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Biomedical subjects

E H Beutner

Publications and source records attributed to E H Beutner.

At least 19 recordsLinked to original sources

A case of dermatitis herpetiformis with IgA endomysial antibodies but negative direct immunofluorescent findings.

A patient with clinical findings of dermatitis herpetiformis (DH), negative direct immunofluorescent (DIF) findings for junctional IgA deposits in 2 biopsy specimens, and positive for IgA endomysial (AEmA) and tissue transglutaminase (tTG) antibodies responded initially to dapsone. After dapsone had to be discontinued because of side effects, a gluten-free diet and supportive therapy controlled the disease; the AEmA and tTG antibodies became negative. Our data on 10 consecutive DH cases examined by DIF and by serum studies for AEmA and antibodies to tTG, point to frequencies of 90% DIF positive and 70% AEmA and tTG positive cases. The use of both DIF and serum tests for AEmA and tTG reveals DH cases not detected by DIF alone that respond to gluten-free diet. Findings on autoantibodies to tTG, an enzyme that metabolizes gliadin, points to a role of tTG in the immunopathology of gluten-sensitive enteropathy and helps to explain the need for a gluten-free diet in the management of DH cases.

Autoantibodies↗

Relevance of complement fixing antinuclear antibodies.

BACKGROUND: Connective tissue diseases (CTDs) are a heterogeneous group of disorders defined by the association of a variety of clinical manifestations with immunologic and other laboratory findings. Overlap of syndromes and aberrant findings appear rather frequently. METHODS: Sera of eight antinuclear antibody (ANA) negative, cases of subacute cutaneous lupus erythematosus (SCLE) with antibodies to Ro (SS-A) and a ninth case with clinical and laboratory signs of Sjögren's syndrome and systemic lupus erythematosus (SLE) were tested for complement (C') fixing antinuclear antibodies (C-ANAs). The ninth case was examined in depth by direct immunofluorescence (DIF) and a two-step "C + DIF" test of biopsies for C' fixation to in vivo bound ANAs, as well as serum tests for C-ANA, ANA, and SCLE markers. RESULTS: Sera of five of the eight ANA negative, Ro(SS-A) positive SCLE cases had C-ANAs. The ninth case, a 50-year-old woman with clinical and laboratory signs of Sjögren's syndrome and SLE, gave a strong positive C + DIF reaction in the skin biopsy for in vivo bound ANAs that fix C', but negative ANAs and C-ANAs in routine serum tests; they revealed antimitochondrial antibodies. Serum tests on normal skin, however, revealed weak ANA and strong C-ANA reactions with in vitro fixed C'. CONCLUSIONS: ANA negative cases of SCLE or Sjögren's syndrome may have C-ANAs. A case with Sjögren's syndrome and signs of SLE had both in vivo and in vitro C' fixing ANAs. C-ANA tests can aid in the identification of such cases.

Antibodies, Antinuclear↗

Pemphigus erythematosus associated with thymoma: a case report.

A 52-year-old Chinese man was found to have a benign thymoma and pemphigus erythematosus (PE). Initially, most of his skin lesions appeared on the scars of the surgical incisions and suture sites three months after he underwent thymectomy. To our knowledge, this is the first report documenting the skin lesions of PE on the scars after thymectomy. Most of the previous cases had myasthenia gravis and skeletal muscle antibodies. However, in this patient, neither condition was present.

Cicatrix↗

Chronic ulcerative stomatitis with stratified epithelium-specific antinuclear antibodies.

BACKGROUND: In 1990 a new disease-associated antinuclear antibody was first recognized as a specific immunologic marker for a chronic form of ulcerative stomatitis (CUS). METHODS: Another case is reported herein and the subject of chronic ulcerative stomatitis with stratified epithelium-specific antinuclear antibodies (SES-ANA) is reviewed. Intraoral biopsies from this patient were submitted for microscopic examination and direct immunofluorescence. Indirect immunofluorescence studies were also performed. Serial SES-ANA titers were obtained with the patient on maintenance treatment with hydroxychloroquine. A skin biopsy of a recent lichenoid eruption was obtained and skin explants grown in the serum of this patient were studied in tissue culture with reference to SES-ANA binding and complement fixation. RESULTS: Biopsy and serum studies confirmed a diagnosis of CUS with SES-ANA in the patient reported. Skin biopsy showed lichen planus. The patient was treated with hydroxychloroquine with a favorable response. Serial SES-ANA titers did not parallel the disease activity. Among the substantive observations made from skin explants cultured in the serum of this patient was widespread fixation of C3 to the nuclei of basal cells. CONCLUSIONS: The case described herein extends the findings in CUS to include lichenoid skin lesions. Records show that at least four of 11 cases of CUS had skin lesions, whereas all had oral lesions. Stratified epithelium-specific antinuclear antibodies serve as the key marker of CUS. Skin explants grown in the serum of this CUS patient bind SES-ANA in tissue culture. Sections of explants fix complement. Titers of SES-ANA have been reported to parallel disease activity in one case, but not in the present case. Thus, there appears to be case-to-case variation. The treatment of choice for CUS is hydroxychloroquine.

Aged↗

Linkage of pemphigus vulgaris antibody to the major histocompatibility complex in healthy relatives of patients.

Pemphigus vulgaris (PV) is an autoimmune disease caused by high concentrations of antibody to an epidermal cadherin. The disease is associated with two kinds of HLA-DR4, DQ8 haplotypes dominantly distributed among Jewish patients, and these plus DR6, DQ5 haplotypes in non-Jewish patients. Low levels of the PV antibody were found in 48% of a total of 120 asymptomatic parents, children, and siblings of 31 patients, thus exhibiting dominant inheritance. The inheritance of these low levels of antibody in asymptomatic relatives was linked to the major histocompatibility complex with a highly significant logarithm of the odds score of 9.07, almost always to a DR4 or DR6 haplotype of the patient. Disease appears to occur in susceptible individuals with low levels of antibody when a second factor, either environmental or genetic, induces high levels, sufficient to produce blisters.

Autoantibodies↗

Erythema annulare-like acantholytic dermatosis (EAAD): nonbullous pemphigus or a new entity?

This article describes a case of unusual annular erythema-like dermatosis, with histological features of pemphigus foliaceus (subcorneal acantholysis) and IgG antibodies in circulation and bound in vivo to the keratinocyte surface. The reactivity of the antibodies, restricted to human squamous epithelium, was unique, differing from that of all known forms of pemphigus. This also was confirmed by immunoprecipitation. The problem is that these circulating antibodies could be missed if not determined on human substrate. It is to be established whether such cases present a new type of pemphigus or a unknown dermatosis with an autoimmune response of a pemphigus type.

Acantholysis↗

Preliminary, dermatologic first step criteria for lupus erythematosus and second step criteria for systemic lupus erythematosus.

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."

Humans↗

A characteristic cutaneous direct immunofluorescent pattern associated with Ro(SS-A) antibodies in subacute cutaneous lupus erythematosus.

BACKGROUND: Antibodies to Ro(SS-A) are present in most patients with subacute cutaneous lupus erythematosus (SCLE). Patients with SCLE also have cutaneous immune deposits. However, no correlation of Ro(SS-A) antibodies with immune deposits in skin biopsy specimens has been described. OBJECTIVE: The purpose of this study is to describe characteristic direct immunofluorescence (IF) findings in 32 patients and their association with serum antibodies and clinical diagnosis. METHODS: Serum from patients with characteristic cutaneous direct IF findings was examined for antinuclear antibodies by indirect IF and for antibodies to ribonucleoprotein, Sm, Ro(SS-A), and La(SS-B) by gel immunodiffusion. RESULTS: Twenty-two of 32 patients who exhibited discrete speckled IgG staining predominantly, but not exclusively, of the basal cell cytoplasm had SCLE. Ro(SS-A) antibodies were identified in 23 of 26 patients and Ro(SS-A) and/or La(SS-B) in 25 of 26 patients. CONCLUSION: Direct IF staining characterized by discrete speckled IgG deposits in the basal cell cytoplasm is associated with Ro(SS-A) antibodies and SCLE.

Acute Disease↗

Differentiation of bullous pemphigoid from epidermolysis bullosa acquisita on frozen skin biopsies.

Patients with bullous pemphigoid and epidermolysis bullosa acquisita may have similar clinical, histologic, and routine immunohistologic features. These two diseases can be distinguished by routine diagnostic studies either on a patient's serum tested by indirect immunofluorescence on salt-split normal skin or by obtaining a fresh perilesional skin biopsy, inducing a split at the lamina lucida, and testing for the site of IgG deposition by direct immunofluorescence. Often the serum studies are negative, while direct immunofluorescent studies yield the characteristic linear IgG staining of the basement membrane zone. To eliminate the need for a repeat biopsy to make a laboratory differential diagnosis, we studied the efficacy of salt-splitting perilesional skin biopsies that had been previously submitted and frozen for routine direct immunofluorescent studies. The biopsies were thawed, salt-split, and processed for direct immunofluorescence. Three epidermolysis bullosa acquisita biopsies and seven bullous pemphigoid biopsies examined demonstrated IgG staining at sites consistent with their respective diagnoses. The IgG appeared in the dermal side of the split biopsies in epidermolysis bullosa acquisita and predominantly, or exclusively, in the epidermal side in bullous pemphigoid. Thus the direct immunofluorescent study of previously frozen and subsequently salt-split skin biopsies may be used for the differential diagnosis of bullous pemphigoid from epidermolysis bullosa acquisita. In most cases, it may eliminate the need for a repeat biopsy.

Basement Membrane↗

A distinct stratum corneum antigen in psoriasis and its reactions with stratum corneum autoantibodies.

Stratum corneum antibodies are ubiquitous and can be detected by various immunological methods. Of these, the ones detected by hemagglutination undergo changes in antibody titers and have been implicated in psoriasis. The purpose of our study was to examine if differences exist in the activities of the antigens isolated from psoriatic scales in comparison to normal callus. Stratum corneum antigens were prepared by trypsin-phenol-water extraction. The water phase, which contains the stratum corneum antigen, was used to sensitize the red blood cells in the hemagglutination assay. The antibody activity in human sera was determined before and after absorption with antigens isolated from callus, psoriatic scales, and cell envelopes. We found notable differences in the antigens obtained from callus and psoriatic scales. These include higher antibody titers to the antigens of the scales, the presence of unique antigenic determinants on psoriatic scales and the localization of the antigen on cell envelopes. These immunological differences were corroborated by the marked biochemical differences of certain amino acids, most notably glycine and proline, and these differences were unique to psoriatic scales as they were not shared with other hyperproliferative disorders.

Amino Acids↗

Quantitative studies of immunofluorescent staining. VII. Quantitative reference standard slide for standardization of fluorescence microscopes.

We evaluated a quantitative reference standard (QRS) slide with 10-microns beads with different concentrations of Coulter Electronics green dye No. 1. Microfluorospectrophotometric readings of the QRS slides provided quantitative comparisons between the sensitivity of the different fluorescence microscopes. The visual comparisons between beads with a range of intensities of fluorescence of Coulter Electronics green dye No. 1 indicated that the end points vary with different optical systems as do the end points in standard indirect immunofluorescent titrations. Fluorescent emission wavelength of QRS slide beads gave the same peak as fluorescein-labeled beads; both differed from the peak of orange beads of an 'Optical Standard' slide. Since shelf life studies show no changes in fluorescence intensity of beads over a period of 27 months or longer, QRS slide beads can afford a device for standardization of fluorescence microscopes used for immunofluorescent tests.

Animals↗

IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis.

A patient with clinical features of herpetiform pemphigus of the foliaceus type had histologic findings consistent with pemphigus. Intercellular IgA deposits in a pattern like that of IgG in pemphigus were present. Circulating pemphigus-type IgA-class antibodies reacted first only with guinea pig and later in the disease also with monkey esophagus sections. IgG-class pemphigus antibodies were blocked by the IgA-class antibodies of this patient. In addition, the IgA-class pemphigus antibodies in this patient were blocked by the IgG-class pemphigus antibodies in tests on guinea pig and monkey esophagus. This indicates that the IgA-class antibodies in this patient were directed either to the same antigen as the IgG-class pemphigus foliaceus antibodies or to one that is close enough to it to give steric hindrance. The skin lesions responded poorly to systemic corticosteroid therapy. Dapsone therapy initially produced dramatic improvement, but the condition flared to the point that plasmapheresis, in addition to high doses of corticosteroids and cyclophosphamide, had to be used to control it.

Acantholysis↗

Studies of laminin and type IV collagen in blisters of porphyria cutanea tarda and drug-induced pseudoporphyria.

Blisters from five patients with porphyria cutanea tarda and two patients with drug-induced pseudoporphyria were examined by direct immunofluorescence and by immunofluorescence mapping with antibodies against laminin and type IV collagen to determine the level of subepidermal separation. Primary screening by direct immunofluorescence revealed the characteristic immune deposits in the vessel walls of the upper dermal plexus in all cases and at the dermoepidermal junction in five of seven cases. Type IV collagen and laminin were reactive in six and five cases, respectively, and appeared in the floor of the bulla. The findings were identical in porphyria and pseudoporphyria. In one case in which the bullous pemphigoid antigen could be detected, it appeared in the epidermal roof of the bulla. These findings indicate that the split in porphyria and pseudoporphyria occurs in the lamina lucida. We propose that a multistep mechanism involved in the induction of blisters may be similar in porphyria and pseudoporphyria.

Autoantigens↗