Background. Assessment methods for atopic dermatitis (AD) are not standardized, and therapeutic studies are difficult to interpret. Aims. To obtain a consensus on assessment methods in AD and to use a statistical method to develop a composite severity index.Methods. Consensus definitions were given for items used in the scoring system (extent, intensity, subjective) and illustrated for intensity items. Slides were reviewed to address within and between-observer variability by a group of 10 trained clinicians, and data were statistically evaluated with a two way analysis of variance. Two variants of an assessment system were compared in 88 patients at 5 different institutions. Data were analyzed using principal-component analysis. Results. For 5 intensity items studied (erythema, edema/papulation, oozing/crusts, excoriations, lichenification), within- and between-observer variability was good overall, except for edema/papulation which was difficult to assess with slides. In the series of 88 patients, principal-component analysis allowed to extract two unrelated components: the first one accounting for 33% of total variance was interpreted as a ‘severity’ component; the second one, accounting for 18% of variance, was interpreted as a ‘profile’ component distinguishing patients with mostly erythema and subjective symptoms and those with mostly lichenification and dryness and lower subjective symptoms. Of the two evaluation systems used, the one using the rule of nine to assess extent was found more workable than the one using a distribution × intensity product. A scoring index (SCORAD) combining extent, severity and subjective symptoms was mathematically derived from the first system and showed a normal distribution of the population studied. Conclusion. The final choice for the evaluation system was mostly made based on simplicity and easy routine use in outpatient clinics. Based on mathematical appreciation of weights of the items used in the assessment of AD, extent and subjective symptoms account for around 20% each of the total score, intensity items representing 60%. The so-designed composite index SCORAD needs to be further tested in clinical trials.

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A 43-year-old white man presented with a generalized eruption of lichen planus and tense blisters within the lichenoid lesions and also on clinically normal skin. Direct immunofluorescence (IF) studies revealed immunological and histopathological characteristics of lichen planus in the lichenoid lesions and of bullous pemphigoid in the bullous lesions, and indirect IF studies showed that the patient had circulating antibasement membrane antibodies. The coexistence of both disorders may indicate a possible link between the pathology in the junctional zone in lichen planus and the appearance of antibasement membrane zone antibodies and bullous lesions, respectively.

Pemphigus vulgaris in Israeli Ashkenazi and non-Ashkenazi Jews and in Austrian non-Jewish patients is strongly associated with the DR4 and DRw6 alleles of the HLA-D region class II genes. Restriction fragment length polymorphism analysis was undertaken with DQ.3, DQoa, and DRP cDNA probes. Hybridization with the DQB probe identifies Pvu II, BamHI, and EcoRV fragments that absolutely discriminate pemphigus vulgaris patients from healthy DR-, DQ-, and ethnic-matched controls. In contrast the DQa and DRB probes failed to identify disease-specific restriction fragment length polymorphism fragments. These studies indicate that DQwl and DQw3 polymorphisms carried by pemphigus vulgaris patients may be directly involved in predisposition to the disease or may be tightly linked to the susceptibility gene itself. To our knowledge, this is the first example of an HLA restriction fragment length polymorphism that is highly associated with susceptibility to autoimmune disease.Pemphigus vulgaris (PV) is a dermatological autoimmune disease mediated by autoantibodies directed against an antigen, intracellular cement substance, in the basement membrane of keratinocytes, resulting in blister formation (1, 2). This disease can be reproduced in monkeys or in mice after transfer of antibodies from PV patients (1, 2). Susceptibility to PV is strongly associated with serologically defined gene products of the HLA-D region (3, 4). technique. HLA-DR and -DQ typing was done on T-celldepleted B-cell-enriched lymphocytes by extended incubation cytotoxicity testing (7). The HLA antisera included alloantisera from the third Asia-Oceania Histocompatibility Workshop and sera of local origin.DNA Extraction, Digestion, and Binding to Nylon Membranes. Genomic DNA was extracted from peripheral leukocytes. After lysis of the erythrocytes, the leukocytes were treated with proteinase K at 0.2 mg/ml (Boehringer Mannheim) for 12 hr at 42°C. Two phenol/chloroform-isoamyl alcohol, 3:1 (vol/vol), and two isoamyl alcohol/chloroform, 1:24 (vol/vol), extractions followed. Thereafter, the DNA was precipitated with two volumes of absolute ethanol. After precipitation and several washes with ethanol, the DNA was dissolved in TE (1 mM Tris HCl/0.1 mM EDTA, pH 7.5) to give a final concentration of 1 mg/ml. Ten micrograms of each DNA sample was digested with 10 units of the following restriction enzymes: BamHI, Dra I, EcoRI, EcoRV, HincII, HindIII, Pst I, Pvu II, and Taq I. Digestion was carried out for 15 hr, and the DNA samples were subjected to electrophoresis at 30 V in agarose gel (0.7% agarose in Tris acetate buffer, pH 6.4, containing ethidium bromide at 20 ,ug/ml) for 36 hr. Gels were then transferred to Hybond N membranes (Amersham) as described by Southern (8).cDNA Probes and Hybridization. The DQa cDNA probe was furnished by Charles Auffray (College de France). The DQ,3 and DR8 cDNA probes were furnished by Hugh McDevitt and John Bell (Stanford University). Full descriptions of the probes appear elsewhere (9, 10). The probes were labeled by t...

A 33-year-old woman is described who suffers from an idiopathic loss of mid-dermal elastic tissue which leads to wrinkling of the skin and to discrete perifollicular protrusions. In accordance with Sheley & Wood (1977) we conclude that these findings represent a new entity for which we propose the term 'non-inflammatory dermal elastolysis'.