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Indian Pediatr 2017;54: 1071

Erythema Nodosum Leprosum Necroticans

 

*Deepika Pandhi and Anita Kulhari

Department of Dermatology and STD, University College of Medical Sciences (University of Delhi)
and Guru Teg Bahadur Hospital, Delhi, India.
Email:
[email protected]
 

 


A 14-year-old girl, resident of Uttar Pradesh, India, presented to us with multiple lesions appearing in crops recurrently for past 1 year. The lesions appeared over the normal appearing skin with a frequency of 6-7 reddish raised lesions per day and healed spontaneously in a period of 10-12 days leaving behind hyperpigmentation. Each episode was associated with high fever and generalized malaise. In this episode, patient noticed pustulation followed by ulceration over the lesion within 5 days of appearance of the crop. There was history of recurrent epistaxis for past one year and ear discharge prior to current exacerbation of lesions. There was no history suggestive of motor weakness, pain or edema over limbs, redness of eye, photophobia, frothy urine, palpitation, or dyspnea on exertion. Lesions of various morphology in form of pustules, nodules and plaques with ulceration and erosions in various stages of healing were present in a generalized fashion all over the body, predominantly involving the limbs (Fig. 1) and trunk. Diffuse infiltration was present over face and ears. Multiple peripheral nerves were thickened bilaterally. However, there was no glove and stocking anesthesia. The patient had received multiple courses of antibiotics with a diagnosis of recurrent furunculosis. Hematological and biochemical investigations were within normal limits and histopathology from nodular lesion was suggestive of erythema nodosum leprosum necroticans. Multibacillary multidrug therapy was initiated along with oral prednisolone 40 mg daily.


(a)

(b)

Fig. 1 Multiple erythematous nodular lesions along with pustules and ulcers with hemorrhagic crusting over forearms (a), and over legs (b).

 

The differential diagnoses for ulcerated nodules with fever would include furunculosis, mycobacterial infections and childhood vasculitis like childhood polyarteritis nodosa, benign cutaneous polyarteritis nodosa and wegeners vasculitis. Cutaneous polyarteritis nodosa is a relapsing chronic disease that presents with crops of painful, erythematous, subcutaneous nodules predominantly over the lower legs, with associated urticaria, livedo reticularis, peripheral gangrene, myalgia, arthralgia, non-erosive arthritis and peripheral neuropathy. Therefore it may closely mimic erythema nodosum leprosum and careful examination will detect diffuse infiltration especially over the face and nerve thickening or deformity suggestive of lepromatous leprosy. This case highlights the need to retain the focus on leprosy so that physicians can ensure early detection and treatment, including screening for family contacts, to reduce morbidity and decrease community burden of leprosy.

 

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