Vulvar lichen planus pemphigoides.

Lichen planus pemphigoides (LPP) is a rare blistering disease with features of both lichen planus and bullous pemphigoid. LPP typically appears on the extremities and occasionally involves the oral mucosa. Herein, we describe a case of LPP of the vulva of an 80-year-old woman, an uncommon location for this disease process. This clinical scenario can be confused with a number of similarly appearing entities such as erosive vulvar lichen planus, mucous membrane pemphigoid, and erosive lichen sclerosus et atrophicus. In fact, our patient carried a diagnosis of lichen sclerosus by an outside physician for 2 years prior to being properly diagnosed and treated. A detailed discussion of the epidemiology, clinical, and pathogenesis as well as the histologic and immunofluorescence characteristics of this uncommon diagnosis is presented. Our case emphasizes the necessity of microscopic analysis to differentiate lookalike disease states when making a diagnosis and choosing the correct therapeutics.

Review article: coeliac disease, new approaches to therapy.

BACKGROUND: Coeliac disease is managed by life-long gluten withdrawal from the diet. However, strict adherence to a gluten-free diet is difficult and is not always effective. Novel therapeutic approaches are needed to supplement or even replace the dietary treatment. AIM: To review recent advances in new therapeutic options for coeliac disease. METHODS: A literature search was performed on MEDLINE, EMBASE, Web of Science, Scopus, DDW.org and ClinicalTrials.gov for English articles and abstracts. The search terms used included, but not limited to, 'Celiac disease', 'new', 'novel', 'Advances', 'alternatives' and 'Drug therapy'. The cited articles were selected based on the relevancy to the review objective. RESULTS: Several new therapeutic approaches for coeliac disease are currently under development by targeting its underlying pathogenesis. Alternative therapies range from reproduction of harmless wheat strains to immunomodulatory approaches. Some of these therapies such as enzymatic cleavage of gluten and permeability inhibitors have shown promise in clinical studies. CONCLUSIONS: Gluten-free diet is still the only practical treatment for patients with coeliac disease. Novel strategies provide promise of alternative adjunctive approaches to diet restriction alone for patients with this disorder.

Spontaneous lupus-like syndrome in HLA-DQ2 transgenic mice with a mixed genetic background.

To investigate the role of HLA-DQ2 in the pathogenesis of associated immune disorders, we generated transgenic mice that expressed HLA-DQ2 in the absence of endogenous murine class II molecules (AE(0)DQ2). These AE(0)DQ2 mice with a mixed genetic background spontaneously developed skin lesions on their ears, whereas control AE(0)DQ6 genotype control mice (also with a mixed genetic background) did not. The skin lesions were characterized by deep subepidermal blistering with hydropic degeneration and lymphoid infiltration in the subepidermal area as determined by histopathology. Immunofluorescence analysis revealed thick band-like granular deposition of IgG, IgM, and a thin band of IgA deposition along the basement membrane. AE(0)DQ2 mice also developed significant and progressive hematuria and proteinuria as compared with the AE(0)DQ6 mice (p < 0.05). Histopathology showed immune complex deposits in the glomeruli of AE(0)DQ2 mice. Immunofluorescence analysis showed progressive mesangial and capillary wall deposition of IgA, IgM, IgG and C1q in the kidney. With electron microscopy, the deposits showed a 'fingerprint' substructure; and tubuloreticular structures were identified within endothelial cells. Conversely, these changes were not observed in AE(0)DQ6 mice. Serum anti-double stranded (ds)DNA IgM and IgG levels were also significantly elevated among AE(0)DQ2 mice compared with AE(0)DQ6 mice (p < 0.001). In conclusion, AE(0)DQ2 mice spontaneously develop an autoimmune lupus-like syndrome and are useful model for this disease. It remains to be determined whether genetic admixture played a role in the development of this systemic lupus erythematosus-like syndrome in HLA-DQ2 transgenic mice. Lupus (2010) 19, 815-829.

Combination testing for antibodies in the diagnosis of coeliac disease: comparison of multiplex immunoassay and ELISA methods.

BACKGROUND: Tissue transglutaminase (TTG) antibodies and newly developed deamidated gliadin peptide (DGP) antibodies have better accuracy than native gliadin antibodies. Multiplex immunoassay (MIA) measures multiple antibodies simultaneously providing a complete antibody phenotype with reduced turnaround time and cost. AIM: To evaluate the agreement between MIA and enzyme-linked immunosorbent assay (ELISA) test results for coeliac autibodies in biopsy-proven coeliac patients and controls and to model the diagnostic utility of combination testing. METHODS: We compared the sensitivity, specificity and accuracy of MIA and ELISA methods for TTG and DGP antibodies in mainly adult untreated coeliac patients (n = 92) and controls (n = 124). RESULTS: There was excellent agreement and a significant correlation between the results of MIA and ELISA methods (k > 0.8, r > 0.7) for all tests, except IgG. Diagnostic indices of individual and combination tests measured by the MIA method did not differ significantly from those measured by ELISA. The combination tests slightly increased sensitivity (if any test was positive) and specificity (if all tests were positive) compared to the individual tests. CONCLUSIONS: Multiplex immunoassay testing for antibodies is as accurate as ELISA for coeliac disease diagnosis and has practical advantages over ELISA method. Rational combination testing can help identify patients who need intestinal biopsy and may reduce unnecessary biopsies.