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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 162
Bullous type II reaction in leprosy: A diagnostic dillemma


Department of Dermatology and Pathology, IMS and SUM Hospital, Bhubaneswar, Orissa, India

Date of Web Publication5-Mar-2013

Correspondence Address:
Bikash Ranjan Kar
Department of Dermatology and Pathology, IMS and SUM Hospital, Bhubaneswar, Orissa
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108101

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How to cite this article:
Kar BR, Dash K. Bullous type II reaction in leprosy: A diagnostic dillemma. Indian J Dermatol 2013;58:162

How to cite this URL:
Kar BR, Dash K. Bullous type II reaction in leprosy: A diagnostic dillemma. Indian J Dermatol [serial online] 2013 [cited 2019 Jun 26];58:162. Available from: http://www.e-ijd.org/text.asp?2013/58/2/162/108101


Sir,

Erythema nodosum leprosum (ENL) [1] is a common manifestation of Type II reaction in leprosy it is characterized by crops of tender, evanescent, subcutaneous nodules or plaques with a tendency for bilateral symmetrical distribution, associated with pain, fever and myalgia the extracutaneous features include iridocyclitis, orchitis, neuritis and lymphadenopathy. Vescicullobullous lesions in ENL are very rarely described mostly from Mexico and South American countries. From India there are few published papers on bullous lesions in type II reaction. [2] These types of lesions are of importance as they can be easily confused with various other vesicullobullous lesions of pemphigus, bullous pemphigoid, drug reactions etc.

A 40-year-old man on WHO Multidrug therapy for multibacillary disease (MB-MDT) comprising of Rifampicin 600 mg once a month, Dapsone 100mg once daily and Clofazimine 300 mg once a month supervised and 50 mg daily since last 8months, presented to the casualty with high-grade fever, joint pain along with multiple vesicullobullous lesions over trunk and extremities since last 3-4 days. He had one such episode 2 months back for which he was started on treatment but discontinued on his own the fever was high grade, intermittent in nature. No history of cough, pain abdomen or burning micturition. He was taking multidrug therapy regularly. There was no history of extensive skin exfoliation, mucosal involvement, intense pruritus, jaundice epistaxis, orchitis, neuritis or haematuria.

Cutaneous examination revealed diffuse symmetrical infiltrated shiny skin with brownish discoloration, infiltration of the ear lobules with few nodularities, glove and stocking type anaesthesia over extremities and bilateral thickened ulnar, radial cutaneous and lateral popliteal nerves. There was no motor weakness in face, upper limbs and lower limbs. He had multiple vesicullobullous lesions over trunk and extremities [Figure 1] the lesions were tense bullae, appearing over a diffusely infiltrated skin and were slightly tender. Nikolsky's sign and bulla spread sign were negative. Oral, conjunctival and genital mucosae were not involved.
Figure 1: Vesicullobullous lesions on extremities in Type II reaction of leprosy

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Routine investigations revealed hemoglobin of 11.2 gm%, mild leukocytosis, ESR of 64 mm after 1 hour. His average Bacillary Index (BI) was 4+. Skin biopsy done from one of the lesions revealed a sub epidermal bullous lesion [Figure 2] along with plenty of polymorphs and few foamy histiocytes the dermis showed multiple macrophage granulomas. Modified Fite's stain confirmed the presence of leprosy bacilli in the tissue [Figure 3] with a Bacillary Index of Granuloma (BIG) of 5+. Direct immunofluorescence (DIF) done from the lesional skin did not reveal any abnormal immunoglobulin deposits. A diagnosis of bullous type II reaction was made and the patient was admitted to the indoor and MDT was continued along with oral Prednisolone 60 mg per day. as he was getting multiple bullous lesions 4 days after starting treatment, oral Thalidomide was added at a dose of 100 mg thrice daily. Rapid improvement was seen after 48 hrs of starting Thalidomide. Thalidomide was gradually tapered down over a few weeks time and the patient remained symptom free after that at the end of 1 year of MDT the patient was free from Type II reactional episodes. He had 3 more episodes of Type II reaction in the second year but all the episodes were of lesser severity and responded to a tapering dose of oral Prednisolone.
Figure 2: Microphotograph showing sub epidermal Bullous lesion (H and E 40 × )

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Figure 3: AFB staining of leprosy bacilli (Modified Fite's, ×1200)

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Bullous lesions are rarely described in leprosy. [3] Infact other variants like ulcerated lesions; pustules, nodules and erythema multiforme like lesions are more frequently described. Bullous diseases like pemphigus and bullous pemphigoid are likely to create diagnostic confusion the absence of orogenital mucosal involvement and negative Nikolsky's sign and bulla spread signs clinically ruled out pemphigus. Bullous pemphigoid has a prodrome of urticarial lesions and lesions are intensely pruritic in both the conditions histopathology and immunoflurescence are of immense help to pinpoint the diagnosis the absence of immunoglobulin deposits in DIF ruled out autoimmune bullous disorders. Bullous lesions in leprosy mimicking pemphigus are described from India. [4] Bullous fixed drug reaction is another diagnostic dilemma the distribution of lesions with dominant involvement of mucocutaneous junctions and the dusky discoloration of the affected area and the histopathology with necrotic keratinocytes are confirmatory. the second point of emphasis in our case is the nonresponsiveness to oral prednisolone and rapid clearance of lesions with thalidomide. Thalidomide [5] is infact considered as the drug of choice for severe type II reactions in leprosy but because of the availability problems and the teratogenecity fear it is not often used in clinical situations where its use is warranted it serves two purposes. Patient is rapidly rendered asymptomatic and it also has got a steroid sparing effect. So it's a suitable alternative in cases where steroid toxicities are expected. [5]

Bullous reactions in leprosy are very rare. Few cases are reported from India. They can confuse easily with a wide array of vesicullobullous lesions. Proper history along with histopathology and immunoflurescence studies help to reach the diagnosis. Role of thalidomide in severe type II reactions is well established but efficacy over oral steroid needs to be confirmed by large well-controlled studies.

 
   References Top

1.Jopling WH, McDuggal AC. Leprosy reactions. In: Handbook of Leprosy. 5 th ed. New Delhi, India: CBS publication and distributors; p. 87.  Back to cited text no. 1
    
2.Dogra S, Kaur I, Kumar B. Bullous reaction in leprosy: A rare phenomenon. Int J Lepr Other Mycobact Dis 2002;70:206-7.  Back to cited text no. 2
[PUBMED]    
3.Petro TS. Bullous type of reaction mimicking pemphigus in lepromatous leprosy. Indian J Lepr 1996;68:179-81.  Back to cited text no. 3
[PUBMED]    
4.Sethuraman G, Jeevan D, Srinivas CR, Ramu G. Bullous erythema nodosum leprosum (bullous type 2 reaction). Int J Dermatol 2002;41:362-4.  Back to cited text no. 4
[PUBMED]    
5.Pannikar V. The return of thalidomide: New uses and renewed concerns. Lepr Rev 2003;74:286-8.  Back to cited text no. 5
[PUBMED]    


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  [Figure 1], [Figure 2], [Figure 3]



 

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