Lazarine Leprosy

a Unique Phenomenon of Leprosy

Abstract

Leprosy, a chronic granulomatous disease, has been known since ages but even today continues to baffle the clinicians with a wide spectrum of clinical, histopathological, and immunological characteristics. Leprosy reactions are mainly of two types, namely, Type 1 and Type 2. In Type 1 leprosy reaction, the preexisting lesions become erythematous, edematous, and rarely ulcerate. Ulcerating Type 1 reaction is called lazarine leprosy. Ulcerations may occur in borderline tuberculoid (BT) pole or borderline lepromatous pole but more common in BT pole. In this postelimination era of leprosy, we report an interesting case report of BT Hansen's disease with Type 1 lepra reaction with ulceration, namely, lazarine leprosy.

INTRODUCTION

Lazarine leprosy, namely, ulcerating Type 1 reaction, was first described in 1852 by Raphael Lucio and Ignacio Alvarado.[¹] Type 1 lepra reaction is a cell-mediated immune reaction seen in borderline forms of leprosy; the preexisting lesions become erythematous, edematous, and rarely ulcerate. In 1930, in the Fifth International Congress of Leprosy, it was concluded that lazarine leprosy can occur in tuberculoid pole because of high inflammation and in lepromatous pole because of high bacillary load.[²]

Case Report

A 69-year-old male presented to our outpatient department (OPD) with multiple, asymptomatic, reddish, raised lesions all over the body for 4 months. No history of fever, tingling numbness of hands and feet, weakness of hands and feet, epistaxis, or redness of the eyes was present. A history of irregular treatment taken in the form of white- and red-colored blister packets for 2–3 months was present. General examination was within normal limits. The body mass index of the patient was 23.3 kg/m². On cutaneous examination, multiple, erythematous, dry, scaly, annular plaques with well-defined, inner borders and sloping outer border giving an inverted saucer-shaped appearance were seen over the chest, back, bilateral upper and lower limbs, and soles. There were sensory impairment and loss of hair over the lesions. On motor examination, book test, card test, and Froment's sign were positive on the right hand, indicating the involvement of the deep branch of the ulnar nerve. Peripheral nerve examination is tabulated in Table 1. The slit-skin smear (SSS) performed from the right earlobe, left eyebrow, and the plaque over the chest was positive for acid-fast bacilli. Morphological index (MI) showed all fragmented bacilli, and bacteriological index (BI) was 1+. Histopathology from the periphery of the plaques over the left thigh and chest showed peri-adnexal dense infiltrate with superficial and deep dermal granulomas consisting of predominantly epithelioid cells, lymphocytes, few neutrophils, and macrophages, with eosinophilic cytoplasm suggestive of borderline tuberculoid (BT) Hansen's disease. Based on the above findings, the patient was diagnosed as a case of BT Hansen's disease and was started on multibacillary multidrug therapy (MBMDT). Four weeks after taking MBMDT, the patient presented to our OPD with spontaneous ulcerations over the preexisting lesions. No history of any new lesions was present. Constitutional symptoms were absent. Cutaneous examination revealed nontender ulcers with well-defined margins and a clear base appearing on the preexisting plaques over the chest [Figure 1a] and back [Figure 1b]. The floor was covered with reddish granulation tissue, and the surrounding skin was hyperpigmented. The ulnar nerves and common peroneal nerves were bilaterally thickened (Grade 1) and nontender, with the right more thickened than the left. Hematological investigations revealed low hemoglobin (Hb – 8.8 g/dL) and low hematocrit (26%). The mean corpuscular volume (90.7 fL), mean corpuscular hemoglobin (MCH) (30.6 pg), MCH concentration (33.8 g/dL), and red cell distribution width (14.1%) were within normal limits. Serum reticulocyte count was 1%. Peripheral blood smear showed mild hypochromasia and microcytosis with rest of the picture being normal. Due to financial constraints, investigations such as serum iron studies could not be performed. Based on the above blood picture, a diagnosis of iron deficiency anemia was made by the physician. Other investigations such as fasting and postprandial blood glucose levels, liver function test (total protein level was 6.3 g/dL and serum albumin level was 4.5 g/dL), renal function test, and urine routine microscopy were within normal limits. Venereal Disease Research Laboratory and HIV ELISA tests were nonreactive. Pus culture revealed growth of Staphylococcus aureus. Blood culture grew no organism. SSS performed from the right earlobe, left eyebrow, and the papule over the back was positive. MI showed all fragmented bacilli, and BI was 1+. Histopathology from the periphery of the ulcerated plaque of the chest and upper back [Figure 2] showed mild hyperkeratosis, focal parakeratosis, and focally thinned-out epidermis with normal rete ridges. The subepidermal zone was variably infiltrated. Reticular dermis showed multiple discrete and mostly coalesced epithelioid cell granulomas composed of epithelioid histiocytes; lymphocytes; and multiple, large, multinucleated (Langhans and foreign-body type) giant cells. Few foci showed neutrophils. Similar infiltrate was seen to extend to the deep dermis and subcutaneous tissue. There was no evidence of any vessel wall damage or macrophages or collagen disruption. Similar histopathology findings were observed from biopsy of the periphery of the plaque over the right sole. On clinicopathological correlation, a diagnosis of BT Hansen's disease with ulcerative Type 1 lepra reaction, i.e., lazarine leprosy, was made. The patient was started on oral prednisolone 40 mg once daily in the morning after breakfast and tapered after every 2 weeks to 30 mg, 20 mg, 15 mg, 10 mg, and 5 mg and then discontinued. Oral amoxicillin/clavulanic acid 625 mg three times a day was given for 10 days and then discontinued. After 3 weeks of treatment, the ulcers healed with hypochromic atrophic scars with a hyperpigmented border [Figure 3].

Table 1: Peripheral nerve examination

Figure 1: (a and b) Multiple, nontender ulcers with well-defined margins and a clear base appearing over the preexisting plaques over the chest (black arrow) and back (red arrow)

Figure 2: Granulomas composed of epithelioid histiocytes, lymphocytes, multiple foreign body and Langhans (black arrow) giant cells, with few neutrophils present (H and E, ×10 and ×40)

Figure 3: Pre- and post-treatment photographs of the ulcers healing with hypochromic atrophic scars and hyperpigmented borders over the chest (red arrows) and back (black arrows)

DISCUSSION

The term “lazarine leprosy” designates a severe, widespread, ulcerative phenomenon in patients with leprosy.[³] Atypical presentations in leprosy are often seen due to partial and delayed treatment. These lead to high morbidity among the patients and a major risk for transmission and disabilities. Two types of ulcers are seen in Hansen's disease, namely, leprous ulcers and trophic ulcers.[³] Trophic ulcers are seen due to sensory impairment, whereas leprous ulcers are seen due to alteration in the immune status of these patients or the excessive load of bacilli in these tissues. In tuberculoid pole, the major cause of lesional ulceration is Type 1 lepra reaction, i.e., lazarine leprosy due to alteration in the immune status of the patient. In lepromatous pole, lesional ulceration is seen in lesions of erythema nodosum leprosum necroticans (Type 2 lepra reaction) or rarely lazarine leprosy due to high bacillary load in the lesions.

Type 1 reaction is a delayed-type of hypersensitivity, where the preexisting lesions become erythematous, edematous, and rarely ulcerate. This rare ulcerating type of Type 1 reaction is called lazarine leprosy. The exact pathogenic mechanism of lazarine leprosy is unknown, but various factors have been implicated such as breakdown of local immunity, increased proliferation of bacilli, and severe tissue edema.[⁴] Protein malnutrition has been suggested to play a main role in the pathogenesis of ulceration.[³] Protein malnutrition leads to an enhanced susceptibility to secondary pathogens, such as streptococci and staphylococci, which results from concomitant debilitation of humoral antibody mechanisms.[⁵] In few cases, lazarine leprosy has also been seen in BT Hansen's disease without any malnutrition or immunosuppression.[⁶] In our patient, there was evidence of superadded bacterial infection along with anemia but no other evidence of protein malnutrition or immunosuppression.

In this form of leprosy, there are no localized skin infiltrations.[¹] Frequent reaction episodes take place with scarlet spots which later darken and ulcerate and usually heal with atrophic and hypochromic scars with a thin hyperpigmented border as was seen in our patient.[⁷] The lesions spread in an upward direction, frequently involve extensor aspects of the feet, legs, hands, forearms, arms, and rarely on the face and torso.[⁸] In our patient, however, the trunk was also involved. Tingling of hands and feet, hypo- or anhidrosis, alopecia of eyelashes and eyebrows, smooth and rosy skin are also reported in these patients.[¹] Other clinical and systemic manifestations include saddle-nose deformity; rhinitis; gastrointestinal, respiratory, and circulatory signs; very prominent varicose veins; absence of eye lesions; and an absolute absence of nodules.[¹⁸] Our patient had tingling of hands and feet. Polymerase chain reaction can help in identifying leprosy diagnosis in suspected patients with clinically atypical lesions, presenting with negative baciloscopy and indecisive histopathology.[⁹] Diagnosis at an early stage is absolutely essential for earlier treatment to prevent leprosy-related disabilities and its benefit for termination or control of infection transmission.[¹⁰]

CONCLUSION

Thus, even today as we are trying hard to eradicate leprosy, newer forms of presentation continue to crop. In an endemic country like India, we still need to have an open eye to recognize the varied manifestations of leprosy so as to ensure prompt diagnosis and treatment, which can reduce the morbidity in potentially treatable patients. We present this case of lazarine leprosy on account of its rare occurrence and its association with anemia and superadded infection along with the involvement of torso which is rare in lazarine leprosy.

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 initial will not be published, and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.