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Dsg, Desmoglein; GFP, Green fluorescent protein; TRAIL, tumor necrosis factor -related apoptosis-inducing ligand; HNSCC, head and neck squamous cell carcinoma; scFvs, single chain variable fragment antibodies; HA, hemaggultinin
Journal of Investigative Dermatology]]>
Aims: To evaluate the patients with pemphigus vulgaris on treatment in respect of osteoporosis and to compare the frequency of osteoporosis in these patients with the healthy ones.
Methods: The study consisted of 40 patients with pemphigus vulgaris and 34 healthy controls. Bone mineral density measurements were obtained by dual- energy X-ray absorptiometry. Blood serum, bone parameters, and biochemical hormonal measurements were examined in both groups.
Results: When the bone mineral density values of patients with pemphigus vulgaris were compared with those of the control group, there was no significant difference between hip bone mineral density values, while lumbar region T and Z scores were found significantly low in the patient group (p = 0.034 and p = 0.006, respectively). Osteoporosis, osteopenia, and normal dual-energy X-ray absorptiometry rates in the patient group were found to be 32.5%, 32.5%, and 35%, respectively. These rates were found to be 18%, 23%, and 59% in control group, respectively. There were more fractures in the patient group and the difference was statistically significant (p = 0.004).
Conclusion: An increase in osteoporosis frequency and secondary fracture to osteoporosis in the patients with pemphigus vulgaris was detected.
Full acticle can be viewed at: Indian Journal of Dermatology]]>
Adjuvant therapeutic methods are employed when pemphigus vulgaris (PV) fails to be controlled by conventional corticosteroid treatment. Objective: The efficacy of double filtration plasmapheresis (DFPP) was investigated in a PV patient with severe, refractory mucosal disease.
A total of 3 DFPP cycles, each cycle consisting of 5 double filtration sessions conducted on alternate days was completed.
DFPP provided immediate clinical relief of symptoms as well as a significant decrease in anti-desmoglein antibody levels and allowed for a much lower corticosteroid dose.
DFPP was an effective and safe adjuvant therapy in our patient with PV and it offers a valid treatment option in PV patients with recalcitrant disease.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis]]>
OBJECTIVE To determine the prevalence and clinical significance of anti-Lam332 autoantibody detection from a large series of patients with MMP. DESIGN Multicenter retrospective study.
SETTING Four French national centers for autoimmune bullous diseases.
PARTICIPANTS One hundred fifty-four patients with MMP and 89 individuals serving as controls were included.
INTERVENTIONS Serum samples were analyzed by a new Lam332 enzyme-linked immunosorbent assay (ELISA); clinical and immunopathologic data were obtained from the patients’ medical records.
MAIN OUTCOME MEASURES The Lam332 ELISA scores were evaluated with respect to clinical characteristics, standard and salt-split indirect immunofluorescence, and bullous pemphigoid (BP) 230 and BP180-NC16A ELISAs.
RESULTS The Lam332 ELISA score was positive (≥9 U/mL) in 20.1% of serum samples from patients with MMP, 1 of 50 patients with bullous pemphigoid (BP), none of 7 with pemphigus, and 3 of 32 other controls. No relationship was evidenced between a positive ELISA Lam332 score and age; sex ratio; oral, ocular, genital, skin, or esophageal/laryngeal involvement; internal malignant neoplasm; or BP180 ELISA score. Salt-split skin indirect immunofluorescence and ELISA BP230 results were more frequently positive when Lam332 ELISA results were positive (P = .04 and .02, respectively). Patients with a positive Lam332 ELISA score frequently had more severe MMP (67.8% vs 47.2%; P = .04).
CONCLUSIONS AND RELEVANCE Results of this novel ELISA showed that serum anti-Lam332 autoantibodies are detected in 20.1% of patients with MMP. Anti-Lam332 autoantibodies are mainly detected in patients with severe MMP but not preferentially in those with a malignant neoplasm. The association between anti-Lam332 and anti-BP230 autoantibodies might arise from an epitope-spreading phenomenon.
JAMA dermatology (Chicago, Ill.)]]>
European journal of dermatology : EJD]]>
To evaluate the therapeutic effects of topical tacrolimus ointment on refractory inflammatory ocular surface diseases.
Retrospective interventional consecutive case series.
In Severance Hospital, Seoul, South Korea, 0.02% tacrolimus ointment was topically applied 1 to 3 times per day, depending on disease severity, for up to 31 months in eyes of 12 consecutive patients with refractory inflammatory ocular surface diseases who had previously been treated with steroid therapy. Seven patients had chronic cicatrizing conjunctivitis (6 cases caused by Stevens-Johnson syndrome and 1 attributable to ocular cicatricial pemphigoid); 4 had scleritis (3 necrotizing scleritis, 1 recurrent nodular scleritis); and 1 patient had Mooren ulcer with corneal perforation. The therapeutic outcomes after tacrolimus treatment were evaluated according to the following criteria: change in clinical findings (eg, decrease of hyperemia, ocular pain, epithelial defect, and pseudomembrane), intraocular pressure (IOP), and need for steroid therapy.
In all 3 groups, tacrolimus showed an immunosuppressive effect, especially on scleritis and Mooren ulcer. These effects included suppression of corneoscleral melting and reduction of hyperemia. In chronic cicatrizing conjunctivitis, simultaneous topical tacrolimus while tapering steroid therapy suppressed inflammatory relapse. The elevated IOP in steroid responders recovered to normal range after successful tapering of steroid. No adverse side effects were noted after 1.5 to 31 months of continuous tacrolimus treatment.
The use of topical tacrolimus ointment is effective in controlling refractory inflammatory ocular surface disease, and can reduce the need for steroid use while reducing inflammation recurrence.
Full article available here: http://www.ajo.com/article/S0002-9394(12)00863-X/abstract]]>
BACKGROUND: We recently reported that the autoantibodies of anti-p200 pemphigoid sera react with laminin γ1 and renamed this entity as anti-laminin γ1 pemphigoid. However, it has not been clarified whether the anti-laminin γ1 autoantibodies, particularly those to the C-terminal integrin binding site, affect the dermoepidermal junction and cause subepidermal blisters.
OBJECTIVE: The aim of this study was to develop animal models for anti-laminin γ1 pemphigoid.
METHODS: We attempted to produce two mouse models for anti-laminin γ1 pemphigoid; (1) a passive transfer model: injection of rabbit IgG to shorter bacterial recombinant protein of the murine laminin γ1 C-terminal 107 amino acids, and (2) an active disease model: direct immunization to mice with this recombinant protein.
RESULTS: Immunoblotting revealed that 70% of patient sera reacted with the shorter recombinant protein of human laminin γ1 C-terminus. In the passive transfer model, rabbit IgG to the murine laminin γ1 C-terminus was deposited, without C3 deposition, at the epidermal basement membrane zone. In contrast, in the active disease model, direct immunofluorescence of mouse skin sections showed no deposition of either murine IgG or C3. Blister formation was not seen in either model both phenotypically and histopathologically.
CONCLUSION: In the two different mouse animal models for anti-laminin γ1 pemphigoid, although rabbit IgG to the recombinant laminin γ1 C-terminus bound to the epidermal basement membrane zone in passive transfer model, no obvious blister formation was seen. To reproduce skin lesions in mouse models for anti-laminin γ1 pemphigoid, further improvement should be needed.
Full article can be purchased here: http://www.sciencedirect.com/science/article/pii/S0929664612005104]]>