Background Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with an increased mortality rate. Objective To determine the incidence and mortality rate of patients with bullous pemphigoid. Methods Eighty-seven residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 – December 2009. Incidence and mortality rate were compared to age- and sex-matched control patients from the same geographic area. Results The adjusted incidence of BP was 2.4 per 100,000 person-years (95% CI, 1.9–2.9). Incidence of BP increased significantly with age (P<.001) and over time (P=0.034). Trend tests indicate increased diagnosis of localized disease (P=.006) may be a contributing factor. Survival observed in the incident BP cohort was significantly poorer than expected (P<.001). Survival was not different among patients with multisite vs localized disease (P=.90). Limitations Retrospective study design and study population from a small geographic area. Conclusion Incidence of BP in the United States is comparable to that found in Europe and Asia. The mortality rate of BP is lower in the United States than Europe, but higher than previous estimates.

Background Bullous pemphigoid has been reported in association with neurologic disorders. Objective To analyze the association between bullous pemphigoid and neurologic disorders. Methods We retrospectively identified residents of Olmsted County, Minnesota, with a first lifetime diagnosis of bullous pemphigoid between January 1, 1960, and December 31, 2009. Three age- and sex-matched Olmsted County residents without bullous pemphigoid were selected as controls for each patient. We compared history of or development of neurologic disorders (dementia, Alzheimer disease, Parkinson disease, multiple sclerosis, cerebrovascular disease, and seizures) between groups using case-control and cohort designs. Results A total of 87 patients with bullous pemphigoid were identified and matched to 261 controls. The odds of a previous diagnosis of any neurologic disorder or a history of dementia were significantly increased among cases compared with controls (odds ratios: 6.85 (3.00–15.64); P<.001, and 6.75 (2.08–21.92); P=.002, respectively). Both Parkinson disease (hazard ratio, 8.56 (1.55–47.25); P=.01) and any type of neurologic disorder (hazard ratio, 2.02 (1.17–3.49); P=.01) were significantly more likely to develop during follow-up in patients with bullous pemphigoid than in those without bullous pemphigoid. Limitations Small geographic area; retrospective study design. Conclusion Our study confirmed an association of bullous pemphigoid with neurologic disorders, especially dementia and Parkinson disease.

In developing organs, parathyroid hormone (PTH)/parathyroid hormone-related protein (PTHrP) receptor (PPR) signaling inhibits proliferation and differentiation of mesenchyme-derived cell types resulting in control of morphogenic events. Previous studies using PPR agonists and antagonists as well as transgenic overexpression of the PPR ligand PTHrP have suggested that this ligand receptor combination might regulate the anagen to catagen transition of the hair cycle. To further understand the precise role of PTHrP and the PPR in the hair cycle, we have evaluated hair growth in the traditional K14-PTHrP (KrP) and an inducible bitransgenic PTHrP mice. High levels of PTHrP trangene expression limited to the adult hair cycle resulted in the production of shorter hair shafts. Morphometric analysis indicated that reduced proliferation in the matrix preceded the appearance of thinner hair follicles and shafts during late anagen. CD31 staining revealed that the late anagen hair follicles of the KrP mice were surrounded by reduced numbers of smaller diameter capillaries as compared to controls. Moreover, the fetal skins of the PTHrP and PPR knockouts (KOs) had reciprocal increases in the length, diameter, and density of capillaries. Finally, crossing the KrP transgene onto a thrombospondin-1 KO background reversed the vascular changes as well as the delayed catagen exhibited by these mice. Taken together, these findings suggest that PTHrP's influence on the hair cycle is mediated in part by its effects on angiogenesis.

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Gonadotropin-releasing hormone (GnRH) is a neuropeptide which regulates hormone release from pituitary gonadotropes. The recently cloned pituitary GnRH receptor consists of a linear chain of amino acids in which seven transmembrane segments separate 4 extracellular and 3 intracellular (cytoplasmic) sequences. The rat receptor is approximately 90% homologous with that of the human.Observations made in several autoimmune diseases and experimental models suggest that anti-receptor antibodies can interfere with receptor function. The present studies were designed to test this hypothesis in female rats (n=9×3) after immunization with synthetic peptides whose overlapping sequences replicated all putative extracellular domains of the cloned human GnRH receptor (Table 1). The peptides (A-H) were conjugated to keyhole limpet hemocyanin and the conjugates were injected i.m. at monthly intervals for four months. A control group (K) received adjuvant only. Anti-peptide antibodies in the sera of immunized rats were detected by ELISA. Recognition of the “native” GnRH receptor was tested in immunocytochemistry on normal pituitary and brain tissue.