Erythema Gyratum Repens as a Manifestation of Drug Reaction with Eosinophilia and Systemic Symptoms

The wide variability noted in the clinical features of drug reaction with eosinophilia and systemic symptoms (DRESS) makes it a difficult to diagnose condition.[1]

A 70-year-old male on indigenous treatment for the past 1 month for osteoarthritis of knee presented with infiltrated and annular urticated plaques on the face [Figure 1], trunk [Figure 2], and limbs for 1 month; facial, pedal, and penile edema for 2 weeks; and high-grade fever for 3-day duration. Withdrawal of the drug did not attain any relief.

Figure 1.

Infiltrated skin lesions on the forehead of a patient with drug reaction with eosinophilia and systemic symptoms

Figure 2.

Urticated plaques on the trunk of the patient with drug reaction with eosinophilia and systemic symptoms

On clinical examination, skin-smear studies for acid-fast bacilli and fungal scrapings ruled out Hansen's disease and dermatophytosis. There was no significant lymphadenopathy. Systemic examination was within normal limits. Laboratory workup revealed neutrophilic leukocytosis and elevated absolute eosinophil count (11,000 cells/mm³). All other blood parameters including blood culture, serology for antinuclear antibody, syphilis, and infections due to Hepatitis B, C, and A viruses and human immunedeficiency viruses detected no abnormality. Chest radiography and computerized tomogram of the thorax showed bilateral, mild pleural effusion. Detailed workup including pleural fluid aspiration analysis was not in favor of pulmonary tuberculosis or malignancy.

Biopsy from the skin lesion showed perivascular inflammatory infiltrate composed of lymphocytes, eosinophils, and a few histiocytes without any evidence of interphase dermatitis or necrotic keratinocytes [Figure 3]. Hence, a provisional diagnosis of DRESS to the indigenous drug was made [Table 1].[2,3,4] Fever, edema, pruritus, and face lesions promptly responded to dexamethasone 4 mg twice daily parenterally,[4,5] while some of the lesions on the upper limbs [Figure 4a] and back of the trunk changed morphologically to assume the appearance of erythema gyratum repens [Figure 4b]. Individual lesion migrated for about 3–5 days before showing gradual resolution.

Figure 3.

(a) Histology of cutaneous rash of drug reaction with eosinophilia and systemic symptoms showing perivascular inflammatory infiltrate (H and E, ×100). (b) Higher magnification of the same showing the inflammatory infiltrate to be composed of lymphocytes, eosinophils, and histiocytes (H and E, ×400)

Table 1.

RegiSCAR DRESS validation scoring[2,3] in our case

Figure 4.

(a) Annular urticated lesion on the upper arm of a patient with drug reaction with eosinophilia and systemic symptoms. (b) Lesions changing morphology to erythema gyratum repens. (c) The same patient after complete clearance of the lesions

Reevaluation including serum prostate-specific antigen and lactate dehydrogenase level, ultrasonogram of the abdomen and pelvis, and colonoscopy and contrast-enhanced tomogram of the abdomen and pelvis were within normal limits. A repeat chest radiography showed resolution of pleural effusion.

Steroids were gradually tapered over 85 days. Even 6 months after stopping steroids, the patient remained asymptomatic [Figure 4c] with normal eosinophil count.

DRESS following indigenous drugs and symptoms persisting despite withdrawal of the offending drug as observed in our patient are well-documented features of DRESS.[1,6] Erythema annulare centrifugum and urticarial vasculitis were unlikely in our patient owing to the larger and rapidly migrating nature of lesions and the absence of neutrophilic vasculitis (histologically), respectively.[7,8] Malignancy-associated erythema gyratum repens with DRESS was considered since fever and edema manifested by our patient could not be explained by erythema gyratum repens alone. A transudate pleural effusion which responded to systemic steroids and the complete resolution of erythema gyratum repens noted in our case were against a malignancy-associated process.[7] Erythema gyratum repens has been reported following drugs such as azathioprine and in the resolving stage of pityriasis rubra pilaris.[9,10] Despite extensive literature search, we did not come across any previous reports of erythema gyratum repens occurring during the treatment of DRESS. Although our patient had rash on the face, trunk, and limbs, erythema gyratum repens pattern spared the face as described in the literature.[9]

Our case highlights the need to consider DRESS in the differential diagnosis of pyrexia of unknown origin or persistent pruritic skin rash in view of its widely varying clinical picture. However, considering the strong association of erythema gyratum repens with malignancy and the scarcity of data on its association with DRESS, we have kept the patient under regular follow-up.

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Conflicts of interest

There are no conflicts of interest.