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Angelo V Marzano

Publications and source records attributed to Angelo V Marzano.

15 recordsLinked to original sources
PubMed2007

Activation of the tissue factor pathway of blood coagulation in patients with chronic urticaria.

BACKGROUND: In patients with chronic urticaria (CU), plasma shows signs of thrombin generation and autologous plasma skin tests score positive in as many as 95% of cases. OBJECTIVE: To evaluate the initiators of blood coagulation that lead to thrombin generation and fibrinolysis in CU. METHODS: Activated factor VII, activated factor XII, fragment F(1+2), and D-dimer plasma levels were measured in 37 patients with CU and 37 controls. Skin specimens from 10 patients with CU and 10 controls were tested for tissue factor immunohistochemically. RESULTS: Mean F(1+2) levels were higher in patients than controls (2.54 [SD 2.57] nmol/L vs 0.87 [0.26] nmol/L; P < .001); disease activity was moderate or severe in 9 of 11 (82%) and 9 of 26 (35%) patients showing high or normal F(1+2) levels, respectively (P < .025). Mean D-dimer plasma levels were higher in patients than controls (329 [188] ng/mL vs 236 [81] ng/mL; P < .01); disease activity was moderate or severe in 6 of 8 (75%) and 11 of 29 (38%) showing elevated or normal plasma D-dimer levels (P = NS). Factor VIIa levels were higher in patients than controls (2.86 ng/mL [0.66] vs 1.97 ng/mL [0.65]; P < .001). Activated factor VII and F(1+2) levels were correlated (r = 0.529; P = .008). Tissue factor reactivity was observed only in CU skin specimens. CONCLUSION: The extrinsic pathway of clotting cascade is activated in CU. Disease severity is associated with the activation of the coagulation cascade. CLINICAL IMPLICATIONS: The involvement of the coagulation pathway in CU opens new perspectives for a better understanding of the pathogenesis and, possibly, for the treatment of this disease.

Adolescent↗
PubMed2006

Wells syndrome in adults and children: a report of 19 cases.

BACKGROUND: Wells syndrome, an uncommon inflammatory dermatosis, is characterized by protean cutaneous manifestations, suggestive but not specific histopathologic findings, and usually a recurrent course. Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents. OBSERVATIONS: The medical records of 19 patients (12 adults and 7 children) with Wells syndrome referred to the Institute of Dermatological Sciences from 1990 to 2005 were evaluated for the type and prevalence of skin lesions, clinical course and response to treatment, and possibly associated systemic symptoms, as well as histologic, laboratory, and immunofluorescence findings. The classic plaque-type variant proved to be the most common presentation in children but not in adults, who more frequently had the annular granuloma-like variant. Unilesional forms were found to occur more frequently in children. The course was recurrent, although slowly progressing, with a mean duration of disease of 5 years for adults and 3 years for children. CONCLUSIONS: We emphasize the concept that the diagnosis of Wells syndrome is a clinicopathologic diagnosis. Although it should be classified within a spectrum that includes multisystem eosinophilic disorders, such as Churg-Strauss and hypereosinophilic syndromes, Wells syndrome, which has 7 variants, is a distinct cutaneous disease lacking systemic involvement.

Adult↗
PubMed2006

Juvenile gangrenous vasculitis of the scrotum: Is it a variant of pyoderma gangrenosum?

Juvenile gangrenous vasculitis of the scrotum was described by Piñol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Piñol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed.

Adolescent↗
PubMed2006

Reticular erythematous mucinosis occurring in a brother and sister.

Reticular erythematous mucinosis (REM) is a rare, primary cutaneous mucinosis clinically characterized by a persistent reticular erythema on the mid chest and mid-upper back, and histologically by a mononuclear cell infiltrate and deposits of mucin in the dermis. To our knowledge, the present report of REM occurring in a Caucasian man and his sister is the first reported case of familial REM. Since a host-specific immune response to unknown antigens may be involved in the pathogenesis of this entity, human leukocyte antigen typing was determined and compared to those reported in autoimmune diseases.

Adult↗
PubMed2005

Fatal CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma with multiorgan involvement.

A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient's history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+ extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial workup. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, the CD8+ epidermotropic cytotoxic subset comprises rare, highly aggressive forms characterized by metastatic spread to unusual sites such as the oral cavity, lungs, testis and the central nervous system but usually not to the lymph nodes. These cases seem to be distinct from mycosis fungoides with CD8+ phenotype, which shows a nonaggressive clinical behavior.

Aged↗
PubMed2003

Widespread livedoid vasculopathy.

A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an "idiopathic" form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal.

Adult↗
PubMed2003

Poikilodermatous subacute cutaneous lupus erythematosus.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus with unique clinical, immunological and genetic features. Among the unusual variants of SCLE, there is a poikilodermic presentation. However, to date, only 1 case of poikilodermatous SCLE has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE. METHODS: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002. RESULTS: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid. CONCLUSION: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.

Adult↗
PubMed2002

Ichthyosiform mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is a skin malignancy of T helper lymphocytes with a wide clinical spectrum. Among the atypical variants of MF, there is an ichthyosis-like presentation. However, to date, only 1 case of ichthyosiform MF has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course, and the pathological, immunohistochemical and molecular genetic findings on 4 patients with ichthyosiform MF. METHODS: A retrospective study was conducted. RESULTS: The 4 patients represented 1.8% of the 221 patients with MF seen by us since 1975. None progressed to systemic disease in up to 12 years (median, 10 years) after the onset of the cutaneous manifestations. Interestingly, skin lesions typical of so-called follicular MF (FMF) were associated in 3 of 4 cases, whereas cutaneous manifestations of classic MF were absent in all 4 patients. CONCLUSION: Ichthyosiform MF represents a rare variant within the clinicopathologic spectrum of MF usually featuring a benign course and a tendency to be associated with lesions of FMF but not with lesions of classic MF.

Adult↗
PubMedDate not supplied

Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases.

We examined, retrospectively, 239 patients (113 adults and 126 children) with LS, referred to our department from 1980 up to 2001. Clinical parameters evaluated were age, sex, LS variant, extracutaneous manifestations, duration of disease and follow-up. We also considered laboratory findings, most notably erythrocyte sedimentation rate, blood eosinophilia, antinuclear antibodies (ANA) and various circulating autoantibodies. Plaque morphea was the most common form in both groups (74 adults and 61 children). In contrast, linear scleroderma affected children much more frequently than adults (22 children vs 7 adults). When the limbs were involved, this variant could lead to severe orthopedic complications (10 children vs one adult patient). On the other hand, linear scleroderma of the scalp and face comprising scleroderma en coup de sabre and Parry-Romberg syndrome was also more frequent in children (14 children vs 5 adults) causing ocular (8 cases), oral (7 cases) and neurologic (8 cases) abnormalities. Typical of childhood were mixed forms (18 pediatric patients), characterized by combination of different LS variants, which usually followed a more protracted and complicated course and showed ANA positivity (11 cases). Among adults, Raynaud's phenomenon was found in 8 patients; interestingly, anticentromere antibodies were detected in 4 of these subjects, identifying a subset at risk for progression to systemic disease. Children and adults developed LS with analogous clinical and immunological features. However, the prevalence of LS variants differed between adult and pediatric populations, leading to different extracutaneous complications.

Adolescent↗
PubMedDate not supplied

Nitrendipine-induced subacute cutaneous lupus erythematosus.

A 66-year-old man presented with widespread annular and bullous subacute cutaneous lupus erythematosus (SCLE), developed after starting treatment for hypertension with the calcium channel blocker nitrendipine. A few days after withdrawal of the drug, while cutaneous manifestations were improving, left hemiparesis occurred. Laboratory investigations showed, in addition to anti-Ro, anti-La and anti-histone antibodies, the presence of lupus anticoagulant, anticardiolipin antibodies, prolonged APTT and thrombocytopenia. On the basis of the spontaneous regression of the patient's skin lesions after discontinuation of the drug, a possible relationship between nitrendipine intake, the clinical events and the biological findings is discussed.

Aged↗
PubMedDate not supplied

Skin infection due to Alternaria species in kidney allograft recipients: report of a new case and review of the literature.

A kidney allograft recipient developed a cutaneous infection 29 months after transplantation, due to the dematiaceous fungus Alternaria infectoria on his right forearm and left leg. Since the lesions were too large to be excised, the patient was treated only with systemic itraconazole and a reduction of the immunosuppressive therapy. After 4 months, the lesions were completely healed, and no relapses were observed at follow-up of 22 months. Twenty-seven other cases of cutaneous alternariosis have been described so far in renal transplant recipients. All types of immunosuppressive treatment can be associated with Alternaria infection, for which predisposing factors are jobs with frequent contact with earth, diabetes mellitus and skin trauma. In 70% of cases the infection occurred within the first year after transplantation. More frequently the lower limbs were involved and the lesions were multiple. Alternaria alternata was the commonest causative agent, followed by Alternaria tenuissima,Alternaria infectoria and Alternaria chartarum. The treatment is far from being standardized, but the best results are obtained with the surgical excision of the lesion(s) associated with systemic antifungal therapy. Since relapses are possible, strict control of the patients over time is essential.

Alternaria↗