Erythema elevatum diutinum in an HIV-positive patient

Journal of Drugs in Dermatology, August, 2003 by Hanna Kim

Abstract

A 53-year old man with HIV infection and hepatitis C infection presented with multiple, firm nodules on the hands and ankles. Erythema elevatum diutinum is a rare, chronic cutaneous vasculitis that may be associated with hematologic, autoimmune, and infectious diseases, which include HIV infection. First-line therapy includes dapsone, as well as treatment of any underlying cause or infection.

Case Report

A 53-year-old man was referred to the Charles C. Harris Skin and Cancer Pavilion in August 2002 with a one-to-two year history of multiple nodules on the hands and around the ankles. The patient had been treated with multiple courses of oral antibiotics, including ciprofloxacin and cephalexin, with improvement. The condition had been worsening over the course of months, with intermittent pain over the palmar aspects but without pruritus or ulcers.

Past medical history included human immunodeficiency virus (HIV) infection, hepatitis C virus infection, and prior intravenous drug use without previous opportunistic infections. Medications include zidovudine, lamivudine, indinavir, interferon alpha-2, and ribavirin.

Physical examination revealed numerous discrete, firm, nontender, coalescent nodules that ranged from 0.3 to 0.6 cm in size and were present over the palmar and dorsal aspect of the hands, particularly over the metacarpophalangeal and proximal and distal interphalangeal joints. Similar lesions were noted on the ankles, more prominently on the lateral aspects (Figure 1).

[FIGURE 1 OMITTED]

The lab reports indicated the human immunodeficiency viral load was < 50 pg/ml, with a CD4 lymphocyte count of 618/[mm.sup.3].

Histopathologically, a perivascular infiltrate of lymphocytes and a few neutrophils were associated with a few extravasated erythrocytes. The vessels showed no evidence of fibrin deposits in their walls or thrombi in their lumens. Around the blood vessels, collagen bundles were arranged in a parallel pattern, alternating with deposits of connective-tissue mucin. We diagnosed the patient with erythema elevatum diutinum.

Discussion

Erythema elevatum diutinum (EED) is a rare chronic form of cutaneous vasculitis which most commonly presents as symmetrical, firm, tender, redbrown or violaceous papules, plaques, and nodules that are distributed acrally over extensor surfaces and located near joints, such as the fingers, hands, elbows, ankles, and knees. The onset of new lesions may be associated with pruritus or a burning sensation, and established lesions may be tender, although some cases are asymptomatic. EED is observed most commonly in the fourth through sixth decade with a slight male predominance (1).

The cause of EED is unknown, although the pathogenesis is believed to be immune complex-mediated. Various conditions have been reported with this disease, which include hematologic and autoimmune disorders, such as rheumatoid arthritis, ulcerative colitis, Crohn's disease, relapsing polychondritis, pyoderma gangrenosum, type I diabetes mellitus, and gluten-sensitive enteropathy. Associated infections include bacterial, viral, tuberculosis, hepatitis, and syphilis.

EED associated with HIV infection has been reported in fewer than 20 cases (2-4). The lesions have been described as nodular with palmar/plantar involvement; more lesions are present at an earlier age. The lesions must be distinguished from Kaposi's sarcoma and bacillary angiomatosis.

Improvement may result from treatment of an underlying cause or infection. Dapsone and sulfonamides are considered first-line treatments for EED; however, lesions often recur with cessation of therapy. Some cases of EED associated with HIV infection have demonstrated a good response to dapsone alone or in combination with antiretroviral therapy (2,4). Dapsone may be less effective, however, in lesions that have progressed to the more fibrotic stage. Surgical excision of larger nodules has also been performed with some success (3).

References

(1.) Gibson LE, El-Azhary RA. Erythema elevatum diutinum. Clin Dermatol 2000; 18:295.

(2.) Suarez J, et al. Nodular erythema elevatum diutinum in an HIV-1 infected woman: response to dapsone and antiretroviral therapy. Br J Dermatol 1998; 138:717.

(3.) Muratori, et al. Erythema elevatum diutinum and HIV infection. Br J Dermatol 1995; 14:335.

(4.) Martin JI, et al. Erythema elevatum diutinum, a clinical entity to be considered in patients infected with HIV-1. Clin Exp Dermatol 2001; 26:725.

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