Rare atypical presentations in Type 2 lepra reaction: a case series.

OBJECTIVES: Type 2 lepra reaction is a Th2-mediated type III hypersensitivity reaction in leprosy, with a characteristic cutaneous manifestation in the form of erythema nodosum leprosum (ENL). We describe unusual presentations of Type 2 lepra reaction in five patients. METHODS: Patient data and dermatological findings were analyzed in three men and two women diagnosed with Hansen's disease. RESULTS: Findings included multiple tender, polycyclic, necrotic lesions distributed over the face in one patient, and painful, fluid-filled lesions on both arms and lower limbs in another. The third patient showed erythematous, tender nodules, bullae, and necrotic ulcers over the back and upper and lower limbs. The fourth showed erythematous tender nodules over the face, neck, back, and extremities, predominantly in sun-exposed areas. The fifth revealed multiple erythematous, severely tender nodules and urticarial plaques mimicking those of Sweet's syndrome. Diagnosis of borderline or lepromatous leprosy with atypical Type 2 reaction were made in all cases. CONCLUSIONS: Type 2 lepra reactions are antigen antibody-mediated immune complex reactions that present with constitutional symptoms and ENL characterized by tender, erythematous, evanescent nodules mainly on the face, arms, and legs. Over 50% of lepromatous leprosy patients and 25% of borderline lepromatous leprosy patients experienced type 2 lepra reactions prior to the advent of multi-drug therapy. Thalidomide is the drug of choice for severe atypical lepra reactions because of its anti-tumor necrosis factor-α action. Awareness of these atypical variants and prompt diagnosis and treatment are essential to prevent mortality and morbidity in potentially treatable patients.

Evaluation of circulating immune complexes in cutaneous diseases associated with immune disorders.

Several skin diseases associated with immune disorders may be related to the formation of circulating immune complexes (CIC) and their skin deposition. Sera from 20 controls and 108 patients, including: 23 psoriasis, 10 lichen planus, 30 atopic eczemas, 32 cases of leprosy, 10 vasculitis and 3 pyoderma gangrenosum were evaluated for the presence of IgG-containing CIC by the microconsumption complement test (MCT). Additionally, the presence of IgE-containing CIC by means of a polyethylene glycol precipitation and radioimmunoassay technique was evaluated in 10 patients with atopic eczema. It was found that 56.5% of psoriatic patients show moderate CIC concentrations, as well as 34% of leprosy patients, with increased levels when bacillus were detected in skin lesions, and in 90% of leukocytoclastic vasculitis. A close relationship between CIC levels and the clinical evolution of skin lesions was demonstrated in patients with pyoderma gangrenosum and lichen planus. IgG-CIC were detected in 33% and IgE-CIC in 30% of patients with atopic eczema, with the presence of both types of CIC in 2 out of 3 cases. The systematic research on CIC presence in some selected skin diseases shows that immune complexes take part, with different degrees of relevance, in the pathogenesis of all the entities.

[Demonstration of C3-binding circulating immune complexes using Raji, conglutinin and anti-C3 assays--a critical review]. / Nachweis C3-bindender zirkulierender Immunkomplexe durch Raji-, Konglutinin- und Anti-C3-Assays--eine kritische Wertung.

There remains no doubt at the present time, that the appearance of circulating immune complexes in illness accompanying vasculitis and for glomerulonephritis correlates with the severity of disease. Moreover, immune complexes are of diagnostic importance where infections with a chronic development or neoplastic diseases are concerned. The choice of IC test system should incorporate their essential biological functions and identify those IC that activate the complement cascade both by the classical and the alternative route. The detection of IC bound C3 cleavage products (C3b, C3bi, C3d) represents the key to identification of a wide range of IC. Of the presently available methods Raji cell test, conglutinin- and anti C3-IC assay, on critical appraisal, the anti C3-IC assay represents the most applicable way of defining complement binding IC. The advantage of this system is that appreciable disturbances and limitations that influence other systems do not affect the antigen-antibody reaction which is the core of the anti C3 assay.

[Lucio's leprosy]. / Lepra de Lucio.

A case of diffuse lepromatous leprosy with lepra reaction type II-Lucio's phenomenon-in a 24 years old male patient is reported. The histological examination of the necrotic lesions and of the apparently normal skin showed the presence of dense perivascular and perianexial lymphohystiocitic infiltrates with great quantities of bacilli. The first biopsy did not show a picture a leuccocytoclastic vasculitis but only areas of necrosis. The immunofluorescence studies revealed on direct examination complement deposits on vessel walls. The complement levels in blood were lowered and circulating inmunecomplexes were also detected. These data confirm the opinion that Lucio's phenomenon is caused by circulating inmunecomplexes fixed on dermal vessel walls causing skin necrotic lesions.

Glomerulonephritis in leprosy.

A renal biopsy from a patient with lepromatous leprosy but no history of erythrema nodosum leprosum (ENL) showed histologic, immunofluorescent, and ultrastructural features typical of immune-complex glomerulonephritis. A literature review found reports of 187 renal biopsies from leprosy patients, with glomerulonephritis in 31% of the cases, and a variety of other renal lesions. This accumulated evidence demonstrates that, contrary to widely held opinion, there is no association between glomerulonephritis and a history of ENL. Furthermore, it suggests that glomerulonephritis in leprosy patients has a similar incidence in lepromatous and non-lepromatous cases. These observations have an important bearing on concepts regarding the pathogenesis of immune complex glomerulonephritis in leprosy and the humoral immune mechanisms in patients with leprosy.

An appetite for armadillo

In October 1979, a 70-year-old man was referred and admitted to the Medical University of South Carolina Hospital. Three or four years earlier, he had begun to notice the appearance of raised, nontender lumps on his arms and legs.

Soluble immune complexes in human disease.

The great variety in biochemical properties of immune complexes occurring in human and animal disease states has made the detection of such complexes a difficult task. Variability in immune complex size, specificity, and interaction with humoral or cellular receptor systems, such as complement and phagocytes, suggests different pathogenic properties. The introduction of radioimmunoassays and the recently improved knowledge of the immune complex-receptor interactions have lead to the description of a large number of detection procedures, which in turn has widened the catalogue of diseases associated with immune complexes. This widespread occurrence of soluble immune complexes has lead many investigators to think that such complexes may occur either as a transient physiological phenomenon, important for fast clearance of the antigen, or as primary pathogenic factors triggering inflammatory reactions. Among the 50 procedures for immune complex detection known today, the article will select some pertinent tests, which will be discussed with respect to their specificity, sensitivity, and reproducibility. Furthermore, it is well known that when applied to the study of a patient group with one particular immune complex disease, various tests will result in different percentages of patients having complexes. This observation is due to differences in the underlying principle on which the various tests are based. Thus immune complexes must be further characterized with respect to their size, to the antibody class or specificity involved and, most difficult, to the antigenic specificity which participates in the complex. Recent advances in such experimental characterization of immune complexes in vitro and in the clinical evaluation of patients with complement activation associated to the presence of immune complexes will be discussed.

Serum complement profile in human leprosy and its comparison with immune complex diseases.

In the present study we have estimated the serum levels of early, middle, and distal complement components, e.g., Clq, C3, C4, C5, C8, and C9 along with C1-inactivator and CH50 in patients with tuberculoid and lepromatous leprosy and have compared these results with the levels in healthy subjects as well as with levels in patients with other immune complex diseases. We have also analyzed the cryoglobulins present in the sera of these patients; they consisted of either a single or mixed IgG, IgA, IgM or fibrinogen in most instances. The component C3 was found in only one sample. It appears that unlike lupus nephritis, in which complement is activated by direct path in which complement is activated by direct path in about 30% to 50% of leprosy patients, significant C3 complement consumption takes place primarily via the alternate pathway and is probably initiated by the aggregated immunoglobulins represented in cryoprecipitates. This is further supported by the study of serum factor B and its breakdown product (Ba) in these patients. The question of the role of the middle and distal complement components, such as C5, C8 and C9, during total hemolytic complement and C3 consumption in leprosy remains unanswered.