|Year : 2018 | Volume
| Issue : 2 | Page : 191-194
Erythema nodosum leprosum presenting as sweet's syndrome-like reaction in a borderline lepromatous leprosy patient
Nyoman Suryawati1, Herman Saputra2
1 Department of Dermatology and Venereology, Faculty of Medicine, Udayana University, Sanglah General Hospital, Denpasar, Indonesia
2 Department of Pathological Anatomy, Faculty of Medicine, Udayana University, Sanglah General Hospital, Denpasar, Indonesia
|Date of Web Publication||13-Jun-2018|
Department of Dermatology and Venereology, Faculty of Medicine, Udayana University, Sanglah General Hospital, Diponegoro Street, Denpasar, Bali, 80113
Source of Support: None, Conflict of Interest: None
Erythema nodosum leprosum (ENL) is characterized by tender erythematous nodules, accompanied by fever, malaise, arthralgia, and systemic complications. Atypical clinical manifestations have been reported such as pustular, bullous, ulceration; livedo reticularis; erythema multiforme-like reaction; and Sweet's syndrome (SS)-like presentation. We reported a case of ENL reaction presenting as SS-like reaction in a borderline lepromatous leprosy patient.
Keywords: Atypical leprosy reaction, erythema nodosum leprosum, Sweet's syndrome-like reaction
|How to cite this article:|
Suryawati N, Saputra H. Erythema nodosum leprosum presenting as sweet's syndrome-like reaction in a borderline lepromatous leprosy patient. Int J Mycobacteriol 2018;7:191-4
|How to cite this URL:|
Suryawati N, Saputra H. Erythema nodosum leprosum presenting as sweet's syndrome-like reaction in a borderline lepromatous leprosy patient. Int J Mycobacteriol [serial online] 2018 [cited 2021 Jul 25];7:191-4. Available from: https://www.ijmyco.org/text.asp?2018/7/2/191/234327
| Introduction|| |
Leprosy is a chronic infectious disease caused by Mycobacterium leprae, characterized by involvement of the skin and peripheral nerves. In the course of leprosy, a patient could obtain an acute inflammatory episode called leprosy reaction. This result can occur before, during, or after the treatment of multiple drug therapy (MDT). There are three types of leprosy reactions: type 1 reaction (reversal reaction/RR), type 2 reaction (erythema nodosum leprosum/ENL), and Lucio's phenomenon. RR mainly occurs in borderline-type leprosy, whereas ENL is more common in borderline lepromatous (BL)-type leprosy and lepromatous-type leprosy.
ENL is characterized by tender erythematous nodules accompanied by constitutional symptoms such as fever, malaise, arthralgia, as well as systemic complications. There are reports of atypical and rare clinical manifestations such as pustular,, bullous,, ulceration; livedo reticularis; erythema multiforme (EM)-like reaction;, or Sweet's syndrome (SS)-like presentation.,,,, This article will present a case of ENL presenting as SS-like reaction in a BL leprosy patient.
| Case Report|| |
A 37-year-old Javanese male complained of painful red bumps all over the body with fever and joint pain since 3 days before the hospitalization. The lumps began at the face and spread almost the entire body. A year earlier, he was diagnosed with BL-type leprosy. He received treatment of MDT package for 8 months, but the medications were terminated by himself.
Upon examination, the general condition was weak but fully conscious (compos mentis). His blood pressure was 120/80 mmHg and his temperature was 38°C. His respiration rate was 20 times per minute. Dermatological status showed edematous appearance of the face, accompanied by papules and multiple erythematous plaques with vesicles in the middle, some with pus. Numerous erythematous nodules measuring 0.5–1 cm, erythematous papules, and erythematous plaques with vesicles in the middle were present in the body and extremities [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e. On physical examination, madarosis, tenar and hypothenar atrophy, and enlargement of auricularis magnus nerve and ulnar nerve were found.
|Figure 1: (a) Clinical manifestation of the patient, there were multiple papules, nodules, and erythematous plaques with edematous skin, some parts with pseudovesicles, pustules, and erosions covered with hemorrhagic crusts (right face). (b) Clinical manifestation of the patient, there were multiple papules, nodules, and erythematous plaques with edematous skin, some parts with pseudovesicles, pustules, and erosions covered with hemorrhagic crusts (left face). (c) Clinical manifestation of the patient, there were multiple papules, nodules, and erythematous plaques with edematous skin, some parts with pseudovesicles (hands). (d) Clinical manifestation of the patient, there were multiple papules, nodules, and erythematous plaques with edematous skin, some parts with pseudovesicles (abdomen). (e) Clinical manifestation of the patient, there were multiple papules, nodules, and erythematous plaques with edematous skin, some parts with pseudovesicles (back and waist)|
Click here to view
Blood test results showed leukocytosis of 28.860/μL, neutrophilia of 88.4%, anemia (hemoglobin) of 8 g/dL, and erythrocyte sedimentation rate of 110 mm/h. His random blood glucose was 124 mg/dL, and 2-h postprandial blood glucose was 150 mg/dl. His liver and kidney function tests were within normal limits. Urine test presented leukocytes 3–6 per high-power field and bacteria. Examination of pus showed leukocytes 1–2 per high-power field and Gram-positive cocci with the result of pus culture revealing Staphylococcus epidermidis. Peripheral blood smear test showed microcytic normochromic anemia, with leukocytosis and reactive thrombocytosis. Acid-fast bacillus test showed bacillus with bacterial index (BI) of + 4 [Figure 2].
|Figure 2: Examination of acid-fast bacilli showed bacillus with bacterial index +4|
Click here to view
Histopathological examination showed edema of the papillary dermis and intraepidermal vesicles [Figure 3]a. There were neutrophilic infiltrates and nuclear dust that diffusely scattered around eccrine glands and blood vessels and extravasation of erythrocytes [Figure 3]b and [Figure 3]c. There were lymphocyte, eosinophil, and histiocyte infiltrates, with some of the histiocytes appearing as phagocyting nuclear debris and rod-shaped bacteria. Some parts of the histiocytes presented in cluster arrangement/granuloma [Figure 3]d. Electromyogram examination result presented mixed polyneuropathy (axonal and demyelinating).
|Figure 3: (a) Histopathologic result showing edema of the papillary dermis and intraepidermal vesicles. (b and c) There were neutrophilic infiltrates and nuclear dust that diffusely scattered around eccrine glands and blood vessels, with extravasation of erythrocytes. (d) There were lymphocyte, eosinophil, and histiocyte infiltrates, with some of the histiocytes appearing as phagocyting nuclear debris and rod-shaped bacteria. Some parts of histiocytes presented in cluster arrangement/granuloma|
Click here to view
The diagnosis of this patient revealed ENL reaction presenting as SS-like reaction and anemia of chronic disease. The patient's condition had improved with methylprednisolone therapy 8 mg three times per day, cefotaxime 1 g three times per day (intravenously), and the provision of MDT for 12 months.
| Discussion|| |
ENL is a type III hypersensitivity reaction (Coombs and Gell) associated with bacterial destruction and the release of large quantities of antigen, which induces antibody production. Antigens from dead bacterial products react with antibodies and form antigen–antibody complexes. These antigen–antibody complexes will activate complementary reaction which causes acute inflammation of tissue in the form of erythema nodules. The clinical signs of ENL are tender erythematous nodules with constitutional symptoms, but there are reports of atypical and rare clinical manifestations such as pustular,, bullous,, ulceration; livedo reticularis; EM-like reaction;, or SS-like reaction.,,,,
SS or acute febrile neutrophilic dermatosis is a condition characterized by papules, tender erythematous nodules, and plaques with or without pseudo-vesiculation with constitutional symptoms such as fever and malaise. Characteristics of SS are the positive finding of leukocytosis with neutrophilia, histopathologic examination of neutrophilic infiltrates in the dermis without evidence of leukocytoclastic vasculitis, and good response to corticosteroids [Table 1].
SS-like leprosy presentation is rarely reported, making it difficult to diagnose, especially in patients who have not been diagnosed with leprosy previously. Kun and Chan reported this variant for the first time in 1987, which described patients that clinically and histopathologically consistent with lepromatous leprosy and SS. After that, only some cases have been reported with this type of reaction.,,, SS-like leprosy reactions are classified as type 2 leprosy reactions and may occur in borderline-type leprosy or lepromatous type,,,, but they were reported more commonly in borderline-type leprosy due to instability of immune system in the body.
Clinical diagnosis of SS-like leprosy reaction is difficult to enforce if edematous plaques dominate the skin lesions, often difficult to distinguish it from type 1 reactions. SS-like reaction has characteristic histopathology such as neutrophilic infiltrates, especially in the dermal papilla, accompanied by pronounced edema that resembles SS, resulting in frequent misdiagnosis of SS. To have a proper diagnosis, pathologists should look for the presence of Virchow's cells in staining with hematoxylin-eosin and Fite-Faraco. In this case, the diagnosis of SS-like leprosy reaction was based on the patient's history of leprosy with previous inadequate therapy, the discovery of leprosy symptoms such as madarosis, nerve thickening, and atrophic muscles of the tenar and hypothenar, the detection of acid-resistant bacilli, and supported by histopathologic results.
In ENL reaction, there were significantly increased interleukin (IL-4), IL-5, IL-10, IL-7, and tumor necrosis factor (TNF)-α levels. The most severe reaction was associated with increased production of TNF-α and interferon-γ. There is an imbalanced in Th 17 and Treg populations, which shows the important role of Treg in controlling the inflammatory response during leprosy reactions. Vieira et al. stated Tregs down modulation may affect the development of Th-17 response that characterizes this reactions. A study by Hungria et al. (2017) showed anti-LID-1 (leprosy IDRI diagnostic-1) serology at diagnosis has shown prognostic value for ENL development in BI positive patients with a sensitivity level of 71% and a specificity of 80%.
SS-like leprosy responds well to corticosteroid therapy, but it is advisable to administer thalidomide to prevent worsening of lesions and prevent the onset of corticosteroid withdrawal. Thalidomide is the drug of choice for severe ENL due to its anti-Tumor Necrosis Factor-α (TNF-α) effect.,, Patients with chronic ENL reactions may be susceptible to corticosteroids dependence, therefore thalidomide is the best option to prevent long-term corticosteroid side effects.,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his 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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Ramesh V, Pahwa M. Some unusual type 2 reactions in leprosy. Int J Dermatol 2010;49:172-5.
Kar H, Sharma P. Leprosy reaction. In: IAL Textbook of Leprosy. 1st
. ed. New Delhi: Jaype Brothers; 2010. p. 269-89.
Ghorpade A. Reactive perforating leprosy: Pustular erythema nodosum leprosum with epidermal perforation and liberation of Mycobacterium leprae. Int J Dermatol 2014;53:221-3.
Gunawan H, Yogya Y, Hafinah R, Marsella R, Ermawaty D, Suwarsa O, et al
. Reactive perforating leprosy, erythema multiforme-like reactions, sweet's syndrome-like reactions as atypical clinical manifestations of Type 2 leprosy reaction. Int J Mycobacteriol 2018;7:97-100.
] [Full text]
Vashisht D, Das AL. Bullous erythema nodosum leprosum. Med J Armed Forces India 2013;69:71-3.
Yogeesh H HRY, Chankramath S, Yadalla HKK, Shariff S, Ramesh SB, Sreekantaiah SB. Type 2 lepra reaction (ENL) presenting with extensive cutaneous ulcerations. Our Dermatol Online 2012;1:17-20.
Naveen KN, Athanikar SB, Hegde SP, Athanikar VS. Livedo reticularis in type 2 lepra reaction: A rare presentation. Indian Dermatol Online J 2014;5:182-4.
] [Full text]
Sgambatti S, Andrade J, Sousa A, Stefani M, Costa M, Andrade A. An unusual presentation of leprosy at diagnosis: Erythema multiforme-like type 2 reaction. Rev Patalogia Trop 2010;39:221-7.
Prakruthi K, Rajesh G, Rajashekhar T, Jose A. An unusual presentation of type 2 lepra reaction: Mimicking Sweet's Syndrome. J Clin Biomed Sci 2015;5:42-4.
Chiaratti FC, Daxbacher EL, Neumann AB, Jeunon T. Type 2 leprosy reaction with Sweet's syndrome-like presentation. An Bras Dermatol 2016;91:345-9.
Vijendran P, Verma R, Vasudevan B, Mitra D, Badad A, Neema S, et al
. Rare atypical presentations in Type 2 lepra reaction: A case series. Int J Dermatol 2014;53:32-6.
Mahajan VK, Abhina C, Mehta KS, Cauhan PS. Erythema nodosum leprosum mimicking Sweet's syndrome. Lepr Rev 2014;85:322-7.
Das T, Ghosh S, Kundu AK, Maity A. Reactional State in Lepromatous Leprosy Simulating Sweet's Syndrome. J Assoc Physicians India 2013;61:856-8.
Heng YK, Chiam YT, Giam YC, Chong WS. Lepromatous leprosy in erythema nodosum leprosum reaction mimicking Sweet's syndrome. Int J Dermatol 2011;50:1124-5.
Aires NB, Refkalefsky Loureiro W, Villela MA, Sakai Valente NY, Trindade MA. Sweet's syndrome type leprosy reaction. J Eur Acad Dermatol Venereol 2009;23:467-9.
Fonseca AB, Simon MD, Cazzaniga RA, de Moura TR, de Almeida RP, Duthie MS, et al
. The influence of innate and adaptative immune responses on the differential clinical outcomes of leprosy. Infect Dis Poverty 2017;6:1-8.
Vieira AP, Trindade MÂB, Pagliari C, Avancini J, Sakai-Valente NY, Duarte AJ da S, et al
. Development of Type 2, But Not Type 1, Leprosy Reactions is Associated with a Severe Reduction of Circulating and In situ
Regulatory T-Cells. Am J Trop Med Hyg 2016;94:721-7.
Hungria EM, Bührer-Sékula S, de Oliveira RM, Aderaldo LC, Pontes A de A, Cruz R, et al
. Leprosy reactions: The predictive value of Mycobacterium leprae-specific serology evaluated in a Brazilian cohort of leprosy patients (U-MDT/CT-BR). Johnson C, editor. PLoS Negl Trop Dis 2017;11:e0005396.
Wu JJ, Huang DB, Pang KR, Hsu S, Tyring SK. Thalidomide: Dermatological indications, mechanisms of action and side-effects. Br J Dermatol 2005;153:254-73.
Walker SL, Waters MF, Lockwood DN. The role of thalidomide in the management of erythema nodosum leprosum. Lepr Rev 2007;78:197-215.
[Figure 1], [Figure 2], [Figure 3]