Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient with multiple myeloma: a rare triple association.

It is well known that primary systemic amyloidosis [light chain (AL) amyloidosis] is associated with hidden dyscrasia or multiple myeloma. Acquired cutis laxa (cutis laxa acquisita; CLA) has also been described in patients with plasma cell dyscrasias, including multiple myeloma. We report a case in which haemorrhagic oral bullae were the first sign of an undiagnosed primary systemic amyloidosis related to multiple myeloma IgG-λ and previously diagnosed CLA. There is only one report in literature of this rare triple association; however, in that case the patient did not have oral mucosal involvement or bullous amyloidosis.

Alteration of the somatostatinergic system in the striatum of rats with acute experimental autoimmune encephalomyelitis.

To date, the neurochemical basis underlying the motor and cognitive deficits described in patients with multiple sclerosis (MS) is unclear. Since the neuropeptide somatostatin (SRIF) and the striatum have been implicated in movement control and implicit memory, the aim of this study was to analyze the striatal somatostatinergic system in an animal model of MS, experimental autoimmune encephalomyelitis (EAE). Female Lewis rats were immunized with an emulsion containing myelin basic protein (MBP) in complete Freund's adjuvant to induce the disease. The animals were decapitated when limp tail (grade 1) or severe hind limb paralysis (grade 3) was observed. Acute EAE in grade 3 did not modify striatal somatostatin-like immunoreactivity (SRIF-LI) content but decreased the overall SRIF receptor density, without affecting the apparent affinity, in the rat striatal membranes. A selective reduction in the protein levels of the SRIF receptor subtype sst2, analyzed by Western blotting, was detected in the EAE rats, which correlated with decreased sst2 mRNA levels. The expression of the receptor subtypes sst1, sst3 or sst4 was unaltered by the disease. The decrease in the SRIF receptor density was accompanied by an attenuated capacity of SRIF to inhibit both basal and forskolin-stimulated adenylyl cyclase activity. No significant changes, however, were found in the protein levels of Gi proteins (G(ialpha1), G(ialpha2) or G(ialpha3)) nor in those of the G-protein-coupled receptor kinase subtypes GRK2, GRK5 or GRK6. Acute EAE in grade 1 did not modify any of the parameters studied. In conclusion, these data demonstrate that acute EAE, in grade 3, disrupts the rat striatal SRIF receptor-effector system. These findings provide new insight into the molecular basis of EAE which might contribute to a better understanding of multiple sclerosis in humans.

[Clinical-biological and radiological study of Wegener's granulomatosis in a university hospital]. / Estudio clínico-biológico y radiológico de la granulomatosis de Wegener en un hospital universitario.

UNLABELLED: Wegener's granulomatosis is a rare systemic vasculitis that fundamentally affects the kidney and respiratory tract. Although the histological findings are very characteristic, they do not occur in all cases, which makes diagnosis difficult. METHODS: The main clinical, biological and radiological characteristics of 13 cases of Wegener's granulomatosis diagnosed in a university hospital of the center of Spain are collected in this present paper. RESULTS: The majority of the patients were men in their 50's. Fever and general syndrome were usual presentations of the disease. Upper respiratory tract (10 cases) and lung involvement (9), followed by renal involvement (8), were the most common. Alveolar infiltrates and bilateral nodules were the most common findings in the X-ray and thoracic CT scan, although the latter permits a greater definition of the lesions. Measurement of antibodies against cytoplasma of the neutrophils (ANCA) was positive in 8 patients (6 cANCA and 2 pANCA). Renal biopsy was used most for the histologic diagnosis, where focal and segmental glomerulonephritis was the most common finding. The majority of the patients received steroids plus cyclophosphamide as induction treatment. CONCLUSIONS: Wegener's disease is a rare systemic vasculitis. The existence of hematuria or respiratory tract involvement in a patient with fever suggests the diagnosis. Conducting a CT scan and ANCAs measurement are useful tools for its diagnosis, since the histology is not definitive.

Estudio clínico-biológico y radiológico de la granulomatosis de Wegener en un hospital universitario / Clinical-biological and radiological study of Wegener's granulomatosis in a university hospital

La granulomatosis de Wegener es una rara vasculitis sistémica que afecta fundamentalmente al riñón y las vías respiratorias. Aunque los hallazgos histológicos son muy característicos, no se presentan en todos los casos, lo que en ocasiones dificulta el diagnóstico. Métodos. En el presente trabajo se recogen las principales características clínicas, biológicas y radiológicas de 13 casos de granulomatosis de Wegener diagnosticados en un hospital universitario del centro de España. Resultados. La mayoría de los pacientes fueron varones en la quinta década de la vida. La fiebre y el síndrome general fueron presentaciones habituales de la enfermedad. La afectación de la vía respiratoria superior (10 casos) y la pulmonar (9) seguidos de la renal (8) fueron las más habituales. Los infiltrados alveolares y los nódulos bilaterales fueron los hallazgos más habituales en la radiografía y tomografía computarizada (TC) torácica, aunque esta última permite una mayor definición de las lesiones. La determinación de anticuerpos frente al citoplasma de los neutrófilos (ANCA) fue positiva en 8 pacientes (6 cANCA y 2 pANCA). La biopsia renal fue la más utilizada para el diagnóstico histológico, donde la glomerulonefritis focal y segmentaria fue el hallazgo más habitual. La mayoría de los pacientes recibieron esteroides más ciclofosfamida como tratamiento de inducción. Conclusiones. La enfermedad de Wegener es una vasculitis sistémica infrecuente. La existencia de hematuria o afectación de la vía respiratoria en un paciente con fiebre sugiere el diagnóstico. La realización de TC y la determinación de los ANCA son herramientas útiles para el diagnóstico, ya que la histología no es definitiva

Wegener's granulomatosis is a rare systemic vasculitis that fundamentally affects the kidney and respiratory tract. Although the histological findings are very characteristic, they do not occur in all cases, which makes diagnosis difficult. Methods. The main clinical, biological and radiological characteristics of 13 cases of Wegener's granulomatosis diagnosed in a university hospital of the center of Spain are collected in this present paper. Results. The majority of the patients were men in their 50's. Fever and general syndrome were usual presentations of the disease. Upper respiratory tract (10 cases) and lung involvement (9), followed by renal involvement (8), were the most common. Alveolar infiltrates and bilateral nodules were the most common findings in the X-ray and thoracic CT scan, although the latter permits a greater definition of the lesions. Measurement of antibodies against cytoplasma of the neutrophils (ANCA) was positive in 8 patients (6 cANCA and 2 pANCA). Renal biopsy was used most for the histologic diagnosis, where focal and segmental glomerulonephritis was the most common finding. The majority of the patients received steroids plus cyclophosphamide as induction treatment. Conclusions. Wegener's disease is a rare systemic vasculitis. The existence of hematuria or respiratory tract involvement in a patient with fever suggests the diagnosis. Conducting a CT scan and ANCAs measurement are useful tools for its diagnosis, since the histology is not definitive