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An episodic eruption
Q1: What is the name given to this rash (SEE p 249), and how is the appearance described?
The rash is erythema gyratum repens. The specific features of this figurate rash are concentric erythematous arcs and rings, as well as serpiginous bands and stripes in alignment. The whorly pattern was initially described in 1953 by Gammel as “knotty cypress wood grain”.1 The rash spreads over the skin at a rate of about 1 cm a day, and may leave a similarly patterned scale in its wake. It is not raised, and is associated with pruritus (50%) and eosinophilia.
Q2: What underlying diseases does it suggest?
The rash is most commonly associated with internal malignancy. In one literature review of 49 cases the most common accompanying neoplasms were bronchial (32%), or were found in the oesophagus (8%) or breast (6%), or were unidentified metastatic malignancies (6%); less common were tumours of the pharynx, stomach, bowel and pancreas, as well as of the genitourinary tract.2 Lymphoreticular neoplasms were under-represented. In addition, the rash has been found in association with tuberculosis, limited systemic sclerosis (CREST syndrome), secondary Sjogren's syndrome,3 virginal breast hypertrophy 4 and hypereosinophilic syndrome,5 although most of these are single case reports. In about 20% of cases, in whom follow up has continued for one to three years, there is no associated underlying disease.6
Erythema gyratum repens is one of several paraneoplastic eruptions which, while rare, are important for signifying the presence of internal malignancy. Such rashes often precede the clinical identification of an underlying tumour by months or years. Consequently, their discovery should prompt an extensive screen for occult malignancy which needs to be repeated regularly for at least several years, but possibly up to 5–10 years after the rash's disappearance. Examples of some of these rashes are listed in table 1.
These rashes have in common the fact that they do not arise from malignant infiltration of the skin, or from local tumour pressure effects (for example, lymphoedema), but are the result of immune, metabolic or endocrine effects, that have been identified in only some cases.
In the case of erythema gyratum repens evidence exists for an immunological basis.2 Techniques employing immunohistochemistry have shown a B cell lymphocytic and eosinophilic infiltrate around dermal vessels, with an inconsistent presence of immunoglobulin and C3 deposits in either the basement membrane or epidermis. Theories for the rash's aetiology include cross reaction of tumour antigens in association with specific human leucocyte antigen (HLA) haplotypes or immune complex deposition.7 Interestingly, the rash conforms in its spatiotemporal pattern to that seen in a chemical reaction diffusion model—the Belousov-Zhabotinskii reaction—that describes other biological patterns, such as that found in slime mould.2,9
Rashes with a paraneoplastic association may also occur in patients in whom no malignancy is ever found, despite extensive investigation and follow up. Moreover, such rashes have a tendency to co-occur: there are several reports of erythema gyratum repens coexisting with pemphigus, pemphigoid, icthyosis, hyperkeratotic palms and soles, and psoriasiform lesions.
Erythema gyratum repens appears to persist in those cases where carcinoma is recognised, but it may resolve with steroid treatment in both patients with and without coincident malignancy.8 Dapsone has also been effective in a patient with hypereosinophilic syndrome.5 In the one case associated with Sjogren's syndrome, secondary to rheumatoid arthritis, the rash appeared episodically, corresponding to adjustments (up and down) to the dose of prednisolone.3 Our patient's dose of prednisolone remained constant throughout.
The patient was screened for occult malignancy, but thorough clinical examination, an oesophagogastroduodenoscopy, barium enema, and computed tomography of the thorax, abdomen, and pelvis failed to identify any neoplasm. A skin biopsy showed non-specific acute inflammatory changes, consistent with a toxic erythema. The rash was seen to disappear and then reappear over a two week period. It was not seen again over the remaining 13 months of her life. Her cause of death was staphylococcal septicaemia that she acquired after a leg amputation, performed for osteomyelitis of the right foot.
In the majority of cases of erythema gyratum repens associated with malignancy, the tumour was diagnosed within seven months of the rash appearing; the longest time was six years.2
Erythema gyratum repens associated with secondary Sjogren's syndrome.