Erythema elevatum diutinum.DS Sachdev, VD Kharkar, SA Mahajan, PD Gupte
Department of Dermatology, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai - 400012, India., India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 12571392
Source of Support: None, Conflict of Interest: None
Keywords: Aged, Anti-Inflammatory Agents, Non-Steroidal, therapeutic use,Case Report, Dapsone, therapeutic use,Erythema, diagnosis,drug therapy,pathology,Female, Human,
A 65-year-old female presented with history of relatively asymptomatic reddish-brown raised lesions over the extensor surface of extremities and the joints of eight yearsí duration. There was history of gradual increase in the number and the size of the lesions. The lesions were associated with occasional itching and burning sensation especially after exposure to cold. The patient also complained of knee and ankle joint pains off and on. There was no history of preceding or associated upper respiratory tract infection, gastro-intestinal disturbances, fever and malaise. She was not a known diabetic or hypertensive and had no past history of tuberculosis or contact with tuberculosis.
On examination her vital parameters were within normal limits. There were multiple bilateral symmetrical reddish-brown plaques on the extensor aspects of elbows, dorsum of hands, fingers, gluteal region, knees [Figure - 1], toes, Achilles tendon [Figure - 2] and ears. The plaques were erythematous, smooth surfaced, firm in consistency, non-tender and not fixed to the underlying tissues. The nerves were not thickened and the sensations over the plaques were normal. There was no significant lymphadenopathy, hepatosplenomegaly or joint deformity.
The complete haemogram, blood biochemistry, X-ray chest and ECG were normal. ASLO titre was normal and throat swab did not show any growth. The rheumatoid factor, ANA, dsDNA and ELISA for HIV were negative. However her ESR was 60 mm at the end of 1 hour and serum IgA levels were elevated on two occasions: 520 mg% and 620 mg% respectively (Normal: 145-285 mg%). Serum IgG and IgM were within normal limits. Urine examination for Bence-Jones proteins was negative. Bone-marrow aspiration study was normal. Skin biopsy from an erythematous plaque revealed normal epidermis and a dense dermal infiltrate of predominant neutrophils with few lymphocytes especially in the perivascular areas [Figure - 3]. The blood vessels showed endothelial cell swelling, infiltration of walls with neutrophils, extravasation of erythrocytes and perivascular nuclear dust [Figure - 4]. Nuclear dust is a result of fragmentation of polymorph nuclei; this phenomenon is known as leucocytoclasia.
On the basis of clinical findings and investigations, the final diagnosis of Erythema elevatum diutinum (EED) was made. Patient was started on 100 mg of dapsone daily along with topical emollient. NSAIDs were given for joint pains. A significant flattening of the lesions was noticed by the end of two months of therapy. Repeat biopsy at this stage revealed dense dermal fibrosis and decrease in the amount of neutrophilic infiltrate. Topical moderate potent steroids were given to hasten the resolution. Patient showed near complete healing of lesions over the next four months.
Erythema elevatum diutinum is a rare form of cutaneous leucocytoclastic vasculitis. It is associated with several immunological and infectious diseases such as recurrent / chronic bacterial infection (esp. streptococcal), HIV, collagen vascular diseases (rheumatoid arthritis, relapsing polychondritis, lupus erythematosus), haematological disorders (hypergammaglobulinemia, multiple myeloma, myelodysplasia), inflammatory bowel disease, B-Cell lymphomas, myeloproliferative disorders and gout.
It has to be differentiated from granuloma faciale, granuloma annulare, xanthomas and multicentric reticulohistiocytosis. The diagnosis is based on the characteristic morphology, distribution pattern of the lesions and histopathological findings of leucocytoclastic vasculitis. A thorough investigative work-up of these patients is important to detect the systemic associations especially myeloproliferative disorders which can be life-threatening.
EED is characterised by the absence of systemic vasculopathy. It was first described in 1888 by Hutchinson and in 1889 by Bury. It is believed to be mediated by the deposition of circulating immune complexes in the dermal perivascular spaces. This induces an inflammatory cascade, which damages the vessels causing fibrosis.
Though it can occur at any age, the onset is common in the third to sixth decade of life. It is clinically characterised by occurrence of red, purple or yellow papules, plaques or nodules, which have a predilection for the extensor surfaces and joints. The trunk and the mucosae are usually spared. The skin lesions may be asymptomatic or associated with pain, itching or burning sensation. Large lesions cause significant cosmetic disfigurement. Skin lesions may mimic those of dermatofibroma, granuloma annulare, granuloma faciale or Kaposiís sarcoma., Arthralgia is the most common systemic symptom in these patients.
Skin biopsy is usually diagnostic; other investigations like direct immunofluorescence and electron microscopy can be done in case of doubt.
Most of the patients show good therapeutic response to dapsone as was seen in our patient. Other alternatives are sulfapyridine, niacinamide, colchicine and corticosteroids. Patients with associated IgA paraproteinemia may be refractory to above line of treatment. Such cases show a good response to intermittent plasma exchange.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]