A population-based study of the association between bullous pemphigoid and neurologic disorders.

BACKGROUND: Bullous pemphigoid (BP) has been associated with neurologic disorders. OBJECTIVE: We sought to analyze the association between BP and neurologic disorders. METHODS: We retrospectively identified residents of Olmsted County, Minnesota, with a first lifetime diagnosis of BP between January 1, 1960, and December 31, 2009. Three age- and sex-matched Olmsted County, Minnesota, residents without BP were selected as control subjects 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: In all, 87 patients with BP were identified and matched to 261 control subjects. The odds of a previous diagnosis of any neurologic disorder or a history of dementia were significantly increased among cases compared with controls (odds ratio 6.85; 95% confidence interval [CI] 3.00-15.64; P < .001; and odds ratio 6.75; 95% CI 2.08-21.92; P = .002, respectively). Both Parkinson disease (hazard ratio 8.56; 95% CI 1.55-47.25; P = .01) and any type of neurologic disorder (hazard ratio 2.02; 95% CI 1.17-3.49; P = .01) were significantly more likely to develop during follow-up in patients with than without BP. LIMITATIONS: Small geographic area and retrospective study design are limitations. CONCLUSION: Findings confirmed an association of BP with neurologic disorders, especially dementia and Parkinson disease.

Incidence of bullous pemphigoid and mortality of patients with bullous pemphigoid in Olmsted County, Minnesota, 1960 through 2009.

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with increased mortality. OBJECTIVE: We sought to determine the incidence and mortality of patients with BP. METHODS: A total of 87 residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 through December 2009. Incidence and mortality were compared with 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% confidence interval, 1.9-2.9). Incidence of BP increased significantly with age (P < .001) and over time (P = .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 versus localized disease (P = .90). LIMITATIONS: Retrospective study design and study population from a small geographic area are limitations. CONCLUSION: Incidence of BP in the United States is comparable with that found in Europe and Asia. The mortality of BP is lower in the United States than Europe, but higher than previous estimates.

A partially regressed, atrophic plaque on a 17-year-old girl: an unusual presentation of myofibromatosis.

Solitary cutaneous myofibromas have been reported in patients of all ages. Infantile and adult forms are distinguished by age of onset, location of lesions, and the potential for infantile myofibromas to regress. We report a case of a 17-year-old girl with a solitary myofibroma on the right leg that presented as a partially regressed, atrophic plaque with multiple surrounding papules. Unusual features of this case include the tumor size, simultaneous regression and proliferation, patient age, and gender.

Moxifloxacin-induced drug hypersensitivity syndrome with features of toxic epidermal necrolysis.

Moxifloxacin was recently reported to induce combined features of drug hypersensitivity syndrome (DHS) and toxic epidermal necrolysis (TEN). A simultaneous presentation of these two potentially life-threatening cutaneous drug eruptions with systemic features in the same patient is considered rare since they are probably induced by two separate mechanisms. There is only one previously reported case in which moxifloxacin was implicated in the induction of these combined drug hypersensitivity processes. This article presents the case of a 44-year-old Asian male who developed features of both TEN and DHS approximately one week after initial ingestion of moxifloxacin.

Parathyroid hormone hormone-related protein and the PTH receptor regulate angiogenesis of the skin.

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.