Erythema gyratum repens

Author: Dr Cathy Yunjia Zhao, Dermatology Registrar, Westmead Hospital, Sydney, New South Wales, Australia, November 2018. DermNet NZ Editor-in-Chief A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy-edited by Gus Mitchell. January 2019.


What is erythema gyratum repens?

Erythema gyratum repens is a rare paraneoplastic type of annular erythema with a distinctive figurate ‘wood-grain’ appearance. It has a strong association with malignancy.

Erythema gyratum repens-like eruption*
* This patient's rash had a wood-grain appearance like erythema gyratum repens but the rash was actually pemphigus foliaceus.

Who gets erythema gyratum repens?

Erythema gyratum repens is most often present in Caucasian men in their 60s. The male-to-female ratio is 2:1. There is an associated underlying malignancy in 80% of patients of erythema gyratum repens. Almost half of the patients have lung cancer (33–47%), and less commonly, oesophageal, breast and stomach cancer [3–4].
In rare cases, erythema gyratum repens has also been described in association with mycobacterial infections, connective tissue diseases and pregnancy [3]. 

What causes erythema gyratum repens?

The strong association between erythema gyratum repens and malignancy have led to several different pathogenetic hypotheses.

  1. The main hypothesis is erythema gyratum repens is the result of a cross-reaction between antibodies against tumour antigens and cutaneous antigens. The responsible cutaneous antigen is not known. This is supported by the fact that direct immunofluorescence (DIF) of skin in erythema gyratum repens has shown granular deposits of IgG and C3 in the dermo-epidermal junction, with similar deposits also seen in the associated neoplasm [1].
  2. An alternate hypothesis is that a tumour may have produced substances capable of changing constituents of the skin, with deposition of antibodies and complement activation [1].
  3. Recently, a link was proposed between cutaneous glutamine (a substance required for the proliferation of cells) and the multi-ring appearance of erythema gyratum repens [2].

What are the clinical features of erythema gyratum repens?

Erythema gyratum repens presents with concentric erythematous rings that have a distinctive wood-grain appearance. The rings progress in waves, with the leading age migrating about 1 cm/day. A trailing or collarette scale may be seen. Erythema gyratum repens is very itchy.
The annular plaques most often involve the limbs and trunk [3].

How is erythema gyratum repens diagnosed?

Although erythema gyratum repens may be diagnosed clinically, a skin biopsy is often performed to exclude other conditions. The histological features of erythema gyratum repens are non-specific.

Other investigations may include complete blood count, biochemistry, antinuclear antibody profile and malignancy screens (chest x-ray, mammogram, cervical smear, tumour markers, CT chest/abdomen/pelvis and, if indicated, endoscopy or colonoscopy) [3].

What is the differential diagnosis for erythema gyratum repens?

Other conditions that should be considered in the diagnosis include:

What is the treatment for erythema gyratum repens?

The treatment for erythema gyratum repens is to identify and treat the primary malignancy. Erythema gyratum repens usually resolves once the malignancy has gone, for example with surgical resection. Topical steroids have not been effective [3].

 

Related information

 

References

  1. Holt PA & Davies MG. Erythema gyratum repens: an immunologically mediated dermatosis? Br J Dermatol 1977; 96: 343–7. PubMed
  2. Forrester DM. Self-assembled multi-ring formations of glutamine and a possible link to erythema gyratum repens. Med Hypotheses 2015; 85: 10–16. PMID: 25804239. PubMed
  3. Rustin M & Cerio R. Chap 47. Reactive inflammatory erythemas. In: Rook’s Textbook of Dermatology. Ninth ed. Oxford: Wiley Publishers, 2016.
  4. Dewitt C, Buescher LS & Stone SP. Chap 153. Cutaneous manifestations of Internal Malignant Disease. In: Fitzpatrick’s Dermatology in General Medicine. Eighth edition. McGraw Hill Medical.

On DermNet NZ

Books about skin diseases

See the DermNet NZ bookstore.