Purpose To report the clinical and genetic findings of a male toddler who presented bilateral bullous retinoschisis with a novel RS1 mutation. Observations This is an observational case report of a patient referred to our hospital with esotropia. A comprehensive ophthalmic examination was performed with the boy (age, 1 year 4 months) under general anesthesia that included fundus examinations,...

Pyoderma gangrenosum (PG) is a chronic neutrophilic disorder characterized by recurrent painful ulcers. There are several clinical subtypes in PG: (classical) ulcerative, pustular, bullous, vegetative, and peristomal. Aseptic inflammation neutrophils plays an essential role and, neutrophil extracellular traps (NETs) formation can contribute to the lesion formation. We examined relationship betw...

A 9-year-old boy presented with itching and bulla formation since the last 3 years. The lesions commenced over the abdomen, scalp and then became generalized. Past perinatal and drug history were uneventful. On examination, tense bullae (0.5 cm to 3.5 cm in diameter) were present all over the body (Fig. 1). Bullae arranged in an annular pattern were seen in perineal region (string of pearl’s si...

PURPOSE To introduce autologous tragal perichondrium transplantation as a novel surgical modality for the management of intractable symptomatic bullous keratopathy. METHODS In three eyes of three patients with painful bullous keratopathy, autologous tragal perichondria were transplanted on the corneal surface with the human amniotic membrane transplanted above. We included an additional three...

Animal models of human diseases are very useful to better understand their pathomechanism and to develop new therapies. However, due to the differences among different species, it is sometimes difficult to reproduce reliable diseases phenotype in animals. Bullous pemphigoid is the most common autoimmune blistering skin disease, in which circulating IgG autoantibodies directed against type XVII ...

BACKGROUND Linear IgA bullous dermatosis is an autoimmune blistering disease characterized clinically by the presence of small tense blisters and immunologically by the presence of IgA at the dermal-epidermal junction. Idiopathic, systemic disease-related, and drug-related versions of this disorder have been described, with the latter most commonly associated with vancomycin. OBSERVATIONS We ...

B ullous pemphigoid is an autoimmune disease, which means that the cells in the body that normally fight infection attack the body instead. The body’s immune system is confused and makes an antibody (type of protein used to fight infection) that targets a part of the skin that normally holds it together. The attack on the skin causes blisters (firm, fluid-filled bubbles on the skin) to form. Th...

BACKGROUND Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent topical corticosteroids could decrease mortality while controlling disease. METHODS A total of 341 patients with bullous pemphigoid were enrolled in a randomized, multicente...

Background: Here we present a case of 56 year old female who presented with mild shortness of breath and right side pain. Chest tomography indicated bilateral bullous pulmonary lesions as the causal of the patient’s discomfort and pain. The patient had familial history of cancer and pneumothorax, and had undergone surgery on the left lung due to bullous lesion and pneumothorax. The patient has ...

OBJECTIVE To investigate the safety and efficacy of oral methylprednisolone combined with azathioprine sodium or mycophenolate mofetil for the treatment of bullous pemphigoid. DESIGN A prospective, multicenter, randomized, nonblinded clinical trial to compare 2 parallel groups of patients with bullous pemphigoid undergoing different treatments. SETTING Thirteen departments of dermatology in...