Erythema gyratum repens
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Gammel's disease.
Overview
Erythema gyratum repens (EGR) is a rare dermatologic disorder associated with a variety of internal malignancies (paraneoplastic cutaneous syndrome). The most common malignancies EGR associated with are Lung, esophageal, and breast cancers. EGR is a systemic disease and it has a characteristic generalized rash [1] Although EGR usually precedes and associated with visceral malignancies, Literature has shown 8 cases of transformation of pityriasis rubra pilaris (PRP) to ERG-like eruption and none of these cases was associated with internal malignancy [2] Histologically, EGR displays hyperkeratosis, parakeratosis, spongiosis, acanthosis, and a superficial perivascular inflammatory infiltrate [3].
Historical Perspective
Erythema gyratum repens was first described in 1952 by a dermatologist, Dr. John A Gammel, in a patient with metastatic breast cancer. The patient was a 55 year old woman with skin eruption for nine months, associated with poorly differentiated adenocarcinoma of the breast. The skin lesions started in the face and neck as erythema and scales lesions that clear while other lesions flared.
In the twentieth century, Dr. Gammel has been trained that there is a possible association between atypical, bizarre, or recalcitrant skin lesions and internal/visceral malignancies.
cases with EGR has been seen in patients with metastatic adenocarcinoma (61 year old woman, 6months history of generalized rash), and cholangiosarcoma (77 year old woman, 3 weeks history of lower limb rash).
Classification
There is no established system for the classification of EGR.
Pathophysiology
The cause of EGR has not been identified. However, there are theories that various immunologic mechanisms have been implicated in its pathogenesis. The first theory is that the tumor may induce antibodies that cross-react with the basement membrane of skin. The second theory is hat the tumor may produce polypeptides that bind skin antigens and render them immunogenic. Other researchers believe that deposition of tumor antigen-antibody complexes onto the basement membrane accounts for this reactive dermatitis.
Causes
The cause of erythema gyratum repens has not been identified.
Differentiating ((Page name)) from Other Diseases
EGR has a narrow differential diagnosis and given its distinctive appearance, it has to be differentiated from those with gyrate erythematous eruptions, such as, necrolytic migratory erythema (NME), erythema annulare centrifugum (EAC), and erythema migrans. Necrolytic migratory erythema (NME) is usually seen in association with pancreatic glucagonomas and favors the perioral and perianal areas. Erythema annulare centrifugum (EAC) has pruritic concentric lesions with central clearing and a trailing edge of scale but usually involves smaller areas of the trunk and extremities and it migrates at a much slower rate than the lesions of EGR. Erythema migrans is the lesion of Lyme disease, it has no scaling and is a more localized skin reaction.
Epidemiology and Demographics
EGR commonly affects Caucasians, with an average age of onset in the seventh decade of life (an average age of 63 years), and the male to female ratio is 2:1. EGR is usually precedes the diagnosis of the associated cancer by several months. EGR is mainly seen in patients with paraneoplastic syndrome, it can rarely be seen in patients with nonneoplastic conditions such as tuberculosis, hypereosinophilic syndrome, bullous pemphigoid vulgaris, systemic lupus erythematosis, and ulcerative colitis.
Risk Factors
There are no established risk factors for EGR.
Screening
There is no screening tests for EGR but the skin rash shouldn't be missed in the the ED and patients should be referred for urgent evaluation and screening for internal malignancies.
Natural History, Complications, and Prognosis
Patients with EGR usually presents with the severely pruritic rash few months prior to the diagnosis of the internal malignancy. The pruritus can be debilitating and it may persist to the time of death.
Diagnosis
Diagnostic Study of Choice
EGR is mainly diagnosed clinically. Eosinophilia is observed in 60% of cases and immunofluorescence shows patterns of IgG, C3, and C4 at the basement membrane.
History and Symptoms
The hallmark of EGR is skin rash and pruritus is almost a universal symptom that can be extreme and debilitating. Patient may also complain of weight loss, anorexia, fatigue, and fever.
Physical Examination
Patients with EGR presents with a rash consisting of wavy erythematous concentric bands that can be figurate, gyrate, or annular. The bands are arranged in parallel rings and lined by a fine trailing edge of scale, a pattern often described as “wood grained. The rash typically involves large areas of the body but tends to spare the face, hands, and feet and it can expands as fast as a cm a day. Bullae can also form from within the areas of erythema.
Laboratory Findings
There are no diagnostic laboratory findings associated with EGR.
Electrocardiogram
There are no ECG findings associated with EGR.
X-ray
There are no x-ray findings associated with EGR.
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with EGR.
CT scan
CT findings could be seen in the associated visceral malignancy in EGR.
MRI
There are no MRI findings associated with EGR.
Other Imaging Findings
Other Diagnostic Studies
Although skin appearance is characteristic, EGR has nonspecific histopathologic features. Biopsy specimens display acanthosis, mild hyperkeratosis, focal parakeratosis, and spongiosis confined to the epidermis and superficial dermis. Mononuclear, lymphocytic, and histiocytic perivascular infiltrate in the superficial plexus can also be seen. Basement membrane deposits of IgG, C3, or C4 have been noted under direct immunofluorescence in some cases.
Treatment
Various dermatologic and immunosuppressive therapies have been used to treat EGR. Systemic steroids are frequently ineffective. Topical steroids, vitamin A, and azathioprine have also failed to relieve skin manifestations. Improvement or resolution of EGR, and its associated intense pruritus, depends on recognition and treatment of the underlying malignancy. In patients with widely metastatic disease, the response of EGR to chemotherapy is variable. In such cases, patients may not experience resolution of the rash until just before the time of death, a time of significant immunosuppression
Medical Therapy
There is no treatment for EGR]; the mainstay of therapy is underlying malignancy.
Surgery
Surgical intervention is not recommended for the management of EGR.
Primary Prevention
There are no established measures for the primary prevention of EGR.
Secondary Prevention
There are no established measures for the secondary prevention of EGR.
References
- ↑ 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 PMID: 22224159 Check
|pmid=value (help). - ↑ Richey PM, Fairley JA, Stone MS (2018). "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases". JAAD Case Rep. 4 (9): 944–946. doi:10.1016/j.jdcr.2018.07.009. PMC 6191946. PMID 30345340.
- ↑ Richey PM, Fairley JA, Stone MS (2018). "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases". JAAD Case Rep. 4 (9): 944–946. doi:10.1016/j.jdcr.2018.07.009. PMC 6191946. PMID 30345340.