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Biomedical subjects

Alfredo Rebora

Publications and source records attributed to Alfredo Rebora.

At least 37 records · Page 2Linked to original sources
PubMed2004

Frequency of IgA antibodies in pemphigus, bullous pemphigoid and mucous membrane pemphigoid.

Circulating and bound IgA antibodies can be found in the autoimmune blistering diseases, but their prevalence, clinical relevance and target antigens remain unknown. Thirty-two patients with pemphigus, 73 with bullous pemphigoid and 28 with mucous membrane pemphigoid were studied retrospectively. Direct immunofluorescence (DIF) analysis of IgG, IgA, IgM and C3 was carried out for all cases. Sera were studied by standard indirect immunofluorescence, indirect immunofluorescence on salt-split skin, immunoblotting for bullous pemphigoid and mucous membrane pemphigoid and ELISA for pemphigus. With DIF, we found IgA autoantibodies in 22 of all 133 cases. Circulating IgA antibodies to skin were detected in 2 of 3 IgA-DIF-positive patients with pemphigus, in 3 of 6 with bullous pemphigoid, and in 6 of 13 with mucous membrane pemphigoid. We confirm that the IgA reactivity is more frequently associated with mucous membrane involvement, especially in cases without critical involvement (5/8). The role of IgA and its antigenic specificity in these diseases remain unclear.

Adult↗
PubMed2003

Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation.

New and relatively old types of vascular (capillary) proliferations in the skin have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, all of them present with multiple, erythematous-violaceous and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve limbs. Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically mimic vascular tumors, they are reactive in that they seem to originate from the occlusion of vascular lumina by different localized or systemic disorders and the vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn. In this article, the authors review all these forms of reactive angioproliferations in the skin, suggest a novel title, cutaneous reactive angiomatoses, and propose a unifying pathogenetic mechanism.

Angiomatosis↗
PubMed2002

Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.

OBJECTIVE: To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen patients with amyopathic dermatomyositis. RESULTS: The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. CONCLUSIONS: Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.

Adult↗
PubMed2002

Acral localized acquired cutis laxa associated with rheumatoid arthritis.

We report the first case of the acral localization of the acquired form of cutis laxa associated with severe rheumatoid arthritis. The skin laxity was preceded by episodes of itching and swelling of the hands and feet. Histopathology showed that the elastic fibers were lost in the areas of cutis laxa and decreased in adjacent skin. The pathogenetic relationship with rheumatoid arthritis or the intake of related drugs is discussed.

Arthritis, Rheumatoid↗
PubMed2002

Anorectal involvement is frequent in limited systemic sclerosis.

The gastrointestinal tract, particularly the oesophagus, is affected in about half of all patients with systemic sclerosis. Only a few studies so far have dealt with the anorectal tract. We studied the anal function using anorectal manometry in 12 patients with limited systemic sclerosis. We also studied the oesophageal function. For the oesophagus, we measured the difference between intragastric and oesophageal pressure, while for the anorectal tract we investigated the maximum resting pressure, the maximum voluntary squeeze effort and the rectoanal inhibitory reflex. Maximum resting pressure and maximum voluntary squeeze effort were found to be decreased in all patients. The rectoanal inhibitory reflex was abnormal in four patients. Statistical analysis showed a significant correlation between maximum resting pressure and maximum voluntary squeeze effort. No correlation was found between oesophageal and anorectal involvement. Anorectal dysfunction is common in patients with limited systemic sclerosis. We suggest that these patients should have an evaluation of their anorectal function including anorectal manometry.

Anal Canal↗
PubMed2002

Disseminated porokeratosis with fatal metastatic squamous cell carcinoma: an additional case of "malignant disseminated porokeratosis".

We report on a patient with a disseminated form of porokeratosis in whom bowenoid lesions and squamous cell carcinoma developed in an apparent sequential progression. Local and disseminated metastases ensued, resulting in the death of the patient. Furthermore, we found an overexpression of the p53 protein in the keratinocytes beneath and adjacent to the cornoid lamella in the porokeratotic lesion and throughout the epidermis in a bowenoid lesion. Although malignancy has been reported previously in various types of porokeratosis, the development of fatal metastatic squamous cell carcinoma in the setting of this disease is a rare event. The histopathologic findings of this case document the association of porokeratotic lesions with bowenoid dysplasia and aggressive squamous cell carcinoma and confirm that a p53 functional aberration can be important in a malignant outcome such as this.

Aged↗