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Background: Mucous membrane pemphigoid (MMP) with anti-laminin 332 autoantibodies may be associated with malignancies, however, current serological assays have considerable limitations. At present, no commercial test for anti-laminin 332 antibodies is available, restricting the diagnosis to specialized laboratories worldwide. Biochip immunofluorescence microscopy has shown promising results in selected cohorts of laminin 332-MMP patients., Objectives: To detect anti-laminin 332 antibodies by biochip immunofluorescence microscopy in a real-life cohort of MMP patients and compare the results with those from traditional immunoblotting., Materials & Methods: Sera were obtained from 31 patients with MMP, 28 with bullous pemphigoid, five with pemphigus vulgaris, five with paraneoplastic pemphigus, five with linear IgA bullous dermatosis, and 10 controls, and analysed by biochip immunofluorescence using human cells expressing laminin 332. Immunoblotting was performed using purified laminin 332., Results: MMP involved the oral mucosa in 65%, ocular mucosa in 9%, oral and ocular mucosae extensively in 13% as well as other mucosae in 13% of patients. Concomitant cutaneous involvement was reported in 35% of patients. Three MMP patients had an underlying malignancy. Anti-laminin 332 antibodies were detected in 2/31 (6%) cases by both methods. Based on immunoblotting, both laminin 332-positive sera reacted with α3 chain (in one case also with β3 chain). Both patients with anti-laminin 332 antibodies had extensive mucosal involvement and only one had cancer. Anti-laminin 332 antibodies were not detected in control groups., Conclusion: Biochip immunofluorescence is an appropriate technique to detect anti-laminin 332 antibodies which should be tested in patients with MMP.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of unknown etiology which usually occurs over the lower extremities; however, unusual presentations such as that involving the genital region have been described. Extracutaneous involvement of PG in the form of sterile neutrophilic infiltrates in various organs has infrequently been reported. We hereby describe a case of PG that was limited to the vulvar and perianal area in a 37-year-old female, with associated renal involvement in the form of a slight increase in the serum creatinine, microhematuria of glomerular origin and proteinuria. The patient had a rapid response of both her mucocutaneous lesions and renal dysfunction after the initiation of systemic steroids. The present case highlights the importance of evaluating all patients with PG for extracutaneous disease to avoid potentially harmful diagnostic or therapeutic procedures. Two other reasons for interest are the localized presentation of disease on the genital region and the presence of vascular involvement, albeit without signs of true vasculitis, vascular changes possibly being a histological hallmark of PG involving genitalia.

The pathophysiology of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) is unclear. Whether autoantibodies against desmoplakin (Dp) I and II play a pathogenic role or result from an epitope spreading phenomenon is uncertain. Our aim was to characterize the keratinocyte antigens recognized in EM, TEN and SJS. Of 33 patients studied, 2 had TEN, 1 SJS, 9 EM major and 21 EM minor, according to Roujeau's criteria. All sera were studied by indirect immunofluorescence (IIF), immunoblotting and immunoprecipitation. Twenty normal sera were used as controls. 10/33 sera reacted with polypeptides of 215 and/or 250-kDa molecular mass, which co-migrate with Dp I and II as assessed by an anti-Dp I and II monoclonal antibody on IB. In IP, none of the anti-Dp I and -Dp II 10 patient sera immunoprecipitated Dp I and/or II from radiolabeled keratinocyte extracts. Two of 10 patient sera (SJS, EM minor) reacted with DpI and II when denaturated by the IB procedure. The reactivity against intracellular antigens DpI and II as denaturated proteins may result from the epidermal damage produced by aggressive autoreactive T cells, playing therefore only a secondary role in the pathogenesis of the disease.

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.

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.

In the blood coagulation process, Factor XIII (F XIII) is responsible for the stabilization of the fibrin clot. The hypothesized ability of this blood coagulation factor to affect collagen synthesis and degradation led to its use in the treatment of scleroderma. However, the complex mechanism of action of F XIII remains unclear. The aim of our study was to assess possible effects of F XIII on endothelial damage, regarded as an early pathogenic event in systemic sclerosis (SSc). Thus, we measured plasma levels of von Willebrand factor antigen (vWF:Ag), a marker of endothelial cell injury, in 22 patients with SSc, 9 of whom were treated with F XIII and 13 who do not receive the drug. The plasma concentration of F XIII has also been analyzed. Interestingly, the vWF:Ag plasma levels within the group of SSc patients treated with F XIII were significantly lower than those of untreated SSc individuals (p < 0. 02). On the other hand, the highest mean value of vWF:Ag was found in a subset of untreated subjects having SSc with severe lung involvement, supporting a strict relationship between elevated levels of vWF:Ag and severity of the disease. In contrast, plasma concentration of F XIII resulted normal in all but 3 SSc patients, ruling out a deficiency of this blood coagulation factor as promoting the occurrence of SSc. These preliminary findings seem to support the hypothesis that F XIII may play an improving role on endothelial damage, other than the initially suggested action on collagen metabolism, in SSc.

The topical corticosteroids are widely used in the treatment of moderate psoriasis, because of their usefulness for reducing inflammation and controlling itching. The therapeutic effect of corticosteroids in different cutaneous inflammatory diseases may be partially explained by their varying ability to block in vitro the synthesis of different cytokines, which play a pivotal role in epidermal hyperproliferation and leukocyte recruitment into the skin. The purpose of the present investigation was to further elucidate the mode of action of mometasone furoate, a medium-high potency, topical corticosteroid, on adhesion molecules, cytokines and cytokine receptor expression in psoriatic skin. Using an immunohistochemical assessment, we examined lesional skin biopsies from ten psoriatic patients before treatment and after 1 and 3 weeks of therapy. The overexpression of alpha 2, alpha 3, alpha 6, and beta 1 integrins detected in the spinous layer of untreated psoriatic skin was significantly decreased after therapy in 8 out of 10 cases, characterized by only partial clinical remission. In the remaining patients, a disappearance of the above integrin reactivity paralleling the disappearance of psoriatic lesions was induced by the treatment. With the exception of GM-CSF, no or only marginal effects of mometasone furoate on the cytokine and cytokine receptor system were observed. A significant reduction of the positive immunostaining with anti-ICAM-1 and ICAM-2 monoclonal antibodies on dermal vascular endothelial cells was also seen. Thus, our findings indicate that the therapeutic effects of mometasone furoate in psoriasis are mediated principally by decreasing adhesion molecule expression and to a lesser degree by inhibiting cytokine synthesis.