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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 419-421
Erythema Elevatum Diutinum: Revisiting A Rare Entity in Pediatric Age Group


Department of Dermatology Venereology and Leprosy, Yenepoya Medical College Hospital, Yenepoya (Deemed to be University), Mangalore, Karnataka, India

Date of Web Publication17-Sep-2021

Correspondence Address:
Spandana P Hegde
Department of Dermatology Venereology and Leprosy, Yenepoya Medical College Hospital, Yenepoya (Deemed to be University), Mangalore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_219_19

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How to cite this article:
Kalekhan FM, Hegde SP, Shenoy M M, M. Iqbal AA. Erythema Elevatum Diutinum: Revisiting A Rare Entity in Pediatric Age Group. Indian J Dermatol 2021;66:419-21

How to cite this URL:
Kalekhan FM, Hegde SP, Shenoy M M, M. Iqbal AA. Erythema Elevatum Diutinum: Revisiting A Rare Entity in Pediatric Age Group. Indian J Dermatol [serial online] 2021 [cited 2021 Dec 3];66:419-21. Available from: https://www.e-ijd.org/text.asp?2021/66/4/419/326112




Sir,

Erythema elevatum diutinum is a rare chronic neutrophilic dermatosis. It is a cutaneous form of leucocytoclastic vasculitis and this feature separates it from other neutrophilic dermatoses and hence it straddles the horizon between neutrophilic dermatoses and vasculitides.[1]

A 6-year-old female child presented for evaluation of recurrent episodes of fever, arthralgia, and asymptomatic skin lesions of three years duration. The lesions healed over a span of 3 to 4 weeks with scarring. There were no other co-morbidities. Examination revealed multiple firm erythematous plaques and nodules, having smooth surface with central ulceration and crusting with atrophic scars distributed in a symmetrical manner predominantly over the extremities [Figure 1]. Lesions were also seen over the face, back, gluteal region, and palms and soles [Figure 2]a and [Figure 2]b. Laboratory evaluation revealed an elevated erythrocyte sedimentation rate (46 mm/h.) Other laboratory investigations like blood counts, serum IgE levels, liver function test, renal function test, urine routine and microscopy, chest X-ray, and Acid fast bacilli (AFB) staining of sputum were normal. Antinuclear antibodies (ANA) by immunofluorescence was negative and serological tests for HIV and hepatitis B were non-reactive. Serum immunoglobulin assay showed IgA, IgG, and IgM were within normal limits. Histopathology of a recent erythematous plaque showed extensive dense inflammatory infiltrate in the upper dermis predominantly composed of neutrophils, extending around the adnexal structures and blood vessels. Leucocytoclastic vasculitis, neutrophilic infiltration of vessels, and extravasation of RBCs were noted focally [Figure 3]. With the above clinical, laboratory, and histopathological evidences, we arrived at a diagnosis of erythema elevatum diutinum (EED). We were unable to find an underlying disease association in spite of detailed work up in this case. She was started on oral dapsone to which she showed partial response [Figure 4]a and [Figure 4]b.
Figure 1: Multiple firm erythematous plaques, nodules, and atrophic scars over extensor aspect of bilateral thighs and knees

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Figure 2: Nodules with atrophic scars over the gluteal region (a) and sole (b)

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Figure 3: Dense upper dermal neutrophilic inflammatory infiltrate with leucocytoclastic vasculitis (H and E, 40×)

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Figure 4: Nodules and plaques on the upper extremities at presentation (a) and improvement after 1 month of dapsone therapy (b)

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EED is most commonly seen in third to sixth decade and is relatively uncommon in children.[2] It is characterized by indolent red-violet to reddish-brown papules and nodules which show symmetrical acral predilection, predominantly involving the extensor aspect of extremities.[1],[3] The initial lesions are relatively softer and progress to form fibrosing scars which are cosmetically disfiguring as seen in our case.[4]

EED is associated with a variety of clinical illness like human immunodeficiency virus infection, malignant conditions (hematologic malignancies, paraproteinemia, non-Hodgkins lymphoma, pulmonary lymphoepithelioma like carcinoma, breast carcinoma, verrucous carcinoma), connective tissue diseases (systemic lupus erythematosus, juvenile idiopathic arthritis, dermatomyositis, primary Sjogren's syndrome), autoimmune disorders (Hashimoto's thyroiditis, antiphospholipid antibody syndrome, myeloperoxidase-anti neutrophil antibody associated pulmonary interstitial fibrosis, IgA Anti- neutrophil cytoplasmic antibodies (ANCA)-associated primary Sjogren's syndrome), chronic infections, celiac disease, pyoderma gangrenosum, and IgA gammopathies.[3],[5]

Histopathology of an early lesion shows perivascular and nodular neutrophilic infiltrates with leucocytoclastic vasculitis and papillary dermal edema. The late lesion shows fibrosis along with macrophages containing prominent lipids and histiocytic infiltrates with granulation tissue.[5] IgA monoclonal gammopathy is most commonly associated with EED patients and routine immunoelectrophoresis testing is necessary.[1] The treatment of choice is dapsone and shows a good therapeutic response. Other alternative drugs used include sulfonamides, sulfasalazine, niacinamide, and colchicine.[4],[5] Systemic corticosteroids are not effective. Underlying associated condition must be addressed accordingly.

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.
Sharma V, Mahajan VK, Mehta KS, Chauhan PS, Chander B. Erythema elevatum diutinum. Indian J Dermatol Venereol Leprol 2013;79:238-9.  Back to cited text no. 1
  [Full text]  
2.
Shinkai K, Fox LP. Cutaneous vasculitis. In: Bolognia J, Jorizza JL, Schaffer JV, editors. Dermatology. 3th ed. China: Elsevier Ltd; 2012. p. 396-7.  Back to cited text no. 2
    
3.
Nguyen GH, Guo EL, Norris D. A rare case of erythema elevatum diutinum presenting as diffuse neuropathy. JAAD Case Rep 2017;3:1-3.  Back to cited text no. 3
    
4.
Sachdev DS, Kharkar VD, Mahajan SA, Gupte PD. Erythema elevatum diutinum. J Postgrad Med 2002;48:310-1.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Panda S, Ghosh A. Neutrophilic dermatoses. In: Sacchidanand S, Oberai C, Inamdar AC, editors. IADVL Textbook of Dermatology. 4th ed. Mumbai: Bhalani Publishers; 2015. p. 1272-3.  Back to cited text no. 5
    


    Figures

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



 

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