|Year : 2022 | Volume
| Issue : 5 | Page : 760-763
Lucio phenomenon in a case of lepromatous leprosy
Saba Mohammed Musaddique Ansari, Uddhao Suresh Zambare, Ankit Gupta, Chitra Shivanand Nayak
Department of Dermatology, T. N. M. C. and B. Y. L. Nair Hospital, Mumbai, Maharashtra, India
|Date of Submission||05-Oct-2020|
|Date of Decision||04-May-2021|
|Date of Acceptance||04-May-2021|
|Date of Web Publication||01-Jun-2022|
Uddhao Suresh Zambare
OPD No-2-37, 2nd Floor, OPD Building, Nair Hospital, Mumbai Central, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
Leprosy is a chronic infectious disease caused by Mycobacterium leprae (M. leprae), mainly affecting skin and nerves. Lucio leprosy (LuLp) is a pure primitive diffuse nonnodular form of leprosy. Lucio phenomenon (LP) is a type of necrotizing reaction seen in untreated or inadequately treated cases of LuLp. We present a case of LP in a patient of lepromatous leprosy. A 55-year-old male presented with hemorrhagic bullae, angulated ulcers with jagged margins on lower limbs, purpuric patches on extremities, trunk, and face, and erythematous plaques with intact sensations on back. LP was diagnosed on the basis of clinicohistopathological features.
Keywords: Lepromatous leprosy, lucio phenomenon, Mycobacterium leprae
|How to cite this article:|
Musaddique Ansari SM, Zambare US, Gupta A, Nayak CS. Lucio phenomenon in a case of lepromatous leprosy. Med J DY Patil Vidyapeeth 2022;15:760-3
|How to cite this URL:|
Musaddique Ansari SM, Zambare US, Gupta A, Nayak CS. Lucio phenomenon in a case of lepromatous leprosy. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2023 Jan 30];15:760-3. Available from: https://www.mjdrdypv.org/text.asp?2022/15/5/760/346452
| Introduction|| |
Mycobacterium leprae (M. leprae) is the causative agent of leprosy, a chronic infectious disease transmitted by inhalation of bacilli mainly affecting the skin and nerves.
Lucio leprosy (LuLp) was first described by Lucio and Alvarado in 1852 as lazarine leprosy or spotted (Manchada) leprosy which presents as diffuse cutaneous infiltration without nodule formation, frequently complicated by reactional episodes presenting with scarlet spots that darken and may ulcerate later, leaving atrophic and hypochromic scars with a thin hyperpigmented border. Later in 1948, the original description by Lucio and Alvarado was elaborated by Latapi and Zamora who coined the term “LuLp” which is a pure primitive diffuse nonnodular form of leprosy, characterized by diffuse nonnodular infiltration of the skin with shiny appearance which may become atrophic later. Lucio phenomenon (LP) is a specific type of reaction seen only in patients with untreated or inadequately treated LuLp. Here, we present a case of LP in a patient of lepromatous leprosy (LL).
| Case Report|| |
A 55-year-old nondiabetic and nonhypertensive male presented with multiple bullae, erosions, ulcers, purpuric patches, blackening of skin, and swelling of both the legs and dorsum of feet for 5 days. There was no history of addictions, fever, arthralgia, or any other constitutional symptoms. He had a history of recurrent ulceration over both heels for 5 years but no history suggestive of taking antileprosy treatment anytime in the past.
Cutaneous examination showed multiple tense hemorrhagic bullae, wet erosions, bizarre, and angulated ulcers with ragged margins and yellowish slough on the floor symmetrically distributed on both legs. Multiple bizarre purpuric patches were distributed symmetrically on all extremities, trunk, pinnae, and face. Face showed diffuse infiltration with shiny appearance. There were multiple erythematous infiltrated plaques with intact sensations on the back [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f. Nodules or hypopigmented patches were not seen.
|Figure 1: (a) Diffuse infiltration with shiny appearance of face without nodule formation. (b) Multiple ill-defined erythematous infiltrated plaques on the back. (c) Resorption of distal phalanges of left little, ring, and middle fingers. (d-f) Multiple tense hemorrhagic bullae, wet erosions, bizarre, and angulated ulcers with ragged margins and yellowish slough on the floor with multiple bizarre purpuric patches on anterior and posterior aspect of both legs and dorsum of both foot, respectively|
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Symmetrical glove and stocking type of anesthesia, ciliary and superciliary madarosis were present. Ulnar, radial, radial cutaneous, and posterior tibial nerves were symmetrically and moderately thickened (Grade 2) on both sides. Cranial nerve examination was normal. Resorption of left middle, ring, little fingers, and tips of left great and second toe were seen. Systemic examination was normal.
Slit skin smear (SSS) examination from both the earlobes showed bacterial index of 6+ with globi formation.
Biopsy (H and E) from erythematous infiltrated plaque on back showed atrophic epidermis, free grenz zone with multiple perivascular and periappendageal granulomas with foamy macrophages, suggestive of BL leprosy [Figure 2]a and [Figure 2]b.
|Figure 2: (a) Biopsy from erythematous infiltrated plaque on back showed atrophic epidermis, free grenz zone with multiple perivascular, and periappendageal granulomas (H and E, ×100). (b) Well-defined periappandageal granuloma consisting of foamy macrophages (H and E, ×400). (c) Fite-Faraco stain from erythematous infiltrated plaque showed numerous acid-fast lepra bacilli lying singly and in clumps forming globi|
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Biopsy (H and E) from the ulcer margin showed an ulcerated epidermis and dermis along with foamy macrophages, ischemic necrotizing vasculitis, fibrinoid necrosis, and presence of clumps of acid-fast bacilli periadnexally, perivascularly, and within macrophages and endothelial cells [Figure 3]a and [Figure 3]b.
|Figure 3: (a) Biopsy from the ulcer margin showed an ulcerated epidermis and dermis along with foamy macrophages, ischemic necrotizing vasculitis, fibrinoid necrosis, and presence of clumps of acid-fast bacilli periadnexally, perivascularly, and within macrophages and endothelial cells (H and E, ×100). (b) Biopsy from the ulcer margin showed foamy macrophages, ischemic necrotizing vasculitis, fibrinoid necrosis, and presence of clumps of acid-fast bacilli periadnexally, perivascularly, and within macrophages and endothelial cells (H and E, ×400). (c) Fite-Faraco stain from ulcer margin showed numerous acid-fast lepra bacilli lying singly and in clumps forming globi|
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Fite-Faraco stain from both erythematous infiltrated plaque and ulcer margin showed numerous acid-fast lepra bacilli lying singly and in clumps forming globi [Figure 2]c and [Figure 3]c.
Complete hemogram revealed hemoglobin of 9.5 g ⁄ dL, leukocytosis, and raised erythrocyte sedimentation rate. Liver and renal function tests, urinalysis, coagulation studies, blood sugar, antinuclear factor, rheumatoid arthritis factor, antiphospholipid antibodies, Mantoux test, and chest X-ray were normal. Serology for HIV, syphilis, and HBsAg was negative. Smears and cultures for fungus and Mycobacterium tuberculosis from the ulcers were negative.
Based on clinicohistopathological findings and positive SSS, diagnosis of LP in a case of LL leprosy was confirmed.
Multibacillary multidrug therapy with clofazimine 300 mg and rifampicin 600 mg once a month, clofazimine 50 mg, and dapsone 100 mg daily along with oral prednisolone 60 mg daily (gradually tapered) and oral and topical antibiotics were prescribed. Appropriate care of ulcers instituted and hematinics were prescribed for anemia. Unfortunately, the patient died within few weeks.
| Discussion|| |
Leprosy is often complicated by reactional episodes. Type 1 reaction (reversal reaction) characterized by inflammation of the preexisting skin lesions and appearance of new lesions is mainly seen in borderline leprosy. Type 2 reaction (erythema nodosum leprosum) presents as crops of evanescent erythematous tender nodules on extremities and trunk with constitutional symptoms is mainly seen in LL.
A third type of leprosy reaction has been identified as LP which is rare necrotizing reaction seen only in untreated or inadequately treated LuLp patients usually manifesting 1–3 years after the disease onset.
Clinically, LP presents with painful erythematous macules and patches which become purpuric, undergo central necrosis progressing to superficial irregular angulated ulcers with jagged margins. Hemorrhagic blisters may develop occasionally. Burning sensations may be present locally. Lesions usually heal within few weeks leaving behind hypochromic atrophic scars with hyperchromic border. Constitutional symptoms are mild to absent. Systemic involvement and neuritis are not seen.
Both LuLp and LP have restricted global distribution, mainly endemic in Mexico, but cases have been reported in the USA, Spain, South, and Central America, including Brazil, East and West Asia. Exact cause of this restricted distribution is not known. In India, cases of LP have been rarely reported.
Exact etiopathogenesis of LP is unknown, but it has been postulated that diffuse cutaneous infiltration results from uninhibited multiplication of M. leprae.
Sehgal et al. reported blood vessels of skin and other organs showing increased deposition of circulating immune complexes, leading to activation of alternative complement pathway.
Another study by Bonomo et al. showed that serum antinuclear factor was positive in around 30% of patients suggesting an autoimmune etiology of LP.
LP has two patterns on histopathology. First pattern is due to bacillary or skin antigen-induced immune complex disease which presents as leukocytoclastic vasculitis, whereas second pattern is characterized by endothelial cell proliferation, mild mononuclear cell infiltrate, thrombosis, and necrosis which is caused by direct invasion of blood vessels by M. leprae, resulting in vascular damage. Proliferation of numerous acid-fast bacilli both solid and fragmented, lying singly and in globi within macrophages and endothelial cells are also seen.,,,,
It is important to differentiate between LP and vasculonecrotic EN as the latter responds dramatically to thalidomide, whereas LP shows no response to thalidomide but responds well to oral steroids. Comparison between the two is listed in [Table 1].
|Table 1: Comparison between Lucio phenomenon and vasculonecrotic erythema nodosum|
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LP occurs most commonly in cases of LuLp but can rarely occur in other forms of LL. Occurrence of LP in nodular and plaque form of LL may be due to high bacterial load, exaggerated humoral response or anergy which is similar to LuLp. Further studies are needed to explain the exact cause of occurrence of LP in nodular or plaque form of LL as very few cases of LP have been reported in the literature, especially from India.
Difference between LP and lazarine leprosy is given in [Table 2].
[Table 3] shows cases of LP reported from India.
Causes of skin ulceration in LL include neuropathic ulcers, erythema nodosum necroticans, LP, lazarine leprosy, vasculitis etc.
Our case highlights an unusual presentation of LP in a patient of LL leprosy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2], [Table 3]