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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 14  |  Issue : 2  |  Page : 101-103

Erythema nodosum leprosum limited to sun-exposed sites: An unusual presentation of type 2 lepra reaction


1 Department of Dermatology, Venereology and Leprology, M.M.I.M.S.R, Mullana, Ambala, Haryana, India
2 Department of Dermatology, Venereology and Leprology, M.M. Institute of Medical Sciences, Solan, Himachal Pradesh, India
3 Department of Pathology, Venereology and Leprology, M.M.I.M.S.R, Mullana, Ambala, Haryana, India

Date of Web Publication18-Apr-2017

Correspondence Address:
Ajay Kumar
E-9/402, Sandeep Vihar, GH-79, Sector 20, Panchkula, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2468-6859.204699

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  Abstract 

The distribution of erythema nodosum leprosum lesions limited to sun-exposed sites is an unusual presentation of a type 2 reaction. We report the case of a 23-year-old female who presented with erythematous papules, nodules and plaques confined to sun-exposed areas over the face, extensor aspect of both arms and forearms, and “V” of chest with fever and joint pains as well as ulnar clawing of the left hand. A diagnosis of type 2 lepra reaction was made which was confirmed by slit skin smear and skin biopsy.

Keywords: Erythema nodosum leprosum, sun-exposed sites, type 2 lepra reaction


How to cite this article:
Kumar A, Dalal A, Gupta S, Dadwal E. Erythema nodosum leprosum limited to sun-exposed sites: An unusual presentation of type 2 lepra reaction. J Clin Sci 2017;14:101-3

How to cite this URL:
Kumar A, Dalal A, Gupta S, Dadwal E. Erythema nodosum leprosum limited to sun-exposed sites: An unusual presentation of type 2 lepra reaction. J Clin Sci [serial online] 2017 [cited 2019 Oct 15];14:101-3. Available from: http://www.jcsjournal.org/text.asp?2017/14/2/101/204699


  Introduction Top


Type 2 lepra reaction is a humoral antibody response in multibacillary leprosy. It is characterized by the crops of evanescent erythematous tender nodules distributed mainly over flexural aspect of the forearm, medial aspect of thighs and face with fever and systemic symptoms. We report an unusual case of type 2 lepra reaction with erythema nodosum leprosum (ENL) lesions limited to sun-exposed sites on the face, upper chest, and extensor aspect of upper arms and forearms. A case of atypical type 2 lepra reaction with ENL lesions limited to sun-exposed areas has been reported previously.[1]


  Case Report Top


A 23-year-old female presented with complaints of an eruption over the face, upper chest, and upper limbs along with fever and body ache of 15 days duration. On dermatological examination, she was found to have multiple erythematous papules, nodules, and plaques distributed symmetrically over sun exposed sites over the face, “V” of chest, and extensor aspect of both arms and forearms [Figure 1] and [Figure 2]. She was also found to have ulnar clawing of the left hand, thickening of both ulnar and common peroneal nerves, and sensory loss in peripheral nerve trunk distribution. Clumps of acid-fast bacilli were found on slit skin smear, and the bacteriological index (BI) was found to be 4.3. Skin biopsy revealed a periadnexal, perineural and perivascular infiltrate of polymorphonuclear leukocytes as well as histiocytes in the dermis with fibrinoid necrosis [Figure 3] and modified Ziehl–Neelsen stain revealed multiple fragmented acid-fast bacilli. Direct immunofluorescence and antinuclear antibody tests were negative. A diagnosis of multibacillary leprosy with type 2 lepra reaction was made, and multidrug therapy (MDT) along with systemic corticosteroids was initiated. Constitutional symptoms, as well as ENL lesions, subsided within 2 weeks of initiating therapy.
Figure 1: Multiple erythematous papules, nodules, and plaques distributed symmetrically over the face and “V” of chest

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Figure 2: The extensor aspect of both arms and forearms are also involved

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Figure 3: Periadnexal and perivascular infiltrate of polymorphonuclear leukocytes as well as histiocytes in the dermis with fibrinoid necrosis (H and E, ×40)

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  Discussion Top


Type 2 lepra reaction is a humoral hypersensitivity response in multibacillary leprosy with the development of acute inflammatory foci at the site of antigen deposits in various tissues due to immune complex formation.[2]

The overall prevalence of type 2 lepra reaction among patients with lepromatous and borderline lepromatous patients has been reported to be 24% in Hyderabad, India.[3] Paradoxically, only 5% of lepromatous patients and borderline lepromatous patients develop type 2 lepra reaction in Shoa region of Ethiopia. Episodes of acute ENL develop in 19.2%, recurrent ENL in 10% of cases while chronic ENL develops in 70.7% of cases resulting in considerable morbidity.[4]

The risk factors in patients of lepromatous leprosy and borderline lepromatous leprosy for developing a type 2 lepra reaction include a higher BI of 3.5 as compared with a BI of 2 in controls. Another risk factor is the duration of MDT of 24.7 months in patients with type 2 lepra reaction which is significantly longer than the duration of MDT of 19.2 months in controls.[3] In our patient, the major risk factor for developing a type 2 lepra reaction is the relatively high BI of 4.3.

A type 2 lepra reaction is characterized by the crops of evanescent tender erythematous nodules and plaques along with fever and constitutional symptoms usually after the first 6 months of therapy. However, ENL may be the presenting feature in multibacillary leprosy [5] as was observed in our patient. In a retrospective hospital-based study, -34% (34.7%) of patients presented with ENL at the time of diagnosis.[4]

ENL lesions are distributed mainly over the flexural aspect of forearms and medial aspect of thighs and face.[6] However, in our patient, ENL lesions had an unusual distribution over sun exposed sites on the face, “V” of chest, and extensor aspect of arms and forearms. We hypothesize that the development of ENL lesions only on sun-exposed sites in our patient may be due to the deposits of lepra bacilli in the corium acting as haptens or incomplete antigens that were activated on ultraviolet exposure. These deposits of lepra bacilli may thus act as photo antigens with the formation of immune complexes and thereby ENL lesions limited to sun-exposed sites.

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.
Vijendran P, Verma R, Vasudevan B, Mitra D, Badad A, Neema S. Rare atypical presentations in type 2 lepra reaction: A case series. Int J Dermatol 2014;53:323-6.  Back to cited text no. 1
    
2.
Bryceson AD, Pfaltzgraff RE. Immunological complications: Reactions. In: Leprosy. 3rd ed., Ch. 8. Edingburgh: Churchill Livingstone; 1990. p. 115-26.  Back to cited text no. 2
    
3.
Pocaterra L, Jain S, Reddy R, Muzaffarullah S, Torres O, Suneetha S, et al . Clinical course of erythema nodosum leprosum: An 11-year cohort study in Hyderabad, India. Am J Trop Med Hyg 2006;74:868-79.  Back to cited text no. 3
    
4.
Walker SL, Lebas E, Doni SN, Lockwood DN, Lambert SM. The mortality associated with erythema nodosum leprosum in Ethiopia: A retrospective hospital-based study. PLoS Negl Trop Dis 2014;8:e2690.  Back to cited text no. 4
    
5.
Prabhu S, Shenoi SD, Pai SB, Sripathi H. Erythema nodosum leprosum as the presenting feature in multibacillary leprosy. Dermatol Online J 2009;15:15.  Back to cited text no. 5
    
6.
Jopling WH, McDougall AC. Leprosy reactions (reactional states). In: Handbook of Leprosy. 4th ed., Ch. 6. Oxford: Heinemann Medical Books; 1988. p. 68-82.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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