|Year : 2019 | Volume
| Issue : 1 | Page : 92-95
A case of erythema elevatum diutinum with dapsone hypersensitivity syndrome
Anirudha Dharnidhar Gulanikar, Shubhangini S Sharma
Department of Dermatology, MGM Medical College and Hospital, Aurangabad, Maharashtra, India
|Date of Web Publication||14-Feb-2019|
Anirudha Dharnidhar Gulanikar
Department of Dermatology, MGM Medical College and Hospital, N-6, CIDCO, Aurangabad - 431 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Erythema elevatum diutinum (EED) is a rare chronic form of leukocytoclastic vasculitis that belongs to the group of neutrophilic dermatoses. The exact pathogenesis of the disease is still unclear. It has been associated with a number of systemic diseases including infections, autoimmune diseases, and both benign and malignant hematologic disorders. We report the case of a 50-year-old woman with complaints of symmetrical lesions on the extensor aspects of bilateral upper and lower limbs and pressure areas for 6 months. This case was difficult to manage because the patient was also hypersensitive to dapsone which is the drug of choice in EED.
Keywords: Dapsone hypersensitivity syndrome, erythema elevatum diutinum, neutrophilic dermatoses
|How to cite this article:|
Gulanikar AD, Sharma SS. A case of erythema elevatum diutinum with dapsone hypersensitivity syndrome. Clin Dermatol Rev 2019;3:92-5
| Introduction|| |
Erythema elevatum diutinum (EED) is a chronic leukocytoclastic vasculitis first described by Hutchinson in 1888. It is characterized by asymptomatic red-to-purple papules and nodules occurring symmetrically on the extensor surfaces and dorsal joints. The disease can occur at any age but most commonly peaks in the sixth decade with an equal sex ratio. This form of vasculitis is a rare form, with approximately 250 cases reported till date. The drug of choice for the disease is dapsone.,
Dapsone has many adverse effects, one of which is the life-threatening systemic hypersensitivity syndrome named dapsone hypersensitivity syndrome (DHS). It is characterized by fever, skin rash, eosinophilia, lymphadenopathy, and hepatic, pulmonary, and other systemic manifestations.,,
We report this case not only because of the minimal number of case reports found on the disease itself, but also to discuss the therapeutic challenge faced because of this patient's hypersensitivity to dapsone.
| Case Report|| |
A 50-year-old woman presented with violaceous, hyperpigmented, and hyperkeratotic lesions on her upper and lower limbs for 6 months. These lesions were associated with burning sensation.
There was no history of previous treatment taken for the same. The patient did not present with any systemic symptoms.
On examination, there were multiple, violaceous, hyperpigmented, and hyperkeratotic plaques and papules over the extensor surfaces of both upper and lower limbs as well as gluteal region. Lesions were bilaterally symmetrical [Figure 1].
The patient's blood counts were within normal limits, and HIV test and Venereal Disease Research Laboratory status were nonreactive. Histopathology revealed a diffuse dense infiltrate surrounding small thickened vessels in the reticular dermis [Figure 2].
|Figure 2: Biopsy shows a diffuse dense infiltrate involving most of the reticular dermis with mild fibroplasia. The infiltrate consists of neutrophils and histiocytes with a scattering of lymphocytes and occasional plasma cells. The infiltrate surrounds small thickened vessels and extends extensively into the interstitium. Small amount of neutrophilic nuclear dust is also present within the infiltrate|
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The patient's G6PD level was normal and she was started on dapsone 100 mg daily. Significant improvement was seen in the next follow-up, a week later [Figure 3].
After 3 weeks of dapsone therapy, the patient developed maculopapular lesions over her trunk and lower limbs [Figure 4], along with a history of fever and cough as well. On repeat investigations, the liver function tests were deranged with raised leukocyte count and decreased hemoglobin, all suggestive of DHS. Abdominal sonography showed no abnormal findings. High-resolution computed tomography of chest showed features of interstitial pneumonia. Dapsone was stopped immediately and the patient was started on systemic corticosteroids and colchicine. Unfortunately, the patient is lost to follow-up.
|Figure 4: Erythematous macular lesions on the back after starting dapsone therapy|
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| Discussion|| |
EED is classically characterized by red-to-purple cutaneous nodules on the extensor surfaces. Vesicular, bullous, and ulcerative types of lesions have also been reported. These lesions are characteristically asymptomatic and are associated with constitutional symptoms such as fever and arthralgia.
The etiology of EED is thought to be due to the deposition of the circulating immune complexes in perivascular spaces, thereby leading to an inflammatory cascade damaging the vessel wall.
The diagnosis can be confirmed on histopathology which shows leukocytoclastic vasculitis with neutrophilic infiltrates around blood vessels in the mid dermis mixed with eosinophils, lymphocytes, plasma cells, and nuclear dust in early lesions. Late lesions show mixed inflammatory cell infiltrate, fibrin deposition, cholesterol deposits in histiocytes, and extracellular tissue.
Treatment options include tetracycline, niacinamide, colchicine, systemic and intralesional corticosteroids, sulfapyridine, and chloroquine; however, dapsone is considered the treatment of choice., Surgical excision is also an option for chronic cases who are not suitable for medical management.
EED is associated with several other diseases such as HIV, Hepatitis B, syphilis, autoimmune diseases, and hematological disorders.,,,
The diagnosis of DHS is based on the history of treatment with dapsone, high-grade fever, skin lesions, lymphadenopathy, hepatitis, and other systemic features including acute pneumonitis. DHS can develop several weeks to as long as 6 months after treatment initiation and the incidence reported ranges between 0.5% and 3%. In our present case, the patient showed clinical signs after 3 weeks of therapy and responded to oral prednisolone with normalization of liver function as well.
This patient was unique when compared to other case reports due to the therapeutic challenge faced while treating her with dapsone. She is now being treated with systemic corticosteroids and was recently started on colchicine. The results, however, are nowhere close to the one achieved by dapsone within a week. The lesions on the dorsal surface of feet are ulcerative but healing, while the other lesions had neither increased in size or number nor reduced [Figure 5]. The patient was treated for interstitial pneumonia and presently has no respiratory complaints.
|Figure 5: Lesions over the Achilles ulcerating after stopping dapsone therapy|
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Case reports, so far, have discussed the association of EED with various underlying conditions such as multiple myeloma and HIV., They have also discussed the unusual presentations, therapeutic success with dapsone, and surgical management.,,,
Association of EED with paraproteinemia, especially monoclonal IgA gammopathy and IgA myeloma, has been reported since 1977. EED being reported as a specific HIV-associated dermatosis is also present in the literature where 11 cases of HIV being linked to EED have been discussed. Kentley et al. described an unusual case of EED with oral ulceration which led to loss of uvula.
Takiwaki et al. reported three cases of peripheral ulcerative keratitis in EED, again which is a rare presentation.
Awareness of this rare disease is important to prevent misdiagnosis, and also relevant information about dapsone and its complications must be explained to the patient for better compliance and prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]