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Notes: Background  Imiquimod is an immune response modifier that acts through toll-like receptor 7 to induce cytokine production and a subsequent innate and adaptive cell-mediated immune response. Clinical studies have demonstrated clinical and histological clearance of superficial basal cell carcinoma (sBCC) after treatment with imiquimod 5% cream.Objectives  To evaluate the safety and clinical efficacy of imiquimod (Aldara™; 3M Pharmaceuticals, St Paul, MN, U.S.A.) 5% cream for the treatment of sBCC in a multicentre, randomized, parallel, vehicle-controlled, double-blind, phase III clinical study conducted at 26 centres in Europe.Methods  Subjects who had at least one histologically confirmed sBCC tumour were randomized to apply imiquimod or vehicle cream to the target tumour once daily, seven times per week (7 ×/week) for 6 weeks. The target tumour location was identified with an indelible ink mark before treatment initiation. The treated tumour site was clinically assessed for treatment response at 12 weeks post-treatment and was then excised for histological evaluation. Efficacy assessments included the composite response rates (proportion of subjects with clinical and histological clearance) and response rates solely based on histology (proportion of subjects with histological clearance). Safety assessments, which included adverse events and scoring of local skin reactions (LSRs), were carried out throughout the study.Results  In total, 166 subjects were enrolled in this study. For the intent-to-treat dataset, there was a statistically significant difference between imiquimod and vehicle groups for both composite clearance rates (clinical and histological assessments) and histological clearance rates. Composite clearance was demonstrated in 77% and 6% of subjects treated with imiquimod and vehicle cream, respectively. Histological clearance was demonstrated in 80% and 6% of subjects treated with imiquimod and vehicle cream, respectively. The most frequently reported safety findings were investigator-assessed LSRs and spontaneous reports by subjects of application site reactions, which occurred more frequently in the imiquimod group than in the vehicle group.Conclusions  Imiquimod 5% cream administered 7 ×/week for 6 weeks is a safe and effective treatment for sBCC when compared with vehicle cream.

Notes: The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-κ antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280–290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal–epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.

Notes: Many nail abnormalities have traditionally been described in association with rheumatoid arthritis (RA), but their specificity has never been assessed in a controlled study. Our purpose was to evaluate the frequency and the specificity of nail changes associated with RA in a case-controlled study including 50 patients suffering from RA and 50 controls. For each patient, a general skin examination was performed and the 20 nails were examined. The nail features were noted and classified. Ax2 test or a Fisher test was used to compare the two groups. The only nail abnormalities significantly associated with RA were longitudinal ridging on nine or 10 finger nails (29 patients in the RA group vs. three in the controls. X2: p〈0.001) and clubbing on at least one nail (24 patients vs. 10.X2:p〈0.01). Other nail changes were noticed but were not frequent enough to be significant. The presence of longitudinal ridging on the finger nails was significantly associated with RA.

Notes: Summary Cutaneous macroglobulinosis is a rare cutaneous manifestatiou of Waldenstrom's disease. Lesions are thought to result from accumulation of macroglobulin in the dermis and are therefore called IgM storage papules. Ultrastructural findings in the previously reported cases were contradictory and the nature of the deposits was not established by electron microscopy. The purpose of this study was to analyse such deposits by the use of immunoelectron microscopy. A 60-year-old woman had multiple erythemalous papules for 1 year. The histopathological changes consisted of plasmocytic infiltration of the dermis and eosinophiiic deposits. The skin changes and other investigations led to a diagnosis of Waldenstrom's disease. Samples from normal and diseased, skin were analysed by electron microscopy and by immunogold labelling with anti-IgM antibodies, after Lowicryl K4M embedding. An extracellular electron dense granular and filamentous material was observed in the mid- and upper dermis, between and within the collagen bundles. No periodicity was noted and no deposits were seen at the dermoepidermal junction. Immunoelectron mieroscopy showed a positive labelling located only on these deposits, in both normal-appearing skin and in lesions. In this patient, immunoelectron microscopy clearly demonstrated the presence of large amounts of IgM in the dermis, which were found in the lesions of cutaneous macroglobulinosis and in normal skin. These results suggest that the IgM storage papules result from a greater density of deposits rather than a site-specitic accumulation.

Notes: Annular lesions are rarely reported in the clinical spectrum of leucocytoclastic vasculitis, except in the acute baemorrbagic oedema of the skin. We report tbree patients who suffered from an extraordinary recurrent annular dermatitis, for 4 years in one case and for decades in tbe otber two. The eruption was cbaracterized by purpuric lesions that bad a centrifugal evolution, creating target0-or polycyclic patches disseminated on the limbs and trunk. Tbe palienls' general condition remained excellent during tbe attacks. All lesions spontaneously disappeared within 2 weeks, but recurred monthly. In all three cases, the histological changes were consisteni with leucocytoclastic vasculitis. One patient had ulcerative colitis and another had a benign immunoglobulin A (IgA) monoclonal gammopalhy. These two patients showed a good response to dapsone tberapy. This dermatosis probably represents a new and rare variant of leucocytoclastic vasculitis.

Notes: Background  Although deficiencies in the early components of the complement system were among the first identified genetic risk factors for systemic lupus erythematosus (SLE), only a few studies addressed their significance in patients with cutaneous LE (CLE). Among environmental factors, it was postulated that cigarette smoking might intervene in the pathogenesis of LE.Objectives  To describe the clinical and biological features of patients with CLE and a complement deficiency. A secondary objective was to assess cigarette smoking in patients with CLE.Patients and methods  A retrospective study including all patients diagnosed as having LE between 1995 and 2003 in the Dermatology Department of Strasbourg University Hospital. Patient charts were reviewed and those patients in whom a C4 and/or C2 deficiency was diagnosed were included. Two patients with a combined C2/C4 deficiency were analysed in detail.Results  There were 48 females and 37 males (F/M ratio = 1·3), with a mean age of 41 years at diagnosis; 73% of the patients had chronic LE and 27% subacute CLE. Among 32 screened patients, 24 patients with a mean age of 36 years had a complement deficiency; 17 had a C4A deficiency, five a C4B deficiency and two a combined C4A/C2 deficiency. A high proportion (58%) of these patients was male; 82% of the patients were smokers. This was especially true in males: 94% were smokers compared with 69% of females.Conclusions  Partial deficiency of C4, C2 or C4 and C2 is a common finding in patients with CLE. Most male patients with CLE are smokers. It is thus suggested that the combination of cigarette smoking and complement deficiency could be a risk factor for LE in men.

Notes: Summary Background Most published series of basal cell carcinomas (BCCs) do not take into account the histopathological subtype. Recent studies suggest that the nodular and superficial types could be induced by different causal factors. Objectives To analyse the three major subtypes of BCC with regard to sex, age and anatomical distribution. Methods We retrospectively included all cases of BCC diagnosed at a single centre of dermatopathology during 1967–96. The diagnosis and subtype (nodular, superficial, morphoeiform) were confirmed by two dermatopathologists. Gender, age at excision, number of tumours and location were recorded, and analysed by histopathological subtype. Results We recorded 13 457 cases in 10 245 patients (M/F ratio 0·92) of mean age 65 years. Of the BCCs, 78·7% were nodular, 15·1% superficial and 6·2% morphoeiform. Nodular tumours occurred at a mean age of 66·3 years, whereas superficial tumours were excised earlier (63·0 years) and more frequently in women (M/F ratio 0·96). Patients with morphoeiform BCC had a mean age of 65·8 years and were predominantly women (M/F ratio 0·73). Both nodular and morphoeiform types predominated on the head (89·6% and 94·8%), whereas the trunk was the most common location for the superficial type (45·9%). Superficial carcinomas were predominantly located on the head in women (44·8% vs. 34·7% in men), whereas they predominated on the trunk in men (49·9% vs. 42% in women). Tumours of the trunk were excised earlier than those of the face, whatever their histological subtype, in both men and women. Conclusions This is the largest series of BCCs in the literature, and shows striking differences in anatomical distribution, sex and mean age according to histological type. The results suggest that the tumours of the trunk, and not only those of the superficial type, could represent a particular subtype of BCC. The analysis also suggests that the hypothesis of chronic vs. intermittent sun exposure cannot be simply extrapolated to BCC. Other causal factors, such as a genetic predisposition, could be involved in the development of carcinomas located on the trunk.