Cytodiagnosis of erythema nodosum leprosum. A case report

BACKGROUND: The application of cytology in leprosy has been restricted to the evaluation of morphologic and bacterial indices by slit skin smears to facilitate diagnosis of cases according to the Ridley-Jopling scale. Isolated reports have now documented the use of fine needle aspiration cytology (FNAC) in the diagnosis of leprosy. CASE: A 45-year-old male presented with the abrupt onset of multiple nodular eruptions all over the body. The clinical diagnosis was Sweet's syndrome. FNAC showed numerous neutrophils in a background of foamy macrophages. Special stains revealed the presence of a large number of fragmented acid-fast bacilli in the smears. A diagnosis of erythema nodosum leprosum (ENL) was made on FNAC. CONCLUSION: The presence of neutrophils in a characteristic milieu of foamy macrophages is seen in lesions of ENL. Such a picture should prompt the cytologist to use a modified Ziehl-Neelsen stain to demonstrate acid-fast bacilli, as ENL can present as an acute episode in patients without a previous diagnosis of leprosy.     

Systemic manifestations of erythema nodosum

The systemic manifestations accompanying erythema nodosum can be differentiated from those associated with the precipitating infectious process and from coincident disease processes. Erythema nodosum itself is characterized by (a) skin lesions at pressure sites, (b) malaise, fever and occasionally chills, (c) arthritis (70 per cent) and (d) over-reactivity of tissue. Tissue hypersensitivity is most pronounced at sites of trauma, at sites of specific skin testing, and in the lymphoid system draining infections in the pharynx and lung. Common infections of the respiratory tract most often antedate attacks of erythema nodosum. In New England, a beta-hemolytic streptococcus infection is a common causative factor, and tuberculosis is an unusual causative factor. In endemic areas, coccidioidomycosis is a common cause of erythema nodosum. The most important coincidental disease process is rheumatic heart disease. Rarely is it a sequel of erythema nodosum. Other "collagen diseases" may coexist with erythema nodosum. Erythema nodosum is its own most common complication. Follow-up studies indicate that over half of the patients have a subsequent attack, and a certain number have recurrent episodes for months to years. The management of erythema nodosum is expectant. In each case the cause should be found and treated. Steroid treatment is rarely justified, and should be used only after tuberculosis and other treatable entities have been ruled out.     

Fine needle aspiration cytologic diagnosis of erythema nodosum leprosum: a case report

BACKGROUND: Erythema nodosum leprosum (ENL), the type 2 lepra reaction occurring in lepromatous or borderline lepromatous leprosy, presents clinically with acute manifestations that compel the patient to seek medical attention. Recognition and timely management of these patients is critical in order to avoid permanent disability. Fine needle aspiration cytology (FNAC) is a simple, effective tool that aids in correct diagnosis and management of ENL. CASE: A 30-year-old woman presented with history of fever, reddening of the face, and multiple raised, reddish, painful swellings of the bilateral forearms and legs for 7 days. One year previously, she was diagnosed and treated for lepromatous leprosy with type 2 reaction. After a thorough clinical examination a diagnosis of ENL was made. FNA smears from the forearm swellings showed pus-like material with intact and degenerated polymorphonuclear leukocytes and many foamy macrophages with strong granular acid-fast bacillus (AFB) positivity. A cytologic diagnosis of ENL was given, which was confirmed on histopathologic examination of skin biopsy. CONCLUSION: Cytologic features such as a large number of intact and degenerated neutrophils with foamy macrophages and strong granular AFB positivity, in an appropriate clinical background, allows a confident diagnosis of ENL.     

Effect of cyclosporine A in erythema nodosum leprosum

Erythema nodosum leprosum (ENL) is a reactional state of lepromatous leprosy in which the loss of suppressor cell function, decrease in suppressor cell numbers, and increase of interleukin 2 production are observed. We reasoned that cyclosporine A (CsA), by opposing these immune responses, could suppress the ENL reaction and restore patients to the quiescent lepromatous state. We tested this hypothesis in vitro by measuring the effect of CsA on M. leprae-triggered suppressor cells. In 24 of 25 patients with ENL, suppressor cell activity was restored by CsA. The target of CsA appeared to be macrophages. These findings are significant in that they provide the first evidence for the potential efficacy of CsA in the treatment of ENL. Preliminary clinical trials indicate a beneficial therapeutic effect associated with increased T suppressor cells in lesions.     

Ultrastructure of nerve in erythema nodosum leprosum (ENL)

Gross changes of myelinated fibres and Schwann cells at different degenerative stages were present in all five ENL nerve lesions. Besides these changes, infiltrating cells mainly macrophages, deposition of excessive collagen and perineurial vessel damage were also observed.     

In situ and in vitro characterization of the cellular immune response in erythema nodosum leprosum

We sought to evaluate cell-mediated immune responses in erythema nodosum leprosum (ENL), a reactional state occurring in lepromatous leprosy. Skin biopsies from patients with leprosy were studied with monoclonal antibodies against T lymphocyte antigenic determinants, interleukin 2 (IL 2), and IL 2 receptors (Tac) by using immunoperoxidase staining of frozen sections. Peripheral blood lymphocytes from 18 ENL patients were tested in vitro for lepromin-induced suppression of Con A stimulation. Serial studies of seven lepromatous patients who developed ENL during the course of the study showed increases in both the Leu-3a:Leu-2a ratio and the number of IL 2-positive cells. IL 2-positive cells comprised 0.3% of the cells in all of the ENL lesions studied as compared with the 0.03% found in nonreactional lepromatous lesions (P less than 0.001). Lepromin-induced suppression of the Con A response, present in nonreactional lepromatous patients, significantly decreased in patients developing the ENL reaction, but returned after recovery from ENL. These changes in tissues and peripheral blood suggest that the pathogenesis of ENL is related to cell-mediated immune processes. Despite these immunologic changes, however, ENL patients do not recover antigen-specific skin tests or eliminate Mycobacterium leprae.