[Nailfold videocapillaroscopy in systemic sclerosis: diagnostic and follow-up parameters and correlation with both specific serum autoantibodies and subsets of skin involvement]. / La videocapillaroscopia periungueale nella sclerosi sistemica: parametri diagnostici e di follow-up della malattia e correlazione con il tipo di impegno cutaneo e con gli autoanticorpi specifici.

INTRODUCTION: The aim of the present study was to demonstrate, by nailfold videocapillaroscopy (NVC), the existence of diagnostic and follow-up parameters of microvascular damage in systemic sclerosis (SS) (grouped in the "early", "active" and "late" NVC patterns). The presence of the different subsets of skin involvement (limSS and difSS), as well as the role of some specific serum autoantibodies in the expression of the NVC parameters were investigated. METHODS: 245 consecutive SS patients were recruited and clinical data assessed. Antinuclear (ANA), antitopoisomerase I (Scl70) and anticentromere (ACA) antibodies were investigated in all patients. RESULTS: Giant capillaries and hemorrhages were confirmed to be the earliest NVC finding in SS (diagnostic parameters). The loss of capillaries, along with ramified capillaries and vascular architectural disorganization were validated as parameters of progression of SS microangiopathy. Really, both Raynaud's phenomenon (RP) and SS duration were detected longer in patients with the "late" than in those with the "active" or the "early" NVC pattern. Patients affected by limSS were found to have shorter disease duration, as well as showed more frequently the "early" or the "active" NVC patterns. Conversely, patients affected by the difSS showed longer disease duration and mostly the presence of the "active" or "late" NVC pattern. The Scl70 positivity was lower in the patients showing the "early" than in those with the "active" and the "late" NVC patterns, whereas no significant correlation was found between the Scl70 presence and both RP and SS duration. The ACA positivity was higher in patients showing the "early" NVC pattern, as well as in patients with longer disease duration. CONCLUSIONS: This study confirms that the identification of distinct NVC patterns may be useful to evaluate the severity and the stage of the SS microvascular damage. The presence of the Scl70 antibodies seems related to a more rapid progression of the SS microangiopathy. On the contrary, the presence of the ACA seems to be related to a slower progression of the SS microvascular damage. The SS peripheral microangiopathy is similar as in patients with limSS, as in those affected by difSS.

Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis.

BACKGROUND: Microvascular lesions are a predominant feature in systemic sclerosis (SSc) and seem to play a central pathogenetic role. Recently, we graded scleroderma microangiopathy by nailfold videocapillaroscopy (NVC) into three NVC patterns (early, active and late). The aim of the present study was to confirm, in a larger number of SSc patients, the presence of three patterns of microvascular damage, and to detect any possible relationship between these patterns and both specific serum autoantibodies and the subsets of cutaneous involvement. METHODS: Two hundred and forty-one consecutive patients (227 women and 14 men) affected by SSc were recruited. One hundred and forty-eight patients were affected by limited cutaneous SSc (lSSc) and 93 patients by diffuse cutaneous SSc (dSSc). The ages at onset of Raynaud's phenomenon (RP) and SSc, the durations of RP and SSc, ANA and antitopoisomerase I (anti-Scl70) and anticentromere (ACA) antibodies were investigated in all patients. The SSc patients were subdivided on the basis of the NVC pattern into three groups. RESULTS: A statistically significant correlation was found between the NVC patterns and the durations of both RP and SSc (P<0.001). Enlarged and giant capillaries, together with haemorrhages, constituted the earliest NVC finding in SSc (early NVC pattern). These abnormalities were mostly expressed in the active NVC pattern. Loss of capillaries, ramified capillaries and vascular architectural disorganization were increased in the late NVC pattern. Age and the duration of both RP and SSc were lower in 24 patients complaining of RP alone. Anti-Scl70 antibodies were statistically less frequent in the early vs both the active and the late NVC pattern, whereas no significant correlation was found between the presence of anti-Scl70 antibodies and the duration of either RP or SSc. ACA positivity was more frequent in patients with longer RP duration. Patients with lSSc had shorter SSc duration and showed the early or active NVC pattern more frequently. Conversely, patients with dSSc showed longer disease duration and mostly showed the late NVC pattern. CONCLUSIONS: NVC is an appropriate tool for differential diagnosis between primary and secondary RP through the clear recognition of the early NVC scleroderma pattern. This study confirms, in a large number of SSc patients, the existence of three distinct NVC patterns that might reflect the evolution of SSc microangiopathy. The presence of anti-Scl70 antibodies seems be related to earlier expression of the active and late NVC patterns of SSc microvascular damage. The presence of ACA seems to be related to delayed expression of the late NVC pattern.

[Rheumatoid arthritis and atherosclerosis]. / Artrite reumatoide ed aterosclerosi.

Evidence continue to accumulate indicating that patients with rheumatoid arthritis (RA) present an increased risk of cardiovascular disease (and death). The risk factors for coronary artery disease (CAD) in RA are not fully understood. However, a number of possible factors have been described, but more than one may be efficient, such as homocysteine, presence of antiphospholipid antibodies, altered serum levels of selected lipoproteins, and all together may have implications for the atherogenesis observed in these patients. Other factors that may facilitate this process, include corticosteroid use, methotrexate therapy and hormonal factors. However, the relative importance of these specific risk factors for the atherogenesis in this disease is poorly known. Recent findings indicate that cardiac death is increased in RA patients when compared with subjects without arthritis and that generally, the inflammatory process may contribute to atherosclerosis. In addition, other studies indicate that serum concentration of pro-inflammatory cytokines are found elevated at baseline, among patients at risk for future coronary occlusion.

[Lichen planus and thymoma. A case]. / Lichen plan et thymome. Un cas.

INTRODUCTION: Lichen planus is a chronic inflammatory disease of the skin, rarely associated with a thymoma. CASE REPORT: A 72-year-old woman with erosive buccal lichen, hypertrophic lichen planus of the lower limbs, severe myasthenia and acquired hypogammaglobulinaemia associated with thymoma. CD8 lymphocyte count was increased. In the months following surgical ablation of the thymoma, the clinical examination and laboratory findings progressively returned to normal. Two years later, the patient was in good health. DISCUSSION: To our knowledge, this is the first report of favourable outcome after surgical treatment despite the aggressive symptomatology. The particular outcome allow confirmation that the thymoma plays a causal role in this syndrome and emphasizes the effects a thymoma can have on the immune system.

[Zinc deficiency syndrome during TPN]. / Sindrome da deficienza di zinco in corso di NPT.

The zinc deficiency syndrome, also called enteropathic acrodermatitis, has been mostly observed in those pathologies of the gastroenteric system characterized by grave food shortages during long term TPN in association with inflammatory intestinal pathologies. The authors believe this to be caused of such syndrome, that in the case in question is demonstrated before the normal period described in the literature to be caused by both increased request related to TPN and a greater loss or less absorption due to intestinal phlogosis. The case described has been noted in a general surgery division in a young patient suffering from Crohn's disease for many years in treatment with medical therapy and now complicated by perianal abscess following burrowing on the outside subjected and therefore in treatment with artificial parenteral nutrition pre and post operative. Such pathology to be due when to begin a symptomatology characterized by consciousness alteration, diarrhoea, vesicular squamous cutaneous lesions around orifices, often infected by bacteria and mycosis in patients in treatment nutritional artificial continued for digestive apparatus diseases. The knowledge of this syndrome and its diagnosis lead, through integrative therapy, to its resolution in a short time. The authors describe the course of a clinical case occurred to then and ended with the patient's recovery. They underline the risks of ignoring this pathology.

[Mibelli's porokeratosis after bone marrow transplantation]. / Porokératose de Mibelli après une greffe de moelle.

A 24-year old male patient developed, on both legs, lesions typical of Mibelli's porokeratosis 22 months after bone marrow transplantation, under treatment with cyclosporin A. He denied any family history. Mibelli's porokeratosis seldom develops after an immunosuppressive treatment, and to our knowledge it has exceptionally been described after bone marrow transplantation. A possible complication of Mibelli's porokeratosis is the development of Bowen's disease, basal or squamous cell carcinomas. Immunosuppressive treatment might facilitate the degeneration. For this reason, these subjects should be periodically and carefully examined.

Cutaneous cryptococcosis resembling molluscum contagiosum in a homosexual man with AIDS. Report of a case and review of the literature.

A 43-year-old homosexual man with the Acquired Immunodeficiency Syndrome (AIDS) developed cutaneous molluscum contagiosum-like lesions on face, ears, neck, hands and feet. He was admitted to our unit with fever, malaise and headache. Cytologic examination of skin brushing revealed numerous encapsulated budding yeasts, identified as Cryptococcus neoformans. Such a finding calls for a cytologic examination of skin lesions in patient with AIDS who present with fever and headache, in order to rule out a potentially life-threatening fungal infection.