Necrotic Erythema Nodosum Leprosum Healing with Extensive Scars.

Sir,

Type-II leprareaction [erythema nodosum leprosum (ENL)] is a type III hypersensitivity reaction which commonly manifests as sudden appearance of crops of erythematous, edematous, evanescent, and tender nodules and plaques associated with fever and joint pain. ENL is associated with involvement of multiple organs leading to various complications. We are reporting a case of necrotic ENL healing with extensive scars associated with significant submental and inguinal lymphadenopathy.

A 24-year-old woman presented with fever, joint pain, and tender skin lesion with ulceration since 20 days. She was diagnosed as a case of lepromatous leprosy with ENL and was on treatment with World Health Organization (WHO) multi drug therapy (MDT) multibacillary (MB adult) regimen since 6 months along with oral prednisolone. During a previous episode, she had nodular ENL, for which she was on oral prednisolone (20–40 mg) since 2.5 months. After sudden stoppage of oral prednisolone, she noticed painful nodules and bullae associated with fever which became ulcerated within 3–4 days in contrast to the previous episode. There was no history of leprosy among family members. On dermatological examination, there were multiple non-tender nodules over the face, ears, and trunk with loss of sensation over bilateral hands and feet [Figure 1a]. Moreover, the patient had erythematous, tender nodules, and few bullae over extremities and trunk. Majority of the lesions over extremities and buttocks were ulcerated with punched out margins having necrotic tissue on the floor [Figure 1a and b]. Systemic examination revealed tender matted submental and inguinal lymphnodes whereas other systems were apparently normal [Figure 2]. Routine hematological parameters were within normal limits. Her slit skin smear for acid fast bacilli (AFB) was 4+ and histopathology of the ulcerated nodule showed ulcerated epidermis and the dermis showed proliferation of multiple blood vessels with dense infiltration of neutrophils and other chronic inflammatory cells without any acid-fast bacilli [Figure 3a and b]. Based upon clinical and histopathological features, the patient was diagnosed with lepromatous leprosy along with severe necrotic ENL. Prednisolone (50 mg) along with WHO, MDT (MB) adult regimen was started. The ulcers healed over 3 weeks forming extensive linear hypopigmented and hypertrophic scars over the extremities with significant reduction in the size of the lymphnodes [Figure 4a–c]. Currently, the patient is on oral prednisolone and WHO MDT.

Figure 1

(a) Multiple ulcers with punched out marginsand necrotic tissue on the floor and nodules over bilateral upper extremities. (b) Ulcers with punched out margins

Figure 2

Enlarged inguinal lymphnode

Figure 3

(a and b) Histopathology revealing ulcerated epidermis, proliferation of blood vessels, and neutrophilic infiltration around blood vessels, dermis, and subcutis (Hematoxylin and eosin; a: ×10 and b:×40)

Figure 4

(a and b) Linear hypopigmented and hypertrophic scars over lower extremities after healing of ulcers. (c) Decrease in the size of inguinal lymphnode after treatment

ENL is an immune complex-mediated type III hypersensitivity reaction involving antigen, antibody, and complements. Neutrophilic activation and T-cell dysfunction occurs. It manifests with erythematous, edematous, evanescent, and tender nodules in crops associated with systemic manifestations.[1] There are various morphological patterns of ENL such as nodular, bullous, pustular, and necrotic, with nodular form being the most common.[2] In the present case, the patient presented with both bullous and nodular lesions which lead to necrotic ENL in the form of severe ulceration. Immunecomplex-mediated damage can lead to the involvement of various organs leading to uveitis, lymphadenitis, synovitis, mastitis, epididymo-orchitis, arthtitis, glomerulonephritis, and hepatitis.[3] Necrotic ENL represents a severe form of ENL and is associated with systemic complications.

Apart from the complications of ENL, leprosy patients can have other deformities such as foot drop, claw hand, shortening of digits, and non-healing ulcers. In the present case, the patient had necrotic ENL with painful ulcers over the extremities and buttocks, tender inguinal, and submental lymphadenopathy. The close differential diagnosis of the present case is lucio phenomenon, which occurs in patients with diffuse form of leprosy and presents with purpuric lesions followed by ulcers with irregular margins. Lucio phenomenon is characterized by absence of constitutional and systemic symptoms. On histopathology there is ischaemic epidermal necrosis, necrotizing vasculitis, endothelial proliferation, and presence of a large number of AFB in endothelial cells. Lucio phenomenon responds to MDT.[4] In our case, the patient had systemic symptoms with multiple nodules getting ulcerated and AFB was absent in histopathology. The patient was already on MDT when she developed nodules and ulcers which later responded to oral steroids. After treatment, the ulcers healed with extensive linear hypopigmented and hypertrophic scar formation over extremities. Hypertrophic scar is an abnormal tissue response to injury leading to excess collagen formation and at times can lead to contracture of the affected parts hindering the movement.[5] Extensive linear hypopigmented and hypertrophic scars over the extremities as a sequel of necrotic ENL is cosmetically unappealing for a young girl and can hamper the daily activities. It also adds to the suffering of a patient who is already psychologically handicapped with leprosy. The case is interesting both for necrotic ENL which is rarely found and because of formation of extensive scars as a complication along with significant lymphadenopathy.

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Conflicts of interest

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