheinungen bei bösartigen Geschwülsten innerer Organe". Archiv für Dermatologie und Syphilis. 149 (1): 99–123. doi:10.1007/BF02297811. ISSN 0340-3696.
  2. Burgdorf WHC, Bickers DR (2015). "The scientific legacy of Stephen Rothman". J Invest Dermatol. 135 (4): 954–959. doi:10.1038/jid.2014.447. PMC 4366295. PMID 25373439.
  3. 3.0 3.1 3.2 Gammel, John A. (1952). "ERYTHEMA GYRATUM REPENS". A.M.A. Archives of Dermatology and Syphilology. 66 (4): 494. doi:10.1001/archderm.1952.01530290070010. ISSN 0096-5979.
  4. Purdy, M. J. (1959). "Erythema Gyratum Repens". A.M.A. Archives of Dermatology. 80 (5): 590. doi:10.1001/archderm.1959.01560230076020. ISSN 0096-5359.
  5. 5.0 5.1 Skolnick, Marvin (1975). "Erythema Gyratum Repens With Metastatic Adenocarcinoma". Archives of Dermatology. 111 (2): 227. doi:10.1001/archderm.1975.01630140085011. ISSN 0003-987X.
  6. Boyd AS, Neldner KH, Menter A (1992). "Erythema gyratum repens: a paraneoplastic eruption". J Am Acad Dermatol. 26 (5 Pt 1): 757–62. PMID 1583177.
  7. 7.0 7.1 7.2 Rongioletti, F.; Fausti, V.; Parodi, A. (2014). "Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience". Journal of the European Academy of Dermatology and Venereology. 28 (1): 112–115. doi:10.1111/j.1468-3083.2012.04663.x. ISSN 0926-9959.
  8. 8.0 8.1 Fukunaga M, Harada K, Mae K, Wakamatsu K, Kiriyama N, Tsuboi R; et al. (2017). "Erythema Gyratum Repens-Like Purpura in a Patient with Sjögren Syndrome". Case Rep Dermatol. 9 (2): 40–43. doi:10.1159/000477375. PMC 5498950. PMID 28690517.
  9. 9.0 9.1 Günther R, Nasser S, Hinrichsen H, Fölsch UR (2002). "[Erythema gyratum repens: drug reaction following azathioprine administration in a patient with type I [[autoimmune]] [[hepatitis]]". Med Klin (Munich). 97 (7): 414–7. PMID 12168480. URL–wikilink conflict (help)
  10. 10.0 10.1 Rongioletti, Franco; Fausti, Valentina; Parodi, Aurora (2012). "Erythema Gyratum Repens Induced by Pegylated Interferon Alfa for Chronic Hepatitis C". Archives of Dermatology. 148 (10): 1213. doi:10.1001/archdermatol.2012.1968. ISSN 0003-987X.
  11. Samotij D, Szczech J, Bencal-Kusinska M, Reich A (2016). "Erythema gyratum repens associated with cryptogenic organizing pneumonia". Indian J Dermatol Venereol Leprol. 82 (2): 212–3. doi:10.4103/0378-6323.173594. PMID 26765132.
  12. Appell ML, Ward WQ, Tyring SK (1988). "Erythema gyratum repens. A cutaneous marker of malignancy". Cancer. 62 (3): 548–50. doi:10.1002/1097-0142(19880801)62:3<548::aid-cncr2820620318>3.0.co;2-h. PMID 3390794.
  13. 13.00 13.01 13.02 13.03 13.04 13.05 13.06 13.07 13.08 13.09 13.10 13.11 Gore M, Winters ME (2011). "Erythema gyratum repens: a rare paraneoplastic rash". West J Emerg Med. 12 (4): 556–8. doi:10.5811/westjem.2010.11.2090. PMC 3236141. PMID 22224159.
  14. 14.0 14.1 14.2 14.3 14.4 14.5 14.6 14.7 14.8 14.9 Tyring SK (1993). "Reactive erythemas: erythema annulare centrifugum and erythema gyratum repens". Clin Dermatol. 11 (1): 135–9. PMID 8339188.
  15. 15.0 15.1 Holt PJ, Davies MG (1977). "Erythema gyratum repens--an immunologically mediated dermatosis?". Br J Dermatol. 96 (4): 343–7. PMID 861171.
  16. Ridge A, Tummon O, Laing M (2019). "Response to "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases"". JAAD Case Rep. 5 (5): 461–462. doi:10.1016/j.jdcr.2019.03.012. PMC 6510971 Check |pmc= value (help). PMID 31111084.

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