Ernst H. Beutner

Scl 70 antibody—a specific marker of systemic sclerosis

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.

Chronic ulcerative stomatitis associated with a specific immunologic marker

Four elderly women with chronic oral ulcerations are described. The lesions are chronic, erosive, or ulcerative; occur on the gingival, buccal, or lingual mucosa; and may occur in the form of desquamative gingivitis. The histopathologic findings are nondiagnostic. The disease is refractory to local and systemic corticosteroids, but treatment with hydroxychloroquine may be effective. Both in vivo binding to the oral mucosa and skin of a stratified epithelium-specific antinuclear antibody and high titers of these antibodies in serum are markers of this disease, which we refer to as chronic ulcerative stomatitis associated with stratified epithelium-specific antinuclear antibody.

A unique form of endemic pemphigus in northern Colombia

BACKGROUND Endemic forms of pemphigus are a unique group of autoimmune diseases that represent opportunities to study interactions of the environment and genetics with the immune system. The restriction to relatively well-defined regions of South and Central America and perhaps Africa characterizes these diseases. OBJECTIVES The aims of this study were to confirm the endemic nature of a new type of autoimmune disease occurring in a mining town in northeastern Colombia in the El Bagre area, to characterize it, and to compare it with other forms of endemic pemphigus. METHODS A 10-year prospective, controlled epidemiologic, humanitarian, and immunologic fieldwork case-control survey was performed in El Bagre, Colombia. RESULTS Our work revealed that this disease is endemic in rural areas surrounding El Bagre. The disease appeared in 4.7% of middle-aged and older men and postmenopausal women from these rural areas. This disease differs from previously described forms of endemic pemphigus. It shares some heterogeneous immunoreactivity with paraneoplastic pemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form. CONCLUSION We have confirmed endemic pemphigus foliaceus in El Bagre as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.

Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia

BACKGROUND We previously described a new focus of endemic pemphigus foliaceus in rural areas of El Bagre, Colombia, with clinical and direct immunofluorescence characteristics of pemphigus erythematosus. OBJECTIVE The aim of this study was to characterize autoantigen profiles for 34 serum samples obtained from patients with this condition. METHODS Immunofluorescence, various immunoblot analyses with different antigen sources and detection methods, and immunoprecipitation were performed. RESULTS Immunofluorescence with the use of human skin sections showed IgG autoantibodies against keratinocyte cell surfaces in all 34 serum samples. Some samples also showed weak reactivity with the basement membrane zone. The results of immunoblot and immunoprecipitation analysis indicated that all sera had antibodies reactive with desmoglein 1, the pemphigus foliaceus antigen. In addition, in various immunoblot assays, many sera reacted with several other proteins with molecular weights of 250 kd, 210 kd, and 190 kd, which appear to correspond to desmoplakin I, envoplakin, and periplakin, respectively. CONCLUSION This endemic pemphigus disease in El Bagre showed immunologic features similar to pemphigus foliaceus or erythematosus. In addition, paraneoplastic pemphigus-like reactivity with various epidermal antigens was detected.

CAN PEMPHIGUS BE PROVOKED BY A BURN

Summary.— The authors describe a case of pemphigus, confirmed histologically (acantholysis) and by immunofluorescence studies (circulating pemphigus autoantibodies in the titre of 1:1280, and in vivo bound immunoglobulins IgG, and complement in the intercellular substance of the epidermis). The symptoms developed 10 months after a burn which refused to heal and involved the site of the burn and other skin areas.

Juvenile dermatitis herpetiformis in the light of immunofluorescence studies.

Summary.— Eight cases of juvenile dermatitis herpetiformis (JDH) were investigated by direct and indirect IF methods with anti‐IgG and anti‐IgA conjugates.

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.

FACTORS CONTRIBUTING TO OCCASIONAL FAILURES IN THE LABORATORY DIAGNOSIS OF BULLOUS PEMPHIGOID BY INDIRECT IMMUNOFLUORESCENCE

Summary.— The basement membrane antibodies in 3 sera of patients with bullous pemphigoid were studied by means of indirect immunofluorescent staining chessboard titrations on monkey, rabbit and guinea‐pig lip tissue. Comparisons of the results of these studies with those obtained in previous studies of this type with 2 other sera, revealed wide variations in the spectra of specificities of the bullous pemphigoid antibodies. Monkey oral or oesophogeal mucosa appears to be the substrate of choice for routine tests for these antibodies, although this must be regarded as an approximation of the “ideal antigen”.

The diagnosis of pemphigus.

I have prepared this brief, up-to-date review on the role of autosensitization in the pathogenesis of pemphigus because some recent developments shed added light on that question and because the relevant information in the literature is not set forth in concise form (Beutner, 1969; Jablonska, 1970; Anon, 1968), Detailed documentation of most of the salient factors can be found in a monograph on the subject which came into print as this was being written (Beutner et al., 1970) but even it does not contain reviews of important observations that have since come to light.

Evaluation of spontaneous autoimmune thyroiditis in chickens of the obese strain (OS) by defined immunofluorescence.

Chickens of the Obese strain (0s) of White Leghorn spontaneously develop chronic thyroiditis during the first few weeks of life.’ Clinically the disease is characterized by symptoms of hyp~thyroidism,”~ and histologically the thyroid glands exhibit various degrees of lymphoid infiltration?-6 Autoantibodies to thyroglobulin have been demonstrated by means of passive hemagglutination and double diffusion in gel,’ and more recently by immunofluorescence8 techniques. In order to obtain reproducible results of immunofluorescent staining, standardized experimental conditions had to be established for the chicken system. Avian antibodies require special conditions for optimal reactivity which differ significantly from those used for the study of mammalian sera.9310 The present report deals mainly with the technical aspects of the immunofluorescence work in 0s chickens.