Future directions in managing aniridia-associated keratopathy

Congenital aniridia is a panocular disorder that is typically characterized by iris hypoplasia and aniridia-associated keratopathy (AAK). AAK results in the progressive loss of corneal transparency and thereby loss of vision. Currently, there is no approved therapy to delay or prevent its progression, and clinical management is challenging because of phenotypic variability and high risk of complications after interventions; however, new insights into the molecular pathogenesis of AAK may help improve its management. Here, we review the current understanding about the pathogenesis and management of AAK. We highlight the biological mechanisms involved in AAK development with the aim to develop future treatment options, including surgical, pharmacological, cell therapies, and gene therapies.     

Gastric Cancer and Paraneoplastic Pemphigus

Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Associations with gastric cancer have only been reported twice. A 78-year-old lady presented with a month’s history of extensive skin lesions that started off as bullous lesions and biopsy revealed bullous pemphigus. Endoscopy for anemia revealed gastric cancer. This case reinforced the need to consider underlying malignancy in elderly patient with new onset dermatological presentation.     

Association between serum 25-hydroxyvitamin D concentration and severity of first-diagnosed bullous pemphigoid in older adults

BackgroundHigher vitamin D status has been associated with symptom improvement and decreased risk of various autoimmune disorders. Our objective was to determine whether higher serum 25-hydroxyvitamin D (25OHD) concentration correlated with less severe first-diagnosed bullous pemphigoid (BP) in older inpatients.MethodsThis cross-sectional study was performed from November 2012 to February 2014 among 30 consecutive older inpatients (21 women; mean ± SD, 83 ± 7 years; all Caucasian) with a de novo diagnosis of active BP recruited in the Department of Dermatology of Angers University Hospital, France. The severity of BP was graded clinically on the basis of i) the number of bullae during the first three days of hospitalization (grade 0–4, worse), and ii) the extent of the lesions (grade 0–5, worse).ResultsSixteen participants had ≤ 5 bullae at the time of diagnosis, 8 had 6–20 bullae, 3 had 20–50 bullae, and 3 had >50 bullae. The lesions were spread over 5 cutaneous areas in 5 participants (17%). The median 25OHD concentration was 23 [IQR, 16–42] nmol/L. Serum 25OHD concentration was inversely correlated with the bullae grade (ρ = − 0.38, p = 0.04) and the lesion extension grade (ρ = − 0.50, p = 0.005).ConclusionsHigher serum 25OHD concentration correlated with less severe BP prior to initiation of treatment among our sample of older inpatients. This result suggests that vitamin D may be involved in the pathophysiology of BP and could serve as prognostic biomarker of BP.     

Erythrodermic bullous pemphigoid

We report a case of erythrodermic type of bullous pemphigoid which is a rare variant of bullous pemphigoid. Our patient had a peculiar clinical presentation with bullae, erosions and extensive erythrodermic areas, and distinct direct immunofluorescent findings which included linear IgG and C3 deposits in the basement membrane and also IgG in the intercellular spaces. Very high levels of serum IgE were also detected in our patient.     

Bullous pemphigoid in patients receiving peritoneal dialysis: a case series and a literature survey

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease. Although several cases of BP in end-stage renal disease patients receiving peritoneal dialysis (PD) or hemodialysis have been reported, the incidence of BP in these patients remains unknown. We recently experienced three PD patients diagnosed with BP. The skin injury was likely to be a trigger of BP in all the three PD patients. Nifedipine and icodextrin exposures were possible factors directly or indirectly affecting the onset of BP, because they were common in the three cases. We also report that the incidence of BP in PD patients was 3/478.3 person-years in a single-center 10-year study. This case series with a literature survey describes that the skin and tissue injuries are potential triggers responsible for the onset of BP in dialysis patients and that the incidence of BP in these patients seems to be much higher than that in the general population.     

Localized Bullous Pemphigoid Following Orthopedic Surgery

Bullous pemphigoid is an autoimmune condition whereby the immune system forms antibodies that target the skin, resulting in the formation of blisters in a generalized and symmetric pattern. Localized bullous pemphigoid can occur in special circumstances. Here, we describe two patients that developed localized bullous pemphigoid in one lower extremity following orthopedic surgery. We propose that underlying edema following orthopedic surgery is a potential trigger for localized bullous pemphigoid.     

生物工程角膜移植治疗感染性角膜炎

目的：观察生物工程角膜移植治疗感染性角膜炎的疗效,评价其临床应用价值和意义。

方法：对我院35例35眼需要进行手术治疗的感染性角膜炎患者分为3组：观察组15例,使用生物工程角膜施行板层角膜移植手术。对照组有两组：对照1组10例,使用保存人角膜施行板层角膜移植手术; 对照2组10例,实施自体结膜瓣遮盖手术。随访3～20mo,观察视力、感染控制情况以及角膜透明度。

结果：观察组15眼术后感染控制,视力不同程度提高,除1眼在随访中发生植片混浊外,其余14眼植片均维持基本透明。对照1组,除1眼病毒性角膜炎因病毒复发导致角膜混浊外,其余9眼角膜植片维持透明。对照2组,除1眼真菌性角膜溃疡感染加重,行眼内容物剜除术,其余角膜溃疡瘢痕修复。

结论：生物工程角膜是一种新型的人角膜替代材料,为解决逐年增长的角膜病患者与短缺的角膜供体之间的矛盾发挥了作用。     

共焦显微镜在角膜病变中应用的新进展

共焦显微镜(confocal microscopy, CM)检查作为一种“活体组织学检查”方法,以其实时、无创、快速、高清的特点,在角膜生理、病理研究及疾病诊断、病情评估、随访等方面显示出优越性,已在眼科临床及科研中得到广泛应用。本文将对CM在感染性角膜疾病、变性性角膜疾病及全身疾病所致角膜病变中应用的新进展作一综述。     

Disorders characterized by predominant or exclusive dermal inflammation

Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called “autoinflammatory” dermatitides comprising polymorphonuclear leukocytes. Eosinophil-dominated lesions include arthropod bite reactions, cutaneous parasitic infestations, the urticarial phase of bullous pemphigoid, Wells syndrome (eosinophilic cellulitis), hypereosinophilic syndrome, and Churg-Strauss disease. In other conditions, eosinophils are admixed with neutrophils in the corium, with or without small-vessel vasculitis. Exemplary disorders with those patterns include drug eruptions, chronic idiopathic urticaria, urticarial vasculitis, granuloma faciale, and Schnitzler syndrome (chronic urticarial with a monoclonal gammopathy).     

Queratopigmentación quirúrgica intraestromal con tinta para tatuaje cutáneo

Case reportA 31-year-old woman who had undergone surgery for a congenital cataract as a newborn developed bilateral malignant glaucoma, which was refractory to medical-surgical treatment. The patient currently has terminal glaucoma and severe band keratopathy. For aesthetic purposes, we performed intrastromal keratopigmentation surgery using ink for skin tattoos supplied in sterile single-dose vessels. No secondary effects or complications were observed.DiscussionKeratopigmentation or corneal tattooing can now be used as a last resort in patients who are unable to tolerate cosmetic contact or intraocular lenses, or in whom corneal transplant is contraindicated.