Erythema elevatum diutinum. Response to dapsone

Abstract

A patient with a 20-year history is presented as a classic example of erythema elevatum diutinum, with a dramatic response to dapsone therapy, and with interesting features of improvement during pregnancies and occurrence of giant cell tumors of soft tissue, neither of these latter features having been previously reported.

Erythema elevatum diutinum: a review of presentation and treatment

“…63 Low-dose (25 mg) dapsone was used in a pregnant patient as a 'safe dose'; during a flare of EED, this dose had no effect on lesions. 66 Of the 66 patients in which dapsone with or without additional therapy had a clinical effect, there were eight (12%) reported cases of lesion recurrence. Recurrence time ranged from rapid recurrence (12 h) 14,64 to longer recurrence (average was 23 months, range 6-24 months).…”

“…69 Often, treatment of underlying disease is of clinical benefit when treating cases of EED. 23,44,52,66,70,71 Intermittent plasma exchange cleared flares of EED in patients with IgA paraproteinemia. 44 Chemotherapy was successful for a patient with invasive ductal breast cancer 57 and when associated with Wegner's granulomatosis, lesions resolved following with methylprednisolone and cyclophosphamide.…”

“…62 In one patient, a relapse of lesions was precipitated by the development of a malignant lymphoma. 66 One patient was followed up for 3 years post-dapsone termination with no lesion recurrence. Le boit et al reported remission of their patients' lesions whilst on low-dose (50 mg) dapsone at 8 months followup.…”

Cutaneous Manifestations of Celiac Disease

“…Its lesions appear as soft erythematous papuleplaques and become hard nodules mainly symmetrically affecting the backs of the hands and other extensor surfaces overlying the joints [67] . Ulceration, arthralgia and pain can be other features of this disease [67,68] . Even if it is presumed to be an immune reaction by bacterial antigens, the etiology of EED is unknown [69] .…”

A case of mistaken identity: unilateral erythema elevatum diutinum associated with IgA paraproteinaemia

“…Our patient's localized disease was treated with excision before confirmation of the diagnosis. EED is commonly treated medically with dapsone with a good outcome, although excision has also been used effectively . For the localized disease in this case, excision appears to have been a successful treatment.…”