Leprosy manifesting with type 2 leprae reaction in a patient presenting with chronic fever: A case report.

Leprosy is a chronic granulomatous disease involving the skin and nerves, leading to a debilitating condition. Leprosy has been controlled in most parts of the world; therefore physicians are not very well versed in the recognition, management and assessment of this disease. The protean manifestations of leprosy often lead to delays in diagnosis and increase the morbidity. We present a case of a 33-year-old male with fever, lymphadenopathy, nodular skin lesions, uveitis and arthritis. Lymphnode, bonemarrow and skin biopsy revealed 3+ AFB smear with negative AFB cultures, leading to the diagnosis of leprosy. The course of illness was complicated by flare of Erythema Nodosum Leprosum (ENL).

Using genes to triangulate the pathophysiology of granulomatous autoinflammatory disease: NOD2, PLCG2 and LACC1.

The intersection of granulomatosis and autoinflammatory disease is a rare occurrence that can be generally subdivided into purely granulomatous phenotypes and disease spectra that are inclusive of granulomatous features. NOD2 (nucleotide-binding oligomerization domain-containing protein 2)-related disease, which includes Blau syndrome and early-onset sarcoidosis, is the prototypic example of granulomatous inflammation in the context of monogenic autoinflammation. Granulomatous inflammation has also been observed in two related autoinflammatory diseases caused by mutations in PLCG2 (phospholipase Cγ2). More recently, mutations in LACC1 (laccase domain-containing protein 1) have been identified as the cause of a monogenic form of systemic juvenile idiopathic arthritis, which does not itself manifest granulomatous inflammation, but the same LACC1 mutations have also been shown to cause an early-onset, familial form of a well-known granulomatous condition, Crohn's disease (CD). Rare genetic variants of PLCG2 have also been shown to cause a monogenic form of CD, and moreover common variants of all three of these genes have been implicated in polygenic forms of CD. Additionally, common variants of NOD2 and LACC1 have been implicated in susceptibility to leprosy, a granulomatous infection. Although no specific mechanistic link exists between these three genes, they form an intriguing web of susceptibility to both monogenic and polygenic autoinflammatory and granulomatous phenotypes.

Administration of Mycobacterium leprae rHsp65 aggravates experimental autoimmune uveitis in mice.

The 60 kDa heat shock protein family, Hsp60, constitutes an abundant and highly conserved class of molecules that are highly expressed in chronic-inflammatory and autoimmune processes. Experimental autoimmune uveitis [EAU] is a T cell mediated intraocular inflammatory disease that resembles human uveitis. Mycobacterial and homologous Hsp60 peptides induces uveitis in rats, however their participation in aggravating the disease is poorly known. We here evaluate the effects of the Mycobacterium leprae Hsp65 in the development/progression of EAU and the autoimmune response against the eye through the induction of the endogenous disequilibrium by enhancing the entropy of the immunobiological system with the addition of homologous Hsp. B10.RIII mice were immunized subcutaneously with interphotoreceptor retinoid-binding protein [IRBP], followed by intraperitoneally inoculation of M. leprae recombinant Hsp65 [rHsp65]. We evaluated the proliferative response, cytokine production and the percentage of CD4(+)IL-17(+), CD4(+)IFN-gamma(+) and CD4(+)Foxp3(+) cells ex vivo, by flow cytometry. Disease severity was determined by eye histological examination and serum levels of anti-IRBP and anti-Hsp60/65 measured by ELISA. EAU scores increased in the Hsp65 group and were associated with an expansion of CD4(+)IFN-gamma(+) and CD4(+)IL-17(+) T cells, corroborating with higher levels of IFN-gamma. Our data indicate that rHsp65 is one of the managers with a significant impact over the immune response during autoimmunity, skewing it to a pathogenic state, promoting both Th1 and Th17 commitment. It seems comprehensible that the specificity and primary function of Hsp60 molecules can be considered as a potential pathogenic factor acting as a whistleblower announcing chronic-inflammatory diseases progression.

Ocular manifestation in treated multibacillary Hansen's disease.

PURPOSE: To report the prevalence of ocular morbidity in patients with treated multibacillary Hansen's disease (HD) using modern ophthalmic diagnostic techniques in a rural community endemic for HD. DESIGN: Cross-sectional, observation study. PARTICIPANTS: All patients with multibacillary HD who had completed their multidrug therapy and who resided in 4 defined geographical areas in Vellore, Tamil Nadu, India. METHODS: All participants underwent a complete eye examination that included slit-lamp examination, esthesiometry, gonioscopy, applanation tonometry, and dilated fundus examination, including a stereobiomicroscopic examination of the fundus at an ophthalmic center set up for that purpose. Glaucoma suspects underwent automated perimetry using a Humphrey Field Analyzer (Humphrey Instruments, San Leandro, CA). MAIN OUTCOME MEASURES: The prevalence of various ocular disease parameters were reported as mean value with 95% confidence interval. The difference of disease prevalence between various leprosy groups was compared using an unpaired t test. The association between eye symptoms and potentially sight-threatening complications was analyzed using the chi-square test. RESULTS: Three hundred eighty-six of the 446 patients with multibacillary HD residing in the defined areas were evaluated. Four patients (1.04%; 95% confidence interval [CI], 0.0%-2.0%) were bilaterally blind; 33 (8.55%; 95% CI, 5.8%-11.3%) had unilateral blindness. Mean intraocular pressure was 12 mmHg (standard deviation, 4.1 mmHg), and prevalence of glaucoma was 3.6% (95% CI, 1.8%-5.5%). Potentially sight-threatening (PST) pathologic features (corneal anesthesia, lagophthalmos, uveitis, scleritis, and advanced glaucoma) were present in 10.4% (95% CI, 7.4%-13.4%) of patients. Significant cataracts occurred 3 times more frequently in those with polar lepromatous leprosy. The odds ratio for PST pathology in the presence of patient-reported symptoms (pain, redness, inability to close eye, burning, and irritation) was 2.9 (95% CI, 1.34-6.26). CONCLUSIONS: Patients who have completed treatment for multibacillary HD continue to have significant ocular morbidity. A history of specific eye symptoms can be the basis for referral by field staff.

Cutaneous sarcoidosis: clinical profile of 23 Indian patients.

BACKGROUND: Sarcoidosis is a multisystem disease of undetermined etiology. Indian studies on cutaneous sarcoidosis are not many and mainly comprise case reports. AIMS: This retrospective study was carried out to assess the clinical profile of sarcoidosis patients presenting with cutaneous lesions. METHODS: All histopathologically proven cases of cutaneous sarcoidosis seen consecutively between 1999 and 2004 were studied. Their age, sex, presenting features, evolution of disease and laboratory parameters were analyzed. RESULTS: A total of 23 patients (F:M 15:8) between 31 to 78 years (mean 44.3 years) of age had the mean duration of skin lesions of 1.4 years. Six patients had one to four lesions; two patients each had scar sarcoidosis and angiolupoid and one patient each had recurrent erythema nodosum, leg lymphedema and subcutaneous sarcoidosis. Others showed combination of papules, nodules, plaques and psoriasiform lesions. Peripheral lymph nodes were involved in two patients. Among 10 patients of pulmonary involvement, three had become symptomatic four months to four years after the cutaneous lesions. Routine laboratory investigations including serum calcium estimation were normal in all cases. Serum angiotensin-converting enzyme levels were raised in 3 out of 6 patients. Asymptomatic lytic lesions of digital bones were detected in hand X-ray of one patient. CONCLUSION: Skin lesions of sarcoidosis are like the tip of an iceberg indicating more changes in other organs. The symptomatology and abnormal laboratory results do not necessarily correlate with the severity of cutaneous involvement in general.

Visual outcome of extracapsular cataract extraction and intraocular lens replacement in leprosy patients.

The study was carried out in Meshed, Islamic Republic of Iran, from 1998 to 2000 to explore the visual outcome of eye surgery with extracapsular cataract extraction and intraocular lens replacement on 18 leprosy patients (20 eyes). The most common complications of leprosy were madarosis (90%) and partial or total corneal opacity (90%). Visual acuity before surgery ranged from 'light perception' to 1/10, and this improved after surgery to 5/10-8/10 for 55% of patients. Postoperative infection leading to endophthalmitis occurred in only 1 patient and was treated with drugs; this patient's visual acuity remained at 10 cm finger count. Posterior synechia due to chronic uveitis in leprosy was diagnosed in 70% of eyes, obstructed iris in 25%, keratic precipitates in 25% and moderate iris atrophia in 10%.

Visual outcome of extracapsular cataract extraction and intraocular lens replacement in leprosy patients

The study was carried out in Meshed, Islamic Republic of Iran, from 1998 to 2000 to explore the visual outcome of eye surgery with extracapsular cataract extraction and intraocular lens replacement on 18 leprosy patients [20 eyes]. The most common complications of leprosy were madarosis [90%] and partial or total corneal opacity [90%]. Visual acuity before surgery ranged from 'light perception' to 1/10, and this improved after surgery to 5/10-8/10 for 55% of patients. Postoperative infection leading to endophthalmitis occurred in only 1 patient and was treated with drugs; this patient's visual acuity remained at 10 cm finger count. Posterior synechia due to chronic uveitis in leprosy was diagnosed in 70% of eyes, obstructed iris in 25%, keratic precipitates in 25% and moderate iris atrophia in 10%

Presence of a 88 kDa Eales protein in uveitis, tuberculosis, leprosy and rheumatoid arthritis.

BACKGROUND: Eales disease (ED) is an idiopathic retinal vasculitis affecting young adult males. We have earlier reported the identification, purification and partial characterization of a novel 88 kDa protein found in the serum of patients with ED. The aim of the present study was to look for the 88 kDa protein in serum samples obtained from cases of retinal vasculitis mimicking ED and in other systemic inflammatory diseases. MATERIAL/METHODS: Serum samples from healthy volunteers and from patients with ED, uveitis, parsplanitis ocular sarcoidosis, toxoplasmosis, leprosy, diabetic retinopathy, viral hepatitis, and rheumatoid arthritis were analyzed for the presence of the 88 kDa protein by polyacralymide gel electrophoresis (PAGE). The immunological identity of the 88 kDa protein found in ED and in other diseases was investigated by Western blot. Immunohistochemistry was performed on epiretinal membranes (ERM) obtained from ED patients to localize the 88 kDa protein. RESULTS: 88 kDa protein were detected in serum samples obtained from patients with posterior uveitis, tuberculosis, leprosy and rheumatoid arthritis. The 88 kDa protein found in serum from patients with ED is immunologically identical to that found in other systemic inflammatory conditions. 88 kDa protein was localized in inflammatory cells and in nonvascular endothelium in ERMs obtained from patients with ED. CONCLUSIONS: We have identified a novel acute phase reactant, which is elaborated in ocular and systemic inflammatory conditions other than Eales disease. Further work is necessary to decipher the precise role of the 88 kDa protein in the pathophysiology of these inflammatory diseases.

Presence of a 88 kDa Eales protein in uveitis, tuberculosis, leprosy and rheumatoid arthritis

BACKGROUND: Eales disease (ED) is an idiopathic retinal vasculitis affecting young adult males. We have earlier reported the identification, purification and partial characterization of a novel 88 kDa protein found in the serum of patients with ED. The aim of the present study was to look for the 88 kDa protein in serum samples obtained from cases of retinal vasculitis mimicking ED and in other systemic inflammatory diseases. MATERIAL/METHODS: Serum samples from healthy volunteers and from patients with ED, uveitis, parsplanitis ocular sarcoidosis, toxoplasmosis, leprosy, diabetic retinopathy, viral hepatitis, and rheumatoid arthritis were analyzed for the presence of the 88 kDa protein by polyacralymide gel electrophoresis (PAGE). The immunological identity of the 88 kDa protein found in ED and in other diseases was investigated by Western blot. Immunohistochemistry was performed on epiretinal membranes (ERM) obtained from ED patients to localize the 88 kDa protein. RESULTS: 88 kDa protein were detected in serum samples obtained from patients with posterior uveitis, tuberculosis, leprosy and rheumatoid arthritis. The 88 kDa protein found in serum from patients with ED is immunologically identical to that found in other systemic inflammatory conditions. 88 kDa protein was localized in inflammatory cells and in nonvascular endothelium in ERMs obtained from patients with ED. CONCLUSIONS: We have identified a novel acute phase reactant, which is elaborated in ocular and systemic inflammatory conditions other than Eales disease. Further work is necessary to decipher the precise role of the 88 kDa protein in the pathophysiology of these inflammatory diseases.

Infectious causes of uveitis in the developing world.

Infectious causes of uveitis are common in the developing world and include some causes that are rarely encountered in industrialized nations, such as tuberculosis, leptospirosis, leprosy, onchocerciasis, and cystercicosis. Ocular toxoplasmosis occurs in all countries but is more common in Central and South America, the South Pacific, and western Europe. AIDS-related opportunistic infections occur wherever HIV infection is prevalent, including North and South America, western and eastern Europe, the former Soviet Union, sub-Saharan Africa, and South and Southeast Asia. Physicians who care for patients in the developing world should consider these infectious possibilities whenever their patients develop uveitis.

Soroprevalência do HTLV I/II nos pacientes com uveítes da Fundação Altino Ventura, Pernambuco / Soroprevalence of HTLV I/II in uveitis patients at the Altino Ventura Foundation, Recife, Brazil

Estabelecer a prevalência da uveíte associada ao vírus linfotrópico de células T humana do tipo I e II (HTLV I/II) em um ambulatório especializado em uveítes no Estado de Pernambuco. Examinaram-se, durante o período de maio de 98 a abril de 99, 224 pacientes assistidos no ambulatório de uveítes da Fundação Altino Ventura. Estes pacientes foram clinicamente examinados mediante protocolo oftalmológico previamente estabelecido, e em seguida encaminhados para realizar o teste HTLV I/II (Elisa como "screaning" e Western blot 2.4 como confirmatório). Cento e vinte e cinco pacientes apresentaram uveíte, cuja etiologia foi definida em 62,4 por cento e indeterminada em 37,6 por cento. Detectaram-se cinco casos de HTLV I reagentes (4,0 por cento), que apresentavam uveítes anatomicamente classificadas em: três anteriores e duas difusas. Em relação à etiologia, quatro casos de uveítes (80,0 por cento) eram inespecíficos e um (20,0 por cento) de provável associação com hanseníase. Estes quatro pacientes HTLV I reagentes com uveíte de causa indeterminada representam 8,5 por cento do grupo de uveítes indeterminadas. A prevalência da uveíte associada ao HTLV pareceu ser alta em Pernambuco (4,0 por cento) quando comparada a outros Estados do Brasil, e especialmente alta nos casos de uveítes indeterminadas.