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Biomedical subjects

V Guerrera

Publications and source records attributed to V Guerrera.

8 recordsLinked to original sources

Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients.

Pemphigus refers to a group of autoimmune blistering skin diseases, mainly identified as pemphigus vulgaris and pemphigus foliaceus, both characterized by the presence of autoantibodies against keratinocyte adhesion molecules, leading to loss of cell-cell adhesion with consequent blister formation. Pemphigus vulgaris is reported to be associated with human leukocyte antigen DR4 and/or DR6 whereas no data are available on pemphigus foliaceus, except for the endemic Brazilian form (fogo selvagem), which is reported to be associated with DR1 and DR4. We here report human leukocyte antigen molecular typing on a total of 87 patients, 61 with pemphigus vulgaris and 26 with pemphigus foliaceus, versus 128 healthy matched controls. Generic typing showed an increase of DRB1*04 and DRB1*14 and a decrease of DRB1*07 in both pemphigus vulgaris and pemphigus foliaceus patients. Molecular subtyping of DR4+ and DR14+ subjects showed a highly significant association between the DRB1*1401 and both pemphigus vulgaris (p < 0.0001) and pemphigus foliaceus patients (p < 0.0001) together with a significant increase of the linked DQB1*0503 (pemphigus vulgaris p < 0.0001; pemphigus foliaceus p < 0.0001). Moreover, whereas the association between DRB1*0402 and pemphigus vulgaris (p < 0.0001) has been confirmed, no significant association between a specific allele of the DR4 group and pemphigus foliaceus, has been found. Therefore, at least in Italian patients, pemphigus vulgaris and pemphigus foliaceus share DRB1*1401 and DQB1*0503, as susceptible human leukocyte antigen alleles, whereas DRB1*0402 is only found associated with pemphigus vulgaris. The observation that both diseases, pemphigus vulgaris and pemphigus foliaceus, carry the same susceptible human leukocyte antigen alleles has been interpreted as a common genetic background predisposing to pemphigus as, like in other autoimmune disorders, it is not sufficient to explain the onset of the disease on the basis of the sole aforementioned alleles. Other linked genes and/or environmental factors should play a facilitating role in the outbreak of pemphigus, either pemphigus vulgaris or pemphigus foliaceus.

Alleles↗

In vivo enalapril-induced acantholysis.

Enalapril is a widely used antihypertensive drug with a very powerful in vitro acantholytic effect. It has been known to potentially induce pemphigus in genetically predisposed subjects. The action mechanism is complex and still only partially understood. We describe the case of a 66-year-old man, affected with intermediary basal cell carcinoma, in whom the histological examination showed suprabasal acantholytic clefts in the perilesional epidermis. Surprisingly a second biopsy taken from the apparently healthy skin of his back confirmed the presence of acantholytic changes. Clinical signs of pemphigus were absent. The patient's history did not reveal any relevant data but a mild arterial hypertension that had been treated for 1 year with 10 mg enalapril. Taking into account the patient's history (enalapril long-term administration), the absence of any bullous or erosive lesions and the histological findings, a diagnosis was made of in vivo enalapril-induced acantholysis.

Acantholysis↗

"Two-step" pemphigus induction by ACE-inhibitors.

Pemphigus lesions appeared in a 58-year-old man who was taking captopril for his hypertension. Drug withdrawal resulted in complete remission of the eruption. The subsequent use of enalapril as an antihypertensive agent caused a recurrence of pemphigus lesions along with onset of itching and dermographism. Intercellular antibodies were not found. Discontinuance of enalapril therapy had no effect on the clinical course. Steroid treatment was needed to resolve the eruption. Recently repeated immunofluorescent studies disclosed intercellular IgG antibodies in the serum at a low titer. Pemphigus induction could be initially related to the thiol acantholytic property of captopril. Subsequent production of intercellular antibodies and drug-activation of the kinin system could be responsible for relapsing.

Anti-Inflammatory Agents↗

An unusual case of trimethylaminuria.

A case is reported of trimethylaminuria that first developed in adulthood without any apparent cause. The patient developed a characteristic fish odour of his sweat, urine and to his breath after the consumption of choline-rich foods. Elevated levels of trimethylamine were present in the urine after dietary tests and identified by means of gas chromatography.

Adult↗

[Classical Kaposi's sarcoma. Study of the lymphatic system of lower limbs].

Eight patients with classical Kaposi's sarcoma histologically confirmed underwent lymphoscopic and/or lymphographic exploration of the lower limbs where the lesions were located. In all patients lymphoscopy showed abnormal images ranging from irregular distribution of the colouring agent to "starlike images" and dermal reflux. Lymphography was performed in 3 patients and demonstrated, in 2 of them, "lymphatic pools", obvious hyperplasia of the superficial lymphatic vessels and areas of lymphangectasia. The finding in all 8 patients of dynamic abnormalities of the lymphatic system should encourage further investigations in this field, notably to detect a possible correlation between lymph stasis, disorders in regional immunity and the development of classical Kaposi's sarcoma.

Female↗

Kaposi's sarcoma on a lymphedematous immunocompromised limb.

Kaposi's sarcoma appeared on a chronically lymphedematous leg of a 75-year-old man. Immunologic investigations revealed a lack of cellular immune response confined to the involved lower limb. Regional disorders of immune surveillance are thought to play an important role in the early stages of Kaposi's sarcoma.

Aged↗