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Biomedical subjects

O Verola

Publications and source records attributed to O Verola.

At least 55 records · Page 3Linked to original sources

Bullous amyloidosis. Report of 3 cases and review of the literature.

We report 3 cases of bullous immunoglobulinic amyloidosis and review 25 published cases. In 2 of our patients, amyloid deposits were not detected with special staining, but by means of ultrastructural methods. Investigations of the skin lesions permitted the diagnosis of associated plasma cell dyscrasia in 2 patients. Unexplained bullous lesions should be investigated for amyloid deposits and the presence of monoclonal gammopathy by methods including electron microscopy and immunochemical analysis of serum and urine.

Aged↗

[Methotrexate-induced skin detachment].

The undesirable effects of high-dose methotrexate on integument and mucosa are well-known, but lesions of the skin proper, characterized by distal erythema with desquamation, are rare. We report the case of a male patient presenting with extensive skin erosions after intravenous infusion of a 5 gram total dose of methotrexate to treat high-grade lymphoma. The skin disease was accompanied by renal, hepatic and mucosal lesions, as well as bone marrow aplasia. The mechanism of cutaneous necrosis is uncertain, but it might be due to direct toxicity of methotrexate to the epidermis. The patient was also treated with G-CSF. He recovered from aplasia within 6 days, without infectious complications. Haematopoietic growth factors might have reduced the risk of infection arising from the skin, by shortening the duration of cytopenia.

Acute Kidney Injury↗

Oral intraepidermal IgA pustulosis and Crohn's disease.

A case is reported of a 60-year-old woman with Crohn's disease who developed recurrent oral blisters and erosions. The histopathology of the buccal mucosa showed intraepidermal bullae and clefts with an intense accumulation of neutrophils in the epithelium. Direct immunofluorescence revealed intercellular IgA predominantly in the basal and suprabasal layers and in one biopsy concurrent deposits of IgG. No circulating autoantibodies were detected.

Crohn Disease↗

Acral persistent papular mucinosis and IgA monoclonal gammopathy: report of a case.

The case of a 60-year-old man with acral persistent papular mucinosis (APPM), thought to represent a new distinctive form of dermal mucinosis not associated with systemic diseases, is reported. The patient had a 4-year history of multiple small papular lesions on the distal forearms, wrists and back of the hands. Histologically, mucin deposits in the upper and mid dermis sparing a superficial subepidermal grenz zone were observed. In contrast to previously described cases, a monoclonal IgA of kappa light chain isotype was detected. Our findings challenge the view that absence of paraproteinemia is a peculiar characteristic of APPM and raise once more the question of its relationship to the discrete papular form of lichen myxedematosus.

Diagnosis, Differential↗

[Deep lupus erythematosus in children].

Deep cutaneous lesions are seldom encountered in lupus erythematosus. The disease described in the literature as lupus erythematosus profundus or lupus erythematosus panniculitis usually occurs in middle-aged women. The authors report a case of deep lupus erythematosus which was exceptional in that it occurred in an 11-year old girl. The lesions were situated on the face and consisted of solid subcutaneous nodules and clear-cut ulcerations leaving atrophic pigmented scars. The histological image of a nodule was one of lobular lymphocytic panniculitis with homogeneous hyalinization of adipose nodules, to which must be added periadnexal and perivascular dermo-epidermal lymphocytic infiltrates. Granular IgM deposits arranged along the dermo-epidermal junction were observed at direct cutaneous immunofluorescence. Laboratory examinations showed leucopenia (3,300/mm3) with neutropenia (1,100/mm3) and the presence of antinuclear antibodies at 1/100 speckled fluorescence, as well as antibodies directed against native DNA. Studies of renal function and for complement gave normal results. The other causes of lobular panniculitis were excluded. The lesions regressed within 3 weeks under hydroxychloroquine; this drug was also successful in arresting a relapse consecutive to withdrawal of treatment. The authors have analysed the 17 paediatric cases of deep lupus erythematosus and were able to determine their main characteristics: 1. The lesions occur mostly in girls (70 p. 100). They are located electively on the face and the lateral aspect of the shoulders. They consist of well-limited, solid or hard subcutaneous nodule which may congregate to form plaques. The epidermis may be normal or pathological, poikilodermic, looking like a discoid or ulcerated lupus erythematosus. The lesions regress, leaving a characteristic atrophic scar.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Antinuclear↗

Non-AIDS-associated Kaposi's sarcoma (classical and endemic African types): treatment with low doses of recombinant interferon-alpha.

In the treatment of the classical and endemic African forms of Kaposi's sarcoma (KS), radiation therapy and chemotherapy have been widely used with varying degrees of success and morbidity. We here report our preliminary experiences with low doses of recombinant interferon alfa-2b (rIFN alpha-2b) in the treatment of these types of KS, non-linked to the acquired immunodeficiency syndrome (AIDS). Ten consecutive patients (eight patients with classical and two with endemic KS) with a median age of 62 years were treated long-term with 5 X 10(6) units of rIFN alpha-2b (Introna) given subcutaneously three times weekly for at least 6 months. Of the 10 patients, six presented with cutaneous disease only, and four had additional visceral involvement. After 6 months of treatment, seven of the 10 patients had a major response of the cutaneous lesions, and three patients showed stable skin diseases. Of the four patients with additional visceral disease, one patient showed a complete regression of an intramyocardial tumor involving the right atrium and ventricle, whereas in the three other patients stabilization of the visceral lesions with marked symptomatic improvement occurred. On the whole, the long-term results over a median duration of 12 months (range, 7 to 30) are also satisfactory: IFN-alpha continued to control KS in all patients. The treatment was generally well tolerated; no serious side effects were observed. Our preliminary data suggest that low-dose rIFN alpha-2b regimens are effective in classical and endemic African KS. However, further studies are needed to establish the exact role for IFN-alpha as alternative to radiation and chemotherapy.

Humans↗

Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome.

We describe two cases of Schnitzler's syndrome presenting with urticarial vasculitis, in which there was a monoclonal IgM of kappa light-chain isotype. Skin histology showed a perivascular leucocytic infiltrate and leucocytoclasis. One patient had an abnormal complement profile and positive rheumatoid factor while the other had antibodies to unidentified soluble nuclear antigens, anti-cytoplasmic antibodies and circulating immune complexes. In addition, in the first case deposits of IgM, C3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.

Aged↗