Library

Notes: Serological studies using indirect immunofluorescence, double immunodiffusion and immunoblot with recombinant Topo I and kinetochore antigens were performed in 180 patients with systemic scleroderma. In the sera of 10 patients, we found coexisting ACA and Scl 70 antibodies, and this was confirmed by all three methods. The relationship between the coexistence of these antibodies and the clinical features of the disease is not clear, although in all these cases there was pronounced vascular involvement.

Notes: Indirect immunofluorescence (IIF) and double immunodiffusion (DID) were performed on the sera of 64 patients who had a nucleolar immunofluorescence pattern on HEp-2 cells. Forty-nine of the sera were from 296 patients with systemic scleroderma (SSc) and 15 sera were from 214 patients with systemic lupus erythematosus (SLE). A homogeneous nucleolar staining pattern was found in 45 of the 64 sera (70.3%), a clumpy fluorescence associated with fibrillarin antibody in 14 (21.8%) and a speckled pattern was found in five of the sera (7.8%).There was a clear correlation between the sera which showed a homogeneous nucleolar staining pattern with symptoms of the polymyositis/scleroderma overlap syndrome that differed from SSc with concomitant myositis. The clumpy pattern was mainly associated with diffuse scleroderma and the speckled pattern with limited scleroderma (previously called acrosclerosis).

Notes: Scl 70 antibodies were tested for in 107 patients with systemic sclerosis: 68 with acrosclerosis and 39 with diffuse scleroderma. Anticentromere antibodies (ACA) and other antinuclear antibodies (ANA) were tested for by indirect immunofiuorescence on HEp-2 cells. Positive results for Scl 70 antibodies were obtained in 77percnt; of cases of diffuse scleroderma and 44percnt; of acrosclerosis. ACA and Scl 70 antibodies were found to be mutually exclusive. If acrosclerosis cases positive for anticentromere antibodies are excluded, the percentage of acrosclerosis cases positive for Scl 70 was 63percnt;. ACA were found to be a marker of a benign, abortive subset of acrosclerosis with almost no cutaneous involvement (CREST), whereas Scl 70 did not discriminate between acrosclerosis and diffuse scleroderma. On HEp-2 cells Scl 70 positive sera gave a characteristic, fine speckled, almost homogeneous nuclear staining pattern.

Notes: Natural killer (NK) cell activity of peripheral blood mononuclear cells from 28 patients with cutaneous discoid lupus erythematosus, seven with subacute cutaneous lupus erythematosus, and 17 with systemic lupus erythematosus was studied using a 4 h 51 Cr release assay using K-562 cells as target cells. NK cell activity was found to be markedly decreased not only in patients with systemic lupus erythematosus but also in a proportion of cases of subacute cutaneous lupus erythematosus and cutaneous discoid lupus erythematosus (57% and 39% respectively). There was a relationship between the activity and severity of LE and the decrease in NK cell activity.

Notes: Peripheral blood mononuclear cells from 63 patients with systemic scleroderma, including incipient or prodromal acrosclerosis, and from 20 healthy individuals were tested for natural killer (NK) cell activity and antibody-dependent cell cytotoxicity in a 4 h 51Cr release assay using K562 and L1210 cell lines respectively. In patients with systemic scleroderma natural killer cell activity was significantly decreased compared with the controls. NK cell activity was markedly lowered in patients with diffuse scleroderma and in transitional form acrosclerosis-diffuse scleroderma, and was normal in cases of acrosclerosis and/or CREST syndrome and in cases of prodromal or incipient scleroderma. Antibody-dependent cell cytotoxicity of mononuclear cells from the systemic scleroderma patients was within the normal range. The lowered natural killer cell activity correlated with the severity of systemic scleroderma, in terms of the extent of skin and organ involvement.

Notes: Direct immunofluorescence (DIF) of the skin biopsy specimens was performed in 62 patients with systemic scleroderma: 26 cases of acrosclerosis (group 1), 26 cases of diffuse scleroderma (group 2) and ten cases of transitional form acrosclerosis-diffuse scleroderma (group 3). If epidermal nuclear fluorescence and lupus band test (LBT) were considered, the results were positive in 15.3% in group 1, 42.3% in group 2, and 10% in group 3. LBT was positive in 13.5% of the cases in groups 1 and 2 and negative in group 3. The immunoglobulin deposits at the dermoepidermal consisted mainly of IgM or a combination of IgG and IgM. There was no correlation between the LBT and antinuclear antibodies as detected by indirect immunofluorescence (IIF) or double immunodiffusion (DID).

Notes: Background. Comparisons of cases of systemic lupus erythematosus (SLE) with cases of rheumatoid arthritis and other rheumatologic disorders affords the basis of the 1982 revised criteria of the American Rheumatism Association (ARA) for classifying SLE cases. We address three questions: Do comparisons of LE cases with non-LE cases that have suggestive skin lesions yield criteria for use in dermatology clinics for primary classification of cases with photo distributions of skin lesions? Do comparisons of SLE with cutaneous LE cases yield the same or similar criteria to the revised ARA criteria for SLE? How should subacute cutaneous LE cases be evaluated for signs of significant systemic involvement? Methods. Discriminant analyses on 168 cases with skin lesions suggestive of LE were performed using data based on the ARA criteria for SLE and study factors for cutaneous LE suggested by the European Academy of Dermatology and Venereology. Results. These yielded two sets of criteria: (1) The 11 preliminary, dermatologic first step criteria (10 plus 1 for discoid lesions and histology) serve to classify cases as LE or non-LE. (2) The 11 preliminary, dermatologic second step criteria classify LE cases as cutaneous LE or systemic LE. Interestingly, 5 of 11 of these second step criteria differ from the 11 ARA criteria for systemic LE. These second step criteria afford a useful means of distinguishing between subacute cutaneous LE cases with or without significant systemic involvement. Conclusions. The study factors included in both the first and the second step criteria fall into three groups, notably clinical criteria, laboratory criteria, and “added study factors.” The latter factors distinguish between the groups compared (LE VS. non-LE and cutaneous vs. systemic LE) but not as well as the study factors included as “criteria.”

Notes: : Responses to ultraviolet B (UVB; 290–320 nm) were tested in 227 patients with main forms of lupus erythematosus (LE): discoid LE (OLE), discoid disseminated LE (DOLE), systemic LE (SLE), and subacute cutaneous LE (SCLE). Four parameters were evaluated: minimal erythema dose (MED), its distribution, persistence of erythema, and photoreproduction of lesions. Patients with LE differed considerably from controls in their UVB reactivity. In addition, there were significant differences between various LE forms, even after a single UV exposure. Lowered MED in comparison to controls was observed most frequently in SLE patients (64.4%) and least frequently in OLE patients (32.1%). Prolonged persistence of erythema induced by 1–2 MED was a constant finding in SCLE. In SLE and DOLE, it was observed in more than 80% of patients, and in OLE in 56.7% of examined cases. Photoreproduction of lesions after single UVB exposure was observed most frequently in SCLE (62.5%) and only in 10% of OLE patients.

Notes: Abstract: A group of 140 cases of various forms of lupus erythematosus (LE) were examined for 24 variables, inducting the 11 criteria of the American Rheumatism Association (ARA) for the classification of systemic lupus erythematosus (SLE), and 13 additional criteria suggested by the European Academy of Dermatology and Venerology (EADV) for studies of cutaneous LE with or without systemic involvement. The EADV study factors included skin histopathology and immunopathology, complement and IgG levels, and other laboratory tests, as well as selected clinical findings, most notably the papulosquamous and/or annular lesions that characterize sub acute cutaneous LE (SCLE). The patients examined included 50 SLE, 35 SCLE, 30 discoid LE (DLE), 25 disseminated DLE (DDLE), and 17 polymorphous light eruption (PMLE) cases. Preliminary analyses of the data reveal the following: (1) The SCLE cases differed significantly from SLE, DLE, and DDLE in 10 of 11 ARA criteria (all but photosensitivity). (2) The frequencies of positive findings in SCLE also differed significantly for 11 of 13 EADV study factors. (3) While no significant differences appeared in the frequency of photosensitivity between the five study groups, photo-testing revealed significant increases in the frequency of persistence of the photo reactions for 10 days and their Koebnerization in the SCLE cases. (4) The presence of SS-A (Ro)/SS-B (La) antibodies had some predictive value for the appearance of systemic involvement in SCLE, as seen by the increased frequencies of five or more ARA criteria, although highly significant differences from SLE occurred in the absence of renal involvement and lower frequency of ANA and LE band test. We conclude from these studies that the classification of the major forms of cutaneous LE, with and without systemic involvement, requires not only the ARA criteria, but also the examination of other factors such as those recommended by the EADV, and that the study groups should include SLE, SCLE, DLE, DDLE, and non-LE controls such as PMLE.

Notes: A 28-year-old man was admitted to our department for investigation in 1992. He presented with a red, scaly, centrifugally spreading eruption, which had appeared in 1990, beginning on the neck and thorax, and later extending to the trunk and limbs. The cutaneous lesions, located mainly on the trunk and proximal upper limbs, were arranged in rings, with a slightly raised prominent scaling edge ( 〈link href="#f1"〉Fig. 1a). The characteristic feature was the presence of rings or waves within already existing rings, whereas the central part was flattened, with the texture of normal skin. The concentric figurate lesions resembled a wood grain pattern ( 〈link href="#f1"〉Fig. 1b). The clinical picture was strikingly similar to tinea imbricata; there was, however, no itching, and repeated mycologic studies did not disclose Trichophyton concentricum.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD996:IJD_996_f1a"/〉〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD996:IJD_996_f1b"/〉(a) Concentric bands migrating over the skin surface in waves. (b) Wood grain pattern characteristic of erythema gyratum repensThe histology was not characteristic. The epidermis, which was slightly edematous, was covered with a heavy crust. In the dermis, a sparse inflammatory infiltrate, somewhat more pronounced in the subpapillary areas, was composed of lymphocytes with some eosinophils. Periodic acid–Schiff (PAS) and other stains for mycotic infection were negative.The general condition was not affected and laboratory studies did not show any abnormalities, except for low serum protein (5.1 g/L) and decreased gamma globulins (10.5%). Cell-mediated immunity was preserved. Immunofluorescence studies (direct and indirect) were negative.In spite of repeatedly negative mycotic examinations and due to the striking similarity to tinea imbricata, we applied various antimycotic therapies (terbinafine, itraconazole), with no effect. The figurate pattern, with normal skin in between, altered from day to day, while new concentric rings appeared within the cleared skin. The migrating rate was about 2–3 cm per 2 weeks. The patient had undergone a thorough search for internal malignancy. During the follow-up period of 1992–98, cutaneous involvement slowly became almost generalized (1996), and the confluent lesions formed large plaques, but still with pronounced concentric rings.Transitional blood eosinophilia (27% in 1993 and 11% in 1996) regressed with no therapy. Since 1995, antibodies to HBs and HBc have been present with no clinical symptoms of liver disease. The blood proteins increased to 7.0 g/L, and gamma globulins to 17.2% (normal). The histology, studied repeatedly, started to display some signs of psoriasis from 1996 and, in 1998, was already consistent with the disease ( 〈link href="#f2"〉Fig. 2). RE-PUVA (0.8 mg/kg acitretin and UVA 0.8 J/cm2 ) was applied for 2 weeks before the patient interrupted the therapy. In spite of this, there was further improvement and, in 1999, the patient was almost free of lesions with some abortive rings left. From time to time, single vesicles appeared within the elevated borders of the rings. The histology of such vesicles was consistent with abortive pustular psoriasis ( 〈link href="#f3"〉Fig. 3).〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD996:IJD_996_f2"/〉Histology of concentric rings 8 years after the appearance of the first cutaneous changes consistent with psoriasis (hematoxylin and eosin, × 200)〈figure xml:id="f3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD996:IJD_996_f3"/〉Biopsy taken from a small vesicle at the margin of the ring. The histologic pattern shows features of pustular psoriasis (hematoxylin and eosin, × 200)