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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 1  |  Page : 92-95

A case of erythema elevatum diutinum with dapsone hypersensitivity syndrome


Department of Dermatology, MGM Medical College and Hospital, Aurangabad, Maharashtra, India

Date of Web Publication14-Feb-2019

Correspondence Address:
Anirudha Dharnidhar Gulanikar
Department of Dermatology, MGM Medical College and Hospital, N-6, CIDCO, Aurangabad - 431 001, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_1_18

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  Abstract 


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

How to cite this URL:
Gulanikar AD, Sharma SS. A case of erythema elevatum diutinum with dapsone hypersensitivity syndrome. Clin Dermatol Rev [serial online] 2019 [cited 2019 Oct 22];3:92-5. Available from: http://www.cdriadvlkn.org/text.asp?2019/3/1/92/252299




  Introduction Top


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.[1] 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.[1],[2]

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.[3],[4],[5]

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 Top


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].
Figure 1: Violaceous plaques on gluteal region

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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].
Figure 3: Size of the lesion reduced after starting dapsone therapy

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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 Top


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.[6] 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[7] 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.[1],[2] Surgical excision is also an option for chronic cases who are not suitable for medical management.[8]

EED is associated with several other diseases such as HIV, Hepatitis B, syphilis, autoimmune diseases, and hematological disorders.[9],[10],[11],[12]

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.[13] DHS can develop several weeks to as long as 6 months after treatment initiation and the incidence reported ranges between 0.5% and 3%.[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.[14],[15] They have also discussed the unusual presentations, therapeutic success with dapsone, and surgical management.[8],[16],[17],[18]

Association of EED with paraproteinemia, especially monoclonal IgA gammopathy and IgA myeloma, has been reported since 1977.[14] 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.[15] Kentley et al. described an unusual case of EED with oral ulceration which led to loss of uvula.[16]

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Momen SE, Jorizzo J, Al-Niaimi F. Erythema elevatum diutinum: A review of presentation and treatment. J Eur Acad Dermatol Venereol 2014;28:1594-602.  Back to cited text no. 1
    
2.
Grabbe J, Haas N, Möller A, Henz BM. Erythema elevatum diutinum – Evidence for disease-dependent leucocyte alterations and response to dapsone. Br J Dermatol 2000;143:415-20.  Back to cited text no. 2
    
3.
Kosseifi SG, Guha B, Nassour DN, Chi DS, Krishnaswamy G. The dapsone hypersensitivity syndrome revisited: A potentially fatal multisystem disorder with prominent hepatopulmonary manifestations. J Occup Med Toxicol 2006;1:9.  Back to cited text no. 3
    
4.
Chalasani P, Baffoe-Bonnie H, Jurado RL. Dapsone therapy causing sulfone syndrome and lethal hepatic failure in an HIV-infected patient. South Med J 1994;87:1145-6.  Back to cited text no. 4
    
5.
Pandey B, Shrestha K, Lewis J, Hawksworth RA, Walker SL. Mortality due to dapsone hypersensitivity syndrome complicating multi-drug therapy for leprosy in Nepal. Trop Doct 2007;37:162-3.  Back to cited text no. 5
    
6.
Qu T, Li L, Jia L, Fang K. Erythema elevatum diutinum: A new vesiculobullous case. Eur J Dermatol 2013;23:530-2.  Back to cited text no. 6
    
7.
Wilkinson SM, English JS, Smith NP, Wilson-Jones E, Winkelmann RK. Erythema elevatum diutinum: A clinicopathological study. Clin Exp Dermatol 1992;17:87-93.  Back to cited text no. 7
    
8.
Rinard JR, Mahabir RC, Greene JF, Grothaus P. Successful surgical treatment of advanced erythema elevatum diutinum. Can J Plast Surg 2010;18:28-30.  Back to cited text no. 8
    
9.
LeBoit PE, Cockerell CJ. Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus. J Am Acad Dermatol 1993;28:919-22.  Back to cited text no. 9
    
10.
Nakajima H, Ikeda M, Yamamoto Y, Kodama H. Erythema elevatum diutinum complicated by rheumatoid arthritis. J Dermatol 1999;26:452-6.  Back to cited text no. 10
    
11.
Chandrasekaran SS, Rai R, Vedachalam S, Dorairaj L, Palaniraman S. Erythema elevatum diutinum in association with dermatitis herpetiformis. Indian Dermatol Online J 2014;5:48-50.  Back to cited text no. 11
[PUBMED]  [Full text]  
12.
Hatzitolios A, Tzellos TG, Savopoulos C, Tzalokostas V, Kaiafa G, Psomas E, et al. Erythema elevatum diutinum with rare distribution as a first clinical sign of non-Hodgkin's lymphoma: A novel association? J Dermatol 2008;35:297-300.  Back to cited text no. 12
    
13.
Allday EJ, Barnes J. Toxic effects of diaminodiphenylsulphone in treatment of leprosy. Lancet 1951;2:205-6.  Back to cited text no. 13
    
14.
Patnala GP, Sunandini AP, Rayavarapu R, Yandapalli PS. Erythema elevatum diutinum in association with IgA monoclonal gammopathy: A rare case report. Indian Dermatol Online J 2016;7:300-3.  Back to cited text no. 14
[PUBMED]  [Full text]  
15.
Muratori S, Carrera C, Gorani A, Alessi E. Erythema elevatum diutinum and HIV infection: A report of five cases. Br J Dermatol 1999;141:335-8.  Back to cited text no. 15
    
16.
Kentley J, Marshall C, Bewley A. Erythema elevatum diutinum-associated with loss of the uvula. JAAD Case Rep 2017;3:212-4.  Back to cited text no. 16
    
17.
Takiwaki H, Kubo Y, Tsuda H, Arase S, Shiota H. Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: A report of three cases. Br J Dermatol 1998;138:893-7.  Back to cited text no. 17
    
18.
Ben-Zvi GT, Bardsley V, Burrows NP. An atypical distribution of erythema elevatum diutinum. Clin Exp Dermatol 2014;39:269-70.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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