Erythema gyratum repens
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Gammel's disease.
Overview
Historical Perspective
- In 1953, the dermatologist, Dr. John A Gammel who was trained to link skin lesions to internal malignancy was the first one who described and labeled Erythema Granulatum Repens in a 55-year-old patient with poorly differentiated breast adenocarcinoma [1]
- Up to 1992, there were only 49 cases in the literature, 41 of which (84%) were associated with a neoplasm [2]
- EGR is associated with internal malignancy in 82% of cases. However, between 1990 and 2010, data was collected from the medical records of patients form dermatology department in University of Genoa and from databases as pubmed and medline, the conclusion of this literature review was that EGR is no longer considered as an obligate paraneoplastic syndrome. More than expected cases of EGR were found with no neoplasm association. Non-paraneoplastic EGR could be: [3]
- Idiopathic EGR
- EGR-like eruptions (different dermatologic lesions that mimic EGR)
- Drug-induced EGR
Classification
- There is no established system for the classification of EGR.
Pathophysiology
- The cause of EGR has not been identified.
- Many theories suggest that EGR is due to immunologic mechanisms
- The immunologic mechanism theory is evidenced by the observed immunofluorescence patterns of IgG, C3, and C4 at the basement membrane
- Theory 1 the tumor induces antibodies that cross-react with the basement membrane of skin
- Theory 2 the tumor produces polypeptides that bind skin antigens and render them immunogenic
- Theory 3 deposition of tumor antigen-antibody complexes onto the basement membrane causes the reactive dermatitis seen in EGR
Causes
The cause of erythema gyratum repens has not been identified.
Differentiating ((Page name)) from Other Diseases
- EGR has a narrow differential diagnosis and it has to be differentiated from skin lesions with gyrate erythematous eruptions, such as:
- Necrolytic migratory erythema (NME)
- Erythema annulare centrifugum (EAC)
- Erythema migrans
Epidemiology and Demographics
- EGR is a rare dermatologic disease
Age
- The average age of onset of EGR is in the seventh decade of life
Gender
- The male to female ratio is 2:1
Race
- EGR commonly affects Caucasians
Risk Factors
- There are no established risk factors for EGR.
Screening
- There are no screening tests for EGR.
- Screening for internal malignancy should be done immediately after EGR is diagnosed.
Natural History, Complications, and Prognosis
- The majority of patients with EGR presents with severely pruritic erythematous skin lesions that appear several months prior to the malignancy diagnosis.
- If the underlying malignancy left untreated, the debilitating pruritus could persist until the patient dies.
- Prognosis depends on the type of the underlying tumor and the probability of its treatment.
Diagnosis
Diagnostic Study of Choice
- EGR is mainly diagnosed clinically by its characteristic skin lesions.
History and Symptoms
- The universal symptoms of EGR are:
- Skin eruptions
- Intense pruritus
- Other symptoms related to the associated internal malignancy are:
- Weight loss
- Anorexia
- Fatigue
- 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 expand 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.
- Eosinophilia is observed in 60% of cases
Imaging Findings
- There are no imaging findings associated with EGR.
- Imaging of the chest and abdomen could show malignancy findings.
Other Diagnostic Studies
- Direct immunofluorescence in some cases shows patterns of IgG, C3, and C4 at the basement membrane.
- The histopathologic features of EGR is non-specific.
- Biopsy specimens show the following:
- 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.
Treatment
Medical Therapy
- There is no treatment for EGR; the mainstay of therapy is supportive care and treating the underlying condition.
- 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 of EGR, and its associated intense pruritus depends on recognition and treatment of the underlying malignancy.
- Chemotherapy can be used to treat the internal malignancy.
Surgery
- Surgical resection of the internal tumor could be recommended as part of the management of EGR.
Prevention
- There are no primary preventive measures available for [disease name].
References
- ↑ "Reorganized text". JAMA Otolaryngol Head Neck Surg. 141 (5): 428. 2015. doi:10.1001/jamaoto.2015.0540. PMID 25996397.
- ↑ Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/https://doi.org/10.1016/0190-9622( Check
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