How Do Experts Treat Patients with Bullous Pemphigoid around the World? An International Survey.

Many treatments are currently proposed for treating patients with bullous pemphigoid (BP). We assessed treatment modalities of BP depending on the different countries, BP extent, and patients' comorbidities. We surveyed worldwide experts about how they treat patients with BP. A total of 61 experts from 27 countries completed the survey. Severe and moderate BP were treated with oral prednisone (61.4 and 53.7%, respectively) or superpotent topical corticosteroids (CSs) (38.6 and 46.3%, respectively). Conventional immunosuppressants were more frequently combined with oral prednisone (74.5%) than with superpotent topical CS (37.5%) in severe BP. Topical CSs were mainly used in Europe in mild (81.1%), moderate (55.3%), and severe (54.3%) BP. In the United States of America and Asia, systemic CSs were mainly proposed for treating severe (77.8 and 100%, respectively), moderate (70 and 77.8%, respectively), and also mild (47.1 and 33.3%, respectively) BP. Most experts reduced the initial dose of oral CS in patients with diabetes mellitus (48.1%) or cardiac insufficiency (40.2%) but rarely changed BP treatment in patients with neurological disorders or neoplasia. This survey showed major differences in the way patients with BP are treated between AmeriPac countries (United State of America, Latin America, and Australia) and Asia on the one hand and Europe and the Middle East on the other hand.

Linear IgA disease: clinical presentation, diagnosis, and pathogenesis.

Linear IgA disease is one of the rarer subepidermal blistering diseases. Linear IgA disease is a chronic, acquired, autoimmune blistering disease that is characterized by subepidermal blistering and linear deposition of IgA basement membrane antibodies. The disease affects both children and adults and, although there are some differences in their clinical presentations, there is considerable overlap with shared immunopathology and immunogenetics.

The association of bullous pemphigoid with cerebrovascular disease and dementia: a case-control study.

OBJECTIVE: To investigate the relationship between bullous pemphigoid (BP) and neurologic disease. DESIGN: Case-control study. SETTING: Tertiary care center for immunobullous diseases and skin tumor clinics at a university hospital in Oxford, England. PARTICIPANTS: Ninety consecutive patients with BP and 141 controls. MAIN OUTCOME MEASURES: Age-adjusted prevalence of neurologic disease in patients and controls. Time interval between the diagnosis of neurologic disease and BP and type of associated neurologic disease. RESULTS: At least 1 neurologic diagnosis was present in 42 patients (46%) compared with 16 controls (11%). Patients had significantly increased odds for neurologic diseases regardless of age and sex (crude odds ratio [OR], 6.8; 95% confidence interval [CI], 3.5-13.3; adjusted OR, 6.2; 95% CI, 3.1-12.4). Four major neurologic diagnoses were observed (cerebrovascular disease, dementia, Parkinson disease, and epilepsy), with statistical significance for cerebrovascular disease and dementia (crude OR for cerebrovascular disease, 6.3; 95% CI, 2.8-14.2; adjusted OR, 6.0; 95% CI, 2.6-13.6; crude OR for dementia, 10.7; 95% CI, 2.3-49.0; adjusted OR, 7.9; 95% CI, 1.7-37.3). When accurate data on time of onset of neurologic disease were present (36 of 42 patients [85%]), BP followed neurologic disease in most patients (26 of 36 patients [72%]), with a median interval of 5.5 years. CONCLUSION: Bullous pemphigoid is significantly associated with cerebrovascular disease and dementia.

Treatment of nevoid hyperkeratosis of the nipple and areola by shave excision.

The case is presented of a 29-year-old female who, at the age of 13 years, developed bilateral verrucous thickening of her areolae. Despite the condition causing her significant psychosocial morbidity, a specialist referral was initially denied on the grounds that no treatment was apparently available. The condition progressively deteriorated over the subsequent 14 years. She was eventually referred for a dermatology opinion, and the diagnosis of nevoid hyperkeratosis was made. Topical therapy with keratolytics was unsuccessful, and she was referred for a plastic surgery review. Bilateral shave excision of the lesion was performed under general anesthesia, with a satisfactory outcome and no evidence of recurrence at 10 months.

Pachydermodactyly: a forme fruste of knuckle pads?

A 16-year-old boy presented with painless swellings localized to the radial and ulnar aspects of his second through to the fifth fingers on the left hand, with more subtle changes affecting two fingers on the opposite hand. This had developed in the absence of mechanical trauma. Investigations for an arthropathy were negative, while a biopsy showed marked epidermal hyperplasia and an expanded dermis. These features are typical of pachydermodactyly, a benign dermatosis of uncertain aetiology. The interesting feature of this case is the presence of knuckle pads in the father of the patient, which raises the possibility that these two similar entities are related.