Syndrome in question: antisynthetase syndrome (anti-PL-7)

Abstract: Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.

Esporotricosis linfocutánea en niños: a propósito de un caso / Lymphocutaneous sporotrichosis in children: apropos of a case

Introducción: La esporotricosis linfocutánea es una micosis subcutánea de evolución sub-aguda o crónica, que es más frecuente en adultos entre los 16 y 30 años de edad pero que también se presenta en la niñez, sobre todo en áreas rurales. Se caracteriza por nódulos que se reblandecen y luego se ulceran, lo cual constituye el chancro inicial. Dos o tres semanas después se observan nódulos eritematovioláceos, no dolorosos siguiendo el trayecto linfático regional. El diagnóstico definitivo se realiza al aislar el agente causal mediante cultivo. En nuestro país el tratamiento usual y eficaz sigue siendo el yoduro de potasio. Caso Clínico: Se presenta el caso de un paciente masculino de 2 años de edad, con dermatosis de 2 semanas de evolución con historia de trauma 2 semanas antes del inicio de las lesiones, caracterizada por úlcera costrosa en dorso de mano derecha con presencia de adenopatías blandas, no dolorosas siguiendo la cadena ganglionar de antebrazo y brazo derecho. Conclusiones: La esporotricosis puede presentarse a cualquier edad aún en niños tan pequeños como este paciente de 2 años. La solución saturada de yoduro de potasio es muy eficaz en el manejo de esta patología por lo que aunado a su bajo costo sigue siendo de elección en países en vías de desarrollo...

Syndrome of remitting seronegative symmetrical synovitis with pitting oedema (RS3PE).

RS3PE syndrome, often mimicking rheumatoid arthritis (RA) or polymyalgia rheumatica (PMR), has puzzled the rheumatologists until late'80s. Though the nature of the disease still remains illusive, the outcome is excellent. This present study analyzes the clinical, radiological and immunogenetical characteristics of five patients diagnosed with RS3PE syndrome, with review of literature.

Tuberous sclerosis complex associated with dyschromatosis universalis hereditaria.

Tuberous sclerosis is an autosomal dominant disease due to mutations in two genetic loci, characterized by hamartoma formation in the skin, nervous system, heart, kidney and other organs. Dyschromatosis universalis hereditaria is an autosomal dominant genodermatosis, characterized by small hyperpigmented and hypopigmented macules, uniformly distributed over the entire body. The face is rarely involved and the palms, soles and mucous membranes are usually spared. We report a case of tuberous sclerosis with dyschromatosis universalis hereditaria, with hyperpigmented and hypopigmented macules affecting the palms, soles and oral mucosa. To our knowledge, this is the first reported case of such an association.

Clinical utility of telangiectasia of hands in scleroderma and other rheumatic disorders.

Telangiectasia of the hands were observed in 76% of patients with scleroderma (n = 53) as compared with 12% of patients with other rheumatic disorders (n = 100) and in 13% of healthy subjects (n = 30). In scleroderma, telangiectasia of the hands were commonly multiple (mean number +/- SD = 22.9+/-30.1) with 7.3% being >1 mm in size. They were found in greater numbers in those patients with the limited subtype. The distribution of telangiectasia was observed on all but 4 of 158 sectors of the hand with significant higher numbers on the ventral surface of the digits. Significant associations of telangiectasia of the hands were also observed with numbers of telangiectasia on face and lips (p = 0.001), disease duration (p = 0.002), surface area of digital calcinosis (p = 0.03) and with the presence of the centromere antibody (p = 0.005). Possible associations were observed with prior gastrointestinal bleeds (particularly with telangiectasia >1 mm) and with isolated pulmonary hypertension. No significant correlation was found between number of telangiectasia and with nailfold capillary size or density. In conclusion, multiple telangiectasia of the hands were most commonly observed in patients with the centromere positive, limited subtype of scleroderma of long duration. Their pathogenesis is unknown.

Tofo gotoso / Thophaceous gout

Apresentamos um caso clínico de gota com tofos gotosos exuberantes, de localizaçäo habitual e lesöes incomuns do tipo pustulosas nas palmas das mäos que também continham cristais de monourato de sódio. Atualmente estes casos näo säo frequentes devido ao diagnóstico precoce e tratamento eficaz da doença.

Distrofia ungueal en el liquen estriado / Ungual dystrophia in the striatus lichen

Presentamos una niña de 7 años de edad, con un liquen estriado en el miembro superior derecho acompañado de onicodistrofia del pulgar. La alteración ungueal, previa al rash cutáneo, mejoró espontáneamente en el curso de tres años

Bacteriology of infected hands and feet in leprosy patients.

70 cases of infected hands and feet admitted to ALERT Hospital during 1986/1987 (3/10/86-5/5/87) were studied for the infecting organisms and the sensitivity of these organisms to available antibiotics. Single organisms were isolated in 56 cases (95%), two organisms were isolated in 3 cases (5%), no organisms were isolated in 11 cases (15.7%). Proteus was the commonest organism. Most effective drug was Ampicillin. Three organisms isolated in 7 cases proved resistant to all drugs tested. The study shows that commonly available drugs are effective in the great majority of secondary infections in leprosy patients.