Comparisons of pathologic and normal skin reactive autoantibodies and the interference phenomenon.

Immunofluorescent serum studies of the roles of the two groups of normal complement-fixing autoantibodies in psoriasis are complicated by the interference phenomenon. Both antibodies have the potential to react in vivo at sites of trauma and in psoriasiform lesions. In serum tests, only one or the other reacts, as demonstrated by immunofluorescent serum tests and absorption studies with isolated stratum corneum antigen.

When to wait for more evidence? Real options analysis in proton therapy.

Purpose: Trends suggest that cancer spending growth will accelerate. One method for controlling costs is to examine whether the benefits of new technologies are worth the extra costs. However, especially new and emerging technologies are often more costly, while limited clinical evidence of superiority is available.

Can direct immunofluorescence testing still be accurate if performed on biopsy specimens after brief inadvertent immersion in formalin?

Background: Direct immunofluorescence is useful in the diagnosis of autoimmune, vesiculobullous, and connective tissue diseases. Michel medium is typically indicated for transport, but clinicians may inadvertently place samples into formalin.

Objective: We set out to determine the amount of time that specimens can remain in 10% buffered formalin and still retain their diagnostic properties.

A case of bullous disease limited to the skin illustrates the spectrum of neoplasia-induced autoimmunity.

Although the initial report of paraneoplastic pemphigus described individuals with mucocutaneous blistering disease, subsequent reports identified patients with lichen planus or graft versus host disease-like changes. We describe a patient with fatal autoimmune blistering disease with absence of mucous membrane lesions. The pattern of complement indirect immunofluoresence helped identify the prognosis prospectively.

Intravenous immunoglobulin selectively decreases circulating autoantibodies in pemphigus.

Background: Autoantibody-mediated diseases such as pemphigus are caused by a single or very limited number of pathogenic autoantibodies. A major problem with all current therapies for these diseases is that they target all antibodies rather than selectively targeting only pathogenic antibodies. The following study was conducted to confirm observations made in a limited number of patients that suggest intravenous immunoglobulin (IVIg) may be able to selectively lower serum levels of only abnormal autoantibodies.

IgG4 as the predominant IgG subclass in pemphigoides gestationis.

Background: Pemphigoides gestationis (PG) is a blistering disorder of pregnancy caused by antibodies against basement membrane proteins. They are directed against the 180 kD bullous pemphigoid antigen (BPAg2), towards the epitopes within the NC 16A domain. There are many similarities between pemphigoid gestationis and bullous pemphigoid (BP), but the literature so far indicated different immunofluorescence results in regards with C3 and IgG, and IgG subclasses (IgG4 vs.

Pemphigus, pregnancy, and plasmapheresis.

Pemphigus vulgaris (PV) is an autoimmune blistering disorder that usually occurs in the fifth and sixth decades of life but may occur at younger ages and during pregnancy. Circulating intercellular antibodies directed at desmosomal proteins may cross the placenta and place children at risk for neonatal pemphigus (NP). We describe the case of a pregnant woman with PV treated successfully with a combination of systemic corticosteroids and plasmapheresis.

Complement-fixing properties of antinuclear antibodies distinguish drug-induced lupus from systemic lupus erythematosus.

The immunofluorescence antinuclear antibody (ANA) test has been widely used to monitor autoimmune disease, but its value for diagnostic purposes is compromised by low specificity and high prevalence in disease-free individuals. The capacity of autoantibodies to fix serum complement proteins when bound to antigen is an important effector function because this property is associated with acute and chronic inflammatory processes. The current study evaluates the complement-fixing properties of antinuclear antibodies (CANA) in three well-defined and clinically-related patient groups: systemic lupus erythematosus (SLE), drug-induced lupus (DIL) and drug-induced autoimmunity (DIA).

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.

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.

A nonfatal case and 2 fatal cases of paraneoplastic pemphigus: can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases?

We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma.

Bullous skin disease: an unusual allergic reaction to vancomycin.

Severe reactions due to vancomycin are uncommon. We describe a case of vancomycin-induced linear immunoglobulin A bullous disease and review the literature pertinent to this entity. This is a rare subepidermal blistering disorder, with a heterogenous clinical presentation.

Diagnostic features of pemphigus vulgaris in patients with bullous pemphigoid. Molecular analysis of autoantibody profile.

Background: The simultaneous presence of features of pemphigus vulgaris (PV) in patients with bullous pemphigoid (BP) has previously been reported in the literature.

Objective: The purpose of this retrospective study is to present 13 patients with an initial diagnosis of BP, who subsequently demonstrated coexistent serological features of both BP and PV.

Methods: The following information on each patient was documented, at the time of initial diagnosis: clinical profile on presentation, histology, direct immunofluorescence, indirect immunofluorescence (IIF) using monkey esophagus as substrate, salt-split skin (SSS) and an immunoblot assay.

Linear IgA bullous dermatosis in one of two piroxicam-induced eruptions: a distinct direct immunofluorescence trend revealed by the literature.

Background: The report focuses first on two patients with piroxicam-induced bullous eruption, one whose disease was diagnosed as linear IgA bullous dermatosis (LABD) and the other with no disease-specific immunologic findings using immunofluorescence methods. A review of the literature points to a distinctive direct immunofluorescence feature of drug-induced LABD cases.

Objective: Our purposes were to focus on divergent piroxicam reactions and to compare immunofluorescence findings in our and other reported drug-induced LABD cases to randomly occurring LABD cases.

Simultaneous presence of mucous membrane pemphigoid and pemphigus vulgaris: molecular characterization of both autoantibodies.

There are several reports in the literature describing the coexistence of features of pemphigus vulgaris and pemphigoid in the same patient. We describe 15 patients with clinical, histological, and immunopathological features of mucous membrane (cicatricial) pemphigoid at the time of initial diagnosis. All 15 patients failed to respond clinically to conventional systemic agents over a mean period of 7.

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.

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.

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).

Antinuclear antibodies (ANA) and complement fixing ANA in systemic connective tissue diseases.

Screening for antinuclear antibodies (ANA) with parallel tests for complement fixing ANA (C-ANA) reveal that C-ANA react either as strongly as or more strongly than ANA in most cases of systemic lupus erythematosus (SLE) and related disorders including CREST syndrome. But sera of drug induced LE and other ANA positive subjects have weak or no C-ANA. (P < 0.