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Case Note

Case Note: Tender Nodules on the Arms and Hands

History

A 48-year-old female, with past medical history notable for HIV infection, presents for management of slightly tender nodules on the arms and hands.

Initial evaluation

  • Healthy-appearing 48-year-old female presents for management of red-brown plaques and small tender nodules on the bilateral extensor arms and dorsal hands
  • Past medical history notable for HIV infection with history of oral candidiasis and pneumocystis pneumonia at CD4 nadir of 44
    • Now with CD4+ T-lymphocyte count of 267 on antiretroviral medications
  • Ocular exam is normal
  • Skin biopsy performed
    • Revealed sclerosis with plasma cells and foci of leucocytoclastic vasculitis
  • Image 1
    Image 1

Enter your diagnosis

Diagnosis

  • A diagnosis of erythema elevatum diutinum (EED) is rendered

Initial Treatment

  • Treatment options discussed; patient opts for treatment to limit progression of new lesions
    • Dapsone 50 mg is prescribed
  • Pre-treatment lab work is ordered
    • Complete blood count with differential, glucose-6-phosphate dehydrogenase levels
  • Additional diagnostic evaluation performed to exclude other associations:
    • Hepatitis serologies, anti-streptolysin O (ASO) titer, treponemal titers, serum protein electrophoresis (to rule out paraproteinemia)
  • Follow up in 1 month

Follow-up evaluation strategy

1-month follow-up evaluation:

  • The skin lesions are much improved and the patient reports that there are fewer new lesions
  • Subsequent blood work remains normal - indicating that the medication is being tolerated
    • Due to the ongoing appearance of the few new lesions, dapsone dose is escalated to 100 mg/day
  • Follow up in 1 month

Subsequent follow-up evaluation:

  • Patient reports significant clinical improvement
    • No new lesions
  • Dapsone dose continued at 100 mg/day

Further recommendations:

  • Intralesional triamcinolone acetonide 2.5-5.0 mg/cc may be used if there are few lesions
  • Niacinamide, colchicine, hydroxychloroquine, clofazimine and cyclophosphamide may be helpful
    • Oral corticosteroids are typically not effective
  • In patients with celiac disease, a gluten-free diet may be useful
  • Intermittent plasma exchange has been successfully employed in patients with immunoglobulin A (IgA) paraproteinemia
  • Antiretroviral treatment will improve EED in a HIV/AIDS patient

Subsequent treatment steps:

  • In patients failing to respond to low-dose dapsone, the dosage may be increased to a maximum of 200 mg/day
  • For patients intolerant of dapsone, or when dapsone is only partially effective, colchicine 0.6 mg one to four times daily may be considered
  • In dapsone-intolerant patients, sulfapyridine 500 mg, twice daily, with a maximum dose of 1 g three times daily can be used

General discussion

EED is rare and is thought of as a chronic, fibrosing vasculitis with firm, red-brown or violaceous, nodular lesions developing over joints of the elbows, knees, hands, and feet, often in a symmetric pattern. Though the etiology of the disease is not known, inflammation resulting from immune complex deposition is suspected. The diagnosis is made on the basis of clinical appearance and histopathologic findings.

EED has been associated with HIV/AIDS, hematologic disorders (IgA monoclonal gammopathy, multiple myeloma), celiac disease, Wegener's granulomatosis, connective tissue disorders (especially systemic lupus erythematosus), and recurrent streptococcal infections. In HIV/AIDS, nodules on the soles are characteristic. EED has been rarely associated with ocular complications, including peripheral ulcerative keratitis. It is a chronic condition with intermittent manifestations that may recur over the span of 10-25 years. The goal of treatment is to limit the development of new lesions, i.e., progression of disease.

Previously categorized as a neutrophilic dermatosis, current nomenclature classifies EED as a form of vasculitis. The biopsy of early lesions of EED may be indistinguishable from lesions of leukocytoclastic vasculitis: neutrophilic infiltration around vessels with neutrophilic 'dust' and fibrin clots within small vessels (specifically venules). Only after lesions have evolved over several weeks do characteristic histopathological features of EED become recognizable, including marked fibrosis. Once lesions are advanced with evidence of fibrosis, they may not be amenable to treatment with anti-inflammatory agents.

Further reading

Bachmeyer C, Aractingi S. Erythema elevatum diutinum associated with HIV. Lancet 1996;347:1041-2.

Gibson LE, El-Azhary RA. Erythema elevatum diutinum. Clinics Derm 2000;18:295-9.

Jiao T, Wang M, Zhu X. A case of erythema elevatum diutinum associated with peripheral ulcerative keratitis. Austr J Derm 2012;53:78-80.

Vaiyavatjamai P, Wattanakrai P. Erythema elevatum diutinum associated with peripheral ulcerative keratitis. JEADV 2011;25:734-46.

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