JURNAL READING Lucio Phenomenon (Sequelae of Neglected leprosy) JURNAL READING Lucio Phenomenon (Sequelae of Neglected leprosy) JURNAL READING Lucio Phenomenon (Sequelae of Neglected leprosy)

JURNAL READING Lucio Phenomenon (Sequelae of Neglected leprosy)

1. Journal Reading Morbus Hansen Subivision LUCIO PHENOMENON: SEQUELAE OF NEGLECTED LEPROSY PROGRAM PENDIDIKAN DOKTER SPESIALIS-1 PROGRAM STUDI DERMATOLOGI DAN VENEREOLOGI FAKULTAS KEDOKTERAN UNIVERSITAS HASANUDDIN MAKASSAR 2024 Read by: dr. Suci Dewi Putri C115232003 Advisor: dr. Safruddin Amin, Sp.D.V.E , Subsp.DT ,MARS, FINSDV, FAADV Dr. dr. Anni Adriani , Sp. D.V.E, Subsp. DT,. FINSDV, FAADV

2. 2 Journal Korean Journal of Family Medicine Year 2021 Authors Siti Nuryati Che Ya Rosediani Muhamad Rosnani Zakaria Azlina Ishak Wan Noor Hasbee Wan Abdullah Volume 42 Issue 3 Article Category Case Report

3. 3 ABSTRACT Lucio phenomenon is a potentially fatal leprosy reaction seen in pure, primitive, and diffuse form of lepromatous leprosy Globally restricted phenomenon that occurs in Mexico and Costa Rica; however, sporadic cases have been reported elsewhere. We report a rare case of a 50-year-old Indonesian, who resided in Malaysia for the past 33 years, which presented with extensive ulcers on all four limbs for 3 weeks, as well as a 16-year history of skin changes and alopecia. Delay in diagnosis  significant disabilities and disease transmission. Full evaluation established the diagnosis of lepromatous leprosy with Lucio phenomenon.

4. 4 INTRODUCTION 1948 by Latapi and Zamora  describing it as histopathologic vasculitis with exclusive occurrence in untreated, diffuse non-nodular lepromatous leprosy. LUCIO LEPROSY Pure, primitive, and diffuse non-nodular form of lepromatous leprosy. Rare, aggressive, and occasionally fatal type-II lepra reaction termed “ erythema necroticans ” that occurs in the course of Lucio leprosy. First described in 1852 by Lucio and Alvarado as a necrotizing skin reaction affecting patients with non-nodular lepromatous leprosy in Mexico.

5. 5 LUCIO PHENOMENON Clinically characterized by necrotic hemorrhagic lesions on the extremities and trunk Reported primarily in Mexico and Costa Rica Sporadic cases  Brazil, Argentina, India, and Singapore First Lucio phenomenon case series in Malaysia was reported in 2009, where 1 out of 3 patients succumbed to the disease. Our patient, an Indonesian , has been a resident of Malaysia for the past 33 years and survived the ordeal. INTRODUCTION

6. 6 CASE REPORT Day 3 of the illness  visited a local general practitioner  prescribed oral antibiotics and a topical antifungal . 50-year-old Indonesian gentleman Worked as a laborer at a remote local estate in Malaysia for the preceding 33 years 2-yearly short visits to Indonesia. Extensive ulcers (upper and lower limbs) The ulcers were painful , with rapid progression over 3 weeks. Associated with fever and rigor . Since 2002 , a d iffuse skin changes, nail loss, elongated ears, and patchy alopecia were noted  did not seek treatment

7. 7 CASE REPORT Waxy infiltration with leonine facies, madarosis, rhinophyma, granulomatous lesion over left alae nasi Left third nerve palsy. Thickened and elongated earlobes PHYSICAL EXAMINATION Febrile Hemodynamically stable Skin  darkened with hyperesthesia and anhidrosis

8. 8 CASE REPORT Widely disseminated necrotic ulcerated hemorrhagic ulcers with serous discharge on both lower limbs. Areas of necrotic eschar and achromic scars were noted Dactylitis with sloughing of all fingers. Axillary nerves were found to be enlarged upon palpation.

9. 9 CASE REPORT Laboratory Result Hemogram : microcytic hypochromic anemia Hemoglobin 10.1 g/dL Mean corpuscular volume 67 fL Mean corpuscular hemoglobin 21 pg White blood cell (normal) 11.6×103/uL Predominantly neutrophils 71% Platelet count (normal) 336×10 Laboratory Result Iron study Serum iron (reduced) 4.8 mcmol /L Hypochromic microcytic anemia secondary to iron deficiency anemia or chronic disease Laboratory Result Hypoalbuminemia 32 g/L Liver enzymes Normal Renal function Normal glucose-6-phosphate-dehydrogenase Normal Swab culture from the right leg grew Pseudomonas aeruginosa

10. 10 CASE REPORT Filtration by neutrophils associated with leucocytoclasis and extravasation of red blood cells Fibrinoid necrosis of blood vessel wall  Vascular thrombosis Positive for acid fast bacilli A SKIN BIOPSYOF THE RIGHT CHEST Ziehl- Neelson stain , original magnification [OM] ×400) ( Wade Fite stain , OM ×400 Slit skin smear from the earlobes indicated a bacterial index of 4.0 (average, 10–1000/field) and a morphological index of 3.0.

11. 11 CASE REPORT Lepromatous leprosy (Ridley-Jopling classification) with Lucio phenomenon complicated with bacterial infection World Health Organisation (WHO) guidelines MDT for multibacillary leprosy Rifampicin 600 mg/month Continued for 18 month Dapsone 100 mg/day Clofazimine 50 mg/day 300 mg/month Combination with Prednisolone 0.5 mg/kg/day for 2 weeks  tapered off due to potential reactions Intravenous Cefuroxime cover the Pseudomonas infection DIAGNOSIS Wound debridement was performed, followed by daily dressing with potassium permanganate solution

12. 12 CASE REPORT

13. 13 DISCUSSION Leprosy patients tend to have reactions based on the pattern of the immune response mounted against the bacilli, and the reactions are further subcategorized based on the mechanism LUCIO PHENOMENON Occurs in the lepromatous version  unresponsiveness of the immune system  unrestricted proliferation of Mycobacterium sp .  manifest distinct clinic-pathologic features Monteiro et al.  histopathologic characteristics is due to the inflammatory reactions and changes in the coagulation systems causing vascular thrombosis, ischemia, infarction, and tissue necrosis.

14. 14 DISCUSSION The predilection of the diffuse lesion is mainly extremities, which may include nodules and heal with atrophic stellate scars, as seen in our patient . The term “Lucio phenomenon” should strictly be used when there is a correlation between clinical and anatomic findings and in accordance with strict clinical criteria Our patient met the 3 criteria Skin ulceration Vascular thrombosis Invasion of blood vessels by Hansen bacilli Characterized by 1. E xistence of immune complexes 2. N ecrotizing vasculitis on superficial and mediumsized vessels 3. Diffuse infiltration of the skin 4. D ermal necrosis 5. Sometimes systemic symptoms

15. 15 DISCUSSION Our patient was treated as having a fungal infection that was superimposed with a bacterial infection on day 3 of illness by the local primary care doctor despite having common signs : Madarosis, Thickened earlobes Sloughing of the fingernails. DIFFERENTIAL DIAGNOSIS Vasculitides Thrombotic vasculopathy Pyoderma gangrenosum Connective tissue disease Kwan et al.  in Malaysia from 27 cases (2008-2013)  up to 44% of cases were misdiagnosed. Rea and Jerskey case series  From 30 patient only 4 were diagnosed with leprosy before the development of the Lucio phenomenon  not be easily recognized in non-endemic countries

16. 16 DISCUSSION The cases from non-endemic countries have mostly presented with painless progressive necrotic ulcers and systemic symptoms. Common manifestation of lepromatous leprosy Loss of eyebrows (100%) Loss of eyelashes Rarely Loss of body and scalp hair Thickening of facial skin and earlobes Waxy infiltrated myxedematous appearance of the skin Peripheral neuropathy Destructive rhinitis Choon and Tey and Curi et al Atypical features  hepatosplenomegaly and generalized lymphadenopathy Lucio phenomenon commonly appears between 1 and 3 years after manifestation

17. 17 DISCUSSION Clinical signs are easily ignored  until more evident findings of the Lucio phenomenon occur. Our patient  classical manifestation onset 16 years ago before he sought treatment when the Lucio phenomenon developed. Lucio phenomenon necessitates the usual multidrug regiment as an effective first-line treatment with systemic corticosteroid to be included in the regimen for severe situations. Prognostic factors and exact mortality rate cannot be predicted  small number of cases reported Response to the treatment appears to be poor

18. 18 DISCUSSION Our case highlighted the diagnostic dilemma of lepromatous leprosy in non-endemic areas It may masquerade as many conditions and should be included in the differential diagnoses It is imminent to train the front-liners in non-endemic countries to be vigilant and have a high index of suspicion Timely diagnosis with the help of slit skin smears and skin biopsies will allow for early institution of appropriate therapy. Levels of community information and education about this neglected tropical disease need to be increased.

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