Reactive perforating leprosy: A rare case report of type 2 leprosy reaction

Hendra Gunawan; Nadilla Carisa Devi Nursjamsi; Pati Aji Achdiat; Oki Suwarsa

doi:10.4103/ijmy.ijmy_169_20

CASE REPORT

Year : 2020 | Volume : 9 | Issue : 4 | Page : 451-453

Reactive perforating leprosy: A rare case report of type 2 leprosy reaction

Hendra Gunawan, Nadilla Carisa Devi Nursjamsi, Pati Aji Achdiat, Oki Suwarsa
Department of Dermatology and Venereology, Faculty of Medicine, Padjadjaran University, Dr Hasan Sadikin Hospital, Bandung, Indonesia

Date of Submission	08-Sep-2020
Date of Acceptance	26-Sep-2020
Date of Web Publication	14-Dec-2020

Correspondence Address:
Hendra Gunawan
Jl. Pasteur 38, Bandung, West Java 40161
Indonesia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijmy.ijmy_169_20

Abstract

Reactive perforating leprosy (RPL) is a rare clinical manifestation of type 2 leprosy reaction. A case of lepromatous leprosy (LL) with type 2 leprosy reaction presented as RPL in one patient was reported. A physical examination showed multiple punched-out ulcers with regular border, without undermined edge, and dermal base. The ulcers mostly covered with hemorrhagic crust, contained pus, and necrotic tissue. A histopathological examination revealed invagination of the epidermis, intracorneal abscess, and infiltration of foamy macrophages with lymphocytes in dermis that supported the diagnosis of LL with RPL. The patient was given multidrug therapy-multibacillary and 40 mg prednisone daily which tapered off and wound dressing. Clinical improvement was observed within 2 weeks of treatment, as some ulcers healed. Type 2 leprosy reaction can provide a variety of clinical manifestations, one of which is RPL.

Keywords: Leprosy, punched-out ulcers, reactive perforating leprosy, type 2 leprosy reaction

How to cite this article:
Gunawan H, Devi Nursjamsi NC, Achdiat PA, Suwarsa O. Reactive perforating leprosy: A rare case report of type 2 leprosy reaction. Int J Mycobacteriol 2020;9:451-3

How to cite this URL:
Gunawan H, Devi Nursjamsi NC, Achdiat PA, Suwarsa O. Reactive perforating leprosy: A rare case report of type 2 leprosy reaction. Int J Mycobacteriol [serial online] 2020 [cited 2021 Jan 15];9:451-3. Available from: https://www.ijmyco.org/text.asp?2020/9/4/451/303443

Introduction

Leprosy reaction is an acute inflammatory condition in chronic leprosy, which can occur before, during, or after the treatment of leprosy. Based on the clinical and histopathological features, leprosy reaction can be divided into type 1 and type 2. The most common clinical manifestation of type 2 leprosy reactions is erythema nodosum leprosum, which is commonly found in lepromatous leprosy (LL) and borderline lepromatous as a result of the deposition of immune complex.^[1] Clinical manifestations of type 2 leprosy reaction as erythema multiforme-like, vesiculobullous, Sweet's syndrome-like,^[2] and reactive perforating leprosy (RPL) have also been reported.^[3],[4] RPL is a rare atypical form of type 2 leprosy reaction that has multiple punched-out ulcers as a clinical manifestation. The aim of this report was to show a rare case of RPL.

Case Report

A 24-year-old female patient presented with multiple punched-out ulcers on the face, hands, and legs. The patient had a history of erythematous macules on almost entire body that did not feel itchy nor painful for the last 8 months. The patient was informed that she had a relapsing leprosy and received World Health Organization (WHO) multibacillary multi-drug therapy (MB-MDT). One month later, there were bullae on both extremities which were treated with wet dressing. Two months before the consultation, there were painful erythematous papules and nodules on both extremities which quickly turned into punched-out ulcers. Then, the ulcers spread to both arms and face accompanied by pus. The patient had been diagnosed with leprosy since 2015 and had been treated with MDT-MB for 1 year and already released from treatment.

A physical examination showed moon face, madarosis on both eyebrows, saddle nose, and pitting edema on both dorsal aspects of the foot. There were multiple punched-out ulcers on the face, hands, legs, and some erythematous macules on the face, abdomen, and back [Figure 1] and [Figure 2]. Slit-skin smear (SSS) examination revealed a bacterial index of 4 + and morphological index 8.33%. Direct microscopic examination with Gram staining from the base of the ulcer revealed Gram-positive cocci. Histopathological examination results demonstrated invagination of the epidermis, intracorneal abscess, and infiltration of foamy macrophages with lymphocytes in the dermis [Figure 3]a and [Figure 3]b. The patient was diagnosed with LL relapse with type 2 reaction manifested as RPL.

Figure 1: Multiple ulcers and erythematous macules

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Figure 2: Punched-out ulcers as manifestation in reactive perforating leprosy

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Figure 3: Histopathological result (a) invagination of the epidermis, intracorneal abscess (b) infiltration of foamy macrophages with lymphocytes in the dermis (arrows)

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The patient received MDT-MB and 40 mg prednisone daily which tapered off and wound dressing. Clinical improvement was observed within 2 weeks after the treatment was started as some ulcers healed.

Discussion

Leprosy is a chronic granulomatous disease caused by the bacillus Mycobacterium leprae. It primarily affects the skin and peripheral nerves.^[5],[6] Although leprosy most commonly found in South-east Asia such as India and Indonesia, it is also endemic in the Southern United States with different clinical signs and symptoms,^[5] including multiple peroneal nerve abscesses that has been reported by Arora et al.^[6] Early detection of leprosy patient can prevent further disabilities and prompt treatment as early as possible.^[5],[7] Govindharaj et al. in 2017 conducted a study among 358 leprosy patients, and 40% of them had a physical disability.^[8] The challenges today also include methods to diagnose leprosy reaction and relapse.^[7] Beside the clinical symptom and SSS, skin biopsy must be conducted for supporting the diagnosis. Naaz et al. suggest that there is a new method using microsatellite typing to elucidate the type of M. leprae strain.^[9]

In the course of chronic leprosy, an acute phenomenon can occur called leprosy reaction. Leprosy reactions are immunologically mediated episodes that affect the skin, nerves, mucous membranes, and/or other sites. Type 2 leprosy reaction is a type 3 Coomb and Gell hypersensitivity reaction usually seen in lepromatous patients.^[1] There were many atypical forms of type 2 leprosy reaction.^[2] Vesiculobullous, pustular, ulcerated, hemorrhagic, and erythema multiforme-like lesions have been reported as the other form of type 2 leprosy reaction.^[4],[10] RPL is an atypical form of type 2 leprosy reaction that was first reported by Ghorpade in 2014 in India. He reported a case with multiple punched-out ulcers on a leprosy patient. The histopathological results showed a dense inflammatory infiltrate with numerous neutrophils that perforate the epidermis to form a sub-corneal pustule.^[3] We have been reported our first case of RPL in 2018 with similar clinical manifestation and histopathological results.^[10] In this case, the patient also presented with multiple painful punched-out ulcers and from histopathological examinations showed invagination of the epidermis, intracorneal abscess, and infiltration of foamy macrophages with lymphocytes in the dermis that supported the diagnosis of RPL.

Treatment strategies for type 2 leprosy reaction are aimed at controlling the acute inflammation of the skin and nerves, reducing the pain, and preventing further deformities and episodes. Type 2 leprosy reaction is said to be severe if there are: (1) Sudden eruption of red, tender papules, nodules, or plaques, which may ulcerate, (2) mild fever, (3) tender enlarged nerves, (4) increased loss of sensation or muscle power, (5) arthritis, (6) lymphadenitis, epididimo-orchitis, iridocyclitis, or episcleritis, (6) edema of extremities or face, and (7) positive Ryrie or Ellis test.^[1] The WHO Global Strategy document does not give specific advice concerning the dosage of prednisolone. We commonly started at a dose of prednisone 40 mg daily, and the dose of prednisone was slowly reduced 5–10 mg every 2 weeks with total duration of administration of 12 weeks.^[1],[3],[10] The patient in this case received MDT-MB and 40 mg prednisone daily which tapered off every 2 weeks and also received hydrocolloid and hydrogel wound dressing for the ulcers. Clinical improvement was observed within 2 weeks after the treatment was started as some ulcers healed and re-epithelization.

Conclusion

As one of the variant type 2 leprosy reactions, RPL should be considered as a diagnosis in leprosy patients who present with punched-out ulcers, and histopathological examination is an important tool to confirm the diagnosis of RPL.

Declaration of patient consent

The authors certify that they had obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images, and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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