Erythema elevatum diutinum: A rare dermatosis with an atypical presentation

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Erythema elevatum diutinum (EED) is a form of chronic cutaneous vasculitis of unknown
underlying etiology. It presents clinically as asymptomatic, firm, erythematous to yellowish
papules, nodules, or plaques involving the extensor surface of extremities often over joints.
Antigen-induced small vessel vasculitis is believed to be the cause. Palmar involvement, as seen
in our case is rather an unusual manifestation of this rare disease. EED can be the first indicator
of underlying comorbidity necessitating the correct diagnosis, screening, and management of
associated conditions. None of the comorbidities was present in our patient but negative
autoimmune serology does not completely rule out other diseases. There are very few reported
idiopathic cases of EED our patient is one of them. Medical treatment of choice is dapsone in the
early stage. Fibrotic lesions may not respond to dapsone. Surgical excision is recommended in
such patients. In addition, anti-inflammatory agents like tetracyclines, niacinamide, non-steroidal
anti-inflammatory drugs, methotrexate, chloroquine, colchicine, topical corticosteroids, and
plasmapheresis are other successful modalities of treatment reported.

Keywords: Erythem elevatum diutinum