A case of subepidermal blistering disease with autoantibodies to multiple laminin subunits who developed later autoantibodies to alpha-5 chain of type IV collagen associated with membranous glomerulonephropathy.

We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332.

Comprehensive citation factor: a novel method in ranking medical journals.

The Institute for Scientific Information (ISI) defines substantive articles as source items, which include only research and review (R&R) articles. This policy encourages some Journals to publish a significant number of original reports in the category of letters to the editor and reviews in the category of Editorials. Consequently, the Impact Factor (IF) fails to provide a fair comparison between medical journals.

Is paromomycin an effective and safe treatment against cutaneous leishmaniasis? A meta-analysis of 14 randomized controlled trials.

Background: High cost, poor compliance, and systemic toxicity have limited the use of pentavalent antimony compounds (SbV), the treatment of choice for cutaneous leishmaniasis (CL). Paromomycin (PR) has been developed as an alternative to SbV, but existing data are conflicting.

Methodology/principal Findings: We searched PubMed, Scopus, and Cochrane Central Register of Controlled Trials, without language restriction, through August 2007, to identify randomized controlled trials that compared the efficacy or safety between PR and placebo or SbV.

Compositional differences between infant and adult human corneal basement membranes.

Purpose: Adult human corneal epithelial basement membrane (EBM) and Descemet's membrane (DM) components exhibit heterogeneous distribution. The purpose of the study was to identify changes of these components during postnatal corneal development.

Methods: Thirty healthy adult corneas and 10 corneas from 12-day- to 3-year-old children were studied by immunofluorescence with antibodies against BM components.

Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype.

Background: Autoantibodies in linear immunoglobulin A (IgA) disease (LAD) are reported to be of IgA class and directed against a 97-120 kDa epidermal antigen.

Methods: We report a 39-year-old woman with clinical features of LAD and with circulating IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180).

Results: Histopathology of lesional skin revealed a subepidermal blister with mixed inflammatory cell infiltrate.

Usefulness of immunoblotting using purified laminin 5 in the diagnosis of anti-laminin 5 cicatricial pemphigoid.

Background: Anti-laminin 5 cicatricial pemphigoid (CP) is a mucosal-dominant subepithelial blistering disease characterized by IgG anti-basement membrane zone autoantibodies, that bind to dermal side of 1 M NaCl split skin and immunoprecipitate laminin 5. Laminin 5 is an epidermis-specific extracellular matrix consisting of alpha3, beta3 and gamma2 subunits. Recent studies have suggested that autoantibodies of anti-laminin 5 CP recognize the G domains of alpha3 subunit.

Crescentic glomerulonephritis and subepidermal blisters with autoantibodies to alpha5 and alpha6 chains of type IV collagen.

We describe a novel autoimmune disease characterized by severe subepidermal bullous eruption and crescentic glomerulonephritis with autoantibodies directed against the noncollagenous domain of the alpha5 and alpha6 chains of type IV collagen. Biopsy of perilesional skin revealed a subepidermal blister with marked polymorphonuclear infiltrate with linear deposits of IgA and C3. Light microscopy of a kidney biopsy specimen revealed a crescentic glomerulonephritis, and immunofluorescence microscopy showed linear basement membrane staining for IgA (3+), C3 (1+), and IgG (1+).

Desmoglein-3 is a target autoantigen in spontaneous canine pemphigus vulgaris.

Pemphigus vulgaris (PV) is an autoimmune blistering skin disease of humans and companion animals. In human patients, PV is associated with the production of IgG autoantibodies specific for keratinocyte desmosomal glycoproteins of the cadherin family. The purpose of this study was to determine whether antikeratinocyte IgG autoantibodies were present in the skin and serum of dogs with PV, and also to identify the canine PV autoantigen(s) targeted by circulating autoantibodies.

Laminin-5 is targeted by autoantibodies in feline mucous membrane (cicatricial) pemphigoid.

In human and canine patients with mucous membrane (cicatricial) pemphigoid (MMP), circulating autoantibodies have been shown to target multiple epidermal basement membrane antigenic epitopes. These autoantigens include collagen XVII in humans and dogs, as well as laminin-5, laminin-6 or integrin alpha-6/beta-4 in human beings. The purpose of this study was to determine if autoantibodies targeted laminin-5 in a cat exhibiting clinical and microscopic lesions resembling those of MMP in humans.

The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators.

Objective: We aimed to develop consensus-based recommendations for streamlining medical communication among various health care professionals, to improve accuracy of diagnosis and treatment, and to facilitate future investigations for mucous membrane pemphigoid.

Participants: Because of the highly specific nature of this group of diseases, the 26 invited participants included either international scholars in the field of mucous membrane pemphigoid or experts in cutaneous pharmacology representing the 3 medical disciplines ophthalmology, oral medicine, and dermatology.

Evidence: The first author (L.

Anti-plakin and desmoglein autoantibodies in a dog with pemphigus vulgaris.

Paraneoplastic pemphigus was suspected in a 14-year-old Labrador retriever because of mucocutaneous erosions, microscopic suprabasal acantholysis, and keratinocyte apoptosis. In this patient, circulating IgG autoantibodies recognized plakin (envoplakin, periplakin) and desmoglein (desmoglein-1 and -3) antigens. Necropsy, however, failed to confirm the concurrent existence of hematopoietic or solid neoplasia.

Desmosomes and their autoimmune pathologies.

Desmosomes guarantee the integrity of the epidermis, by functioning both as an adhesive complex and as a cell-surface attachment site for the keratin intermediate filaments of the cytoskeleton. Considerable progress has been made in our knowledge of desmosomes and their components. The structure and function of many of the desmosomal molecules have been determined, and a number of the molecular interactions between desmosomal proteins have been elucidated.

The alpha 5 chain of type IV collagen is the target of IgG autoantibodies in a novel autoimmune disease with subepidermal blisters and renal insufficiency.

We describe a novel autoimmune disease characterized by severe subepidermal bullous eruptions and renal insufficiency with IgG autoantibodies directed against the NC1 domain of the alpha5(IV) collagen chain. In vivo deposits of IgG and C3 were found along the dermal-epidermal junction of skin lesions. The identity of the target antigen was determined by immunochemical analyses of candidate antigens using the patients' autoantibodies.

Lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen).

We describe a 75-year-old patient with pruritic papules on her trunk and extremities, typical of lichen planus, who later experienced subepidermal blisters. These clinical features are consistent with lichen planus pemphigoides. Immunofluorescence of perilesional skin showed linear deposits of C3 along the dermoepidermal junction.

Linear IgA disease associated with lymphocytic colitis.

A 66-year-old woman presented with a bullous skin eruption and chronic diarrhoea. Lesional skin showed subepidermal blistering, and direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the dermoepidermal junction, establishing a diagnosis of linear IgA disease (LAD). Chronic watery diarrhoea complicated by substantial loss of body weight preceded the skin eruption for several months.

[Epidermolysis bullosa acquisita and hepatitis C].

Introduction: Epidermolysis bullosa acquisita is a bullous dermatosis. Its etiology remains unknown and the efficacy of its treatment is low.

Observation: We report the first association between epidermolysis bullosa acquisita, chronic hepatitis C and cryoglobulinemia, healing with interferon alpha and ribavirine.

IgE antibodies in sera from patients with bullous pemphigoid are autoantibodies preferentially directed against the 230-kDa epidermal antigen (BP230).

Bullous pemphigoid (BP) is unique among autoimmune skin diseases in which a high serum IgE level has been detected. We sought to determine the antigenic specificity of these IgE antibodies in 39 BP sera by immunofluorescence microscopy, immunoblot, and ELISA. The patient's sera contained IgG antibodies to 230-kDa (BP230) (n = 20), 180-kDa (BP180) (n = 9), and both BP230 and BP180 (n = 10) antigens.