Leprosy presenting as remitting seronegative symmetrical synovitis with pitting oedema syndrome - a case report.

BACKGROUND: Leprosy typically manifests with skin and peripheral nerve involvement. Musculoskeletal complaints are the third most common, and can be the sole presenting manifestation. They range from arthralgia/arthritis in reactional states to full mimics of systemic rheumatic diseases. Remitting Seronegative Symmetrical Synovitis with Pitting Oedema syndrome has only been described once in a patient with already diagnosed Leprosy. CASE REPORT: A 68-year-old male, from an endemic region of familial amyloid polyneuropathy, presented with an inaugural Remitting Seronegative Symmetrical Synovitis with Pitting Oedema like syndrome, more that 20 years after travelling to Leprosy endemic areas. Arthritis would resurface whenever oral prednisone was tapered, so methotrexate was started, controlling the complaints. Only one year later, after the appearance of peripheral neuropathy and skin lesions, it was possible to diagnose Leprosy, through the identification of Mycobacterium leprae bacilli in a peripheral nerve biopsy. CONCLUSION: This report is an example of the heterogeneity of manifestations of Leprosy, namely rheumatic, and the challenge of diagnosing it when typical complaints are absent. It is also a reminder that this disease should be considered whenever a patient with a combination of skin/neurologic/rheumatic complaints has travelled to endemic countries in the past.

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.

[Lepromatous leprosy revealed by a swollen hands syndrome]. / Syndrome des mains boudinées révélant une lèpre lépromateuse.

Osteoarticular pathology in leprosy is common and described at all stages, but rarely as the most evident clinical manifestation. We report a case of borderline lepromatous leprosy with initial and disabling hands edema. The swollen hands syndrome is probably due to chronic Mycobacterium leprae tenosynovitis.

Remitting seronegative symmetrical synovitis with pitting edema in leprosy.

A 67-year-old man, who had widespread and well-defined erythematous violaceous hyperkeratotic plaques on his skin, was diagnosed with borderline tuberculoid leprosy. The patient began treatment with clofazimine, rifampicin, and dapsone, but 15 days afterwards he complained of acral edema with godet sign. Magnetic resonance imaging was done, and the case was interpreted as remitting seronegative symmetrical synovitis with pitting edema. About 8 mg/day of methylprednisolone were started with excellent response.

Leprous synovitis. A study of fifty cases.

A study of 50 synovial biopsies of proved lepromatous patients with arthritis was carried out. Out of these 50 cases, 14 cases were suffering from lepra reaction and the histopathological study of the synovium in these 14 cases revealed the presence of only vasculitis and lymphocytic infiltration. In the remaining 36 cases, not associated with lepra reaction, the synovial lining showed hyperplasia and villous hypertrophy, and the synovial tissue showed congestion, pannus formation, the presence of macrophage granulomas and, in some cases, even intact acid-fast bacilli. Since there was no other cause for arthritis, it is perhaps likely that the primary synovial involvement in these 36 cases may be the cause of the arthritis. In the ten cases of lepromatous leprosy without arthritis studied as controls, no histopathological abnormality in the synovium could be detected.

Modelo experimental de sinovite transitória do quadril, produzida por reagente de fenol: estudo em coelhos jovens / Experimental model of transient hip synovitis, produced by phenol reagent: study in young rabbits

Foi produzido um método experimental de sinovite transitória do quadril em coelhos jovens, através de injeçao intra-articular de Reagente de Fenol. Foram utilizados sessenta e sete coelhos, dos quais vinte e sete serviram às observaçoes do grupo piloto. Dos quarenta coelhos estudados na fase definitiva, dez serviram de controle através de injeçao intra-articular de soluçao fisiológica. Atributos clínicos, radiológicos e anátomo-pathológicos foram analisados nos tempos de 1 hora, 6 horas, 24 horas, 48 horas e 7 dias, após a injeçao intra-articular. Os resultados obtivos revelaram que, tanto clínica como anátomo-patologicamente, houve a produçao de sinovite aguda que se iniciava no grupo de seis horas, intensificando-se até 48 horas e com regressao da inflamaçao ao 7§ dia, sem resultar em necrose intra-articular, nem em cronificaçao da sinovite. Radiologicamente foram observados: aumento dos espaço articular e ausência de lesoes ósseas nos quadris tratados. Foram discutidos o método e as técnicas empregadas, com especiais referências à movimentaçao passiva relacionada com a intensidade da sinovite, ao resultado radiológico do método e ao quadro anátomo-patológico obtido. Conclui-se tratar de método válido na produçao de modelo experimental de sinovite transitória do quadril.

Mycobacterium fortuitum: an unsuspected cause of synovitis and osteomyelitis

Two men with progressive synovitis and osteomyelitis of the wrist were found to have infection with Mycobacterium fortuitum. Immunological defects were demonstrated in both cases in association with renal failure and renal transplantation respectively. Failure of appropriate antibiotic therapy to eradicate infection necessitated amputation in one case. M. fortuitum is frequently sensitive to only amikacin or kanamycin. Treatment with amikacin alone was unsuccessful. This organism is widespread in the environment and may need to be considered in undiagnosed chronic infection in the immunologically suspectible host, as it poses unusual problems in diagnosis and therapy.

Manifestations articulaires au cours de deux cas de lèpre

Deux observations d'arthrosynovite survenant au cours d'une > sont rapportées: l'une concerne un cas de lèpre nerveuse où une monoarthrite d'un genou succède à une intradermo-réaction de lépromine; l'autre un cas de lèpre lépromateuse où, quelques mois après l'administration de disulone, survient une atteinte polyarticulaire au cours d'un érythème noueux. Divers cas relevés dans la littérature sont analysés. Les désorders immunitaires observés au cours de la réaction lépreuse fournissent l'explication la plus satisfaisante. La rifampicine, utilisée avec succès dans l'un des cas rapportés, completè lers moyens thérapeutiques de la réaction lépresuse où corticoides, thalidomide, lamprène, sont doués d'une réelle efficacité.