blistering skin disorders
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Background: Autoimmune skin disorders (ASDs) are complex diseases triggered by autoantibodies action against epidermal antigens or the dermo epidermal junction. Although rare, they present high morbidity, affecting the quality of life (QoL) of patients and financial status of patient.Methods: This prospective, observational study was carried out in department of dermatology for 2-3 months after ethical approval. Drug usage pattern, heath related QoL (HRQOL) by using DLQI (Dermatology life quality index) and cost were evaluated in patients with ASDs. Statistical analysis was done using Microsoft excel office 2019 and rechecked with SPSS (version 23.0). P<0.001 was considered as statistically significant.Results: Out of 73 patients enrolled, 32 were male and 41 were female with the mean age was 48.27±14.93 years; 55% patients had autoimmune blistering skin disorders (AIBDs) and 45% having other ASDs (OADs). Pemphigus vulgaris (PV) (35%) being the most common among all ASDs. Systemic steroid (60.27%), topical steroid (79.45%), levocetirizine (63%) were most commonly prescribed drugs. Mean DLQI score at baseline and after treatment was 11.64±2.49 and 6.8±2.75 respectively. It was highly significant statistically (p<0.0001). Total cost of illness per month was 813.64±481.21 INR. Maximum percentage variation in cost was seen with prednisolone (1706.28%).Conclusions: ASDs have a female bias and inflict severe impairment to the QoL of patients. Appropriate drug therapy with corticosteroids and other adjuvant drug lead to positive impact on QoL. There was very wide price variation of different brands of the same generic most commonly prednisolone and levocetirizine.

Staphylococcal scalded skin syndrome (SSSS) describes a spectrum of superficial blistering skin disorders caused by the exfoliative toxins of Staphylococcus aureus that originates from a focus of infection that may be a purulent conjunctivitis, otitis media, or occult nasopharyngeal infection. It usually begins with fever, irritability, and a generalized, paint, orange-red, macular erythema with cutaneous tenderness, and the rash progress from scarlatiniform to a blistering eruption in 24 to 48 hours. A diagnosis must distinguish SSSS from other skin diseases, such as toxic epidermal necrolysis, epidermolysis bullosa, bullous erythema multiforme, Streptococcal impetigo or listeriosis and thermal or chemical burns, all of which can manifest with similar symptoms. The prognosis of SSSS in children who are appropriately treated is good, with a mortality of less than 5%. A case was a three moths old boy hospitalized in Pediatric ward M. Djamil hospital with chief complain redness and peeling of the skin since 2 days before hospitalized. Culture of the skin, eyes and nose was Staphylococcus aureus, and patients was given ampicillin and gentamycin for seven days.

Staphylococcal scalded skin syndrome (SSSS) describes a spectrum of superficial blistering skin disorders caused by the exfoliative toxins of Staphylococcus aureus that originates from a focus of infection that may be a purulent conjunctivitis, otitis media, or occult nasopharyngeal infection. It usually begins with fever, irritability, and a generalized, paint, orange-red, macular erythema with cutaneous tenderness, and the rash progress from scarlatiniform to a blistering eruption in 24 to 48 hours. A diagnosis must distinguish SSSS from other skin diseases, such as toxic epidermal necrolysis, epidermolysis bullosa, bullous erythema multiforme, Streptococcal impetigo or listeriosis and thermal or chemical burns, all of which can manifest with similar symptoms. The prognosis of SSSS in children who are appropriately treated is good, with a mortality of less than 5%. A case was a three moths old boy hospitalized in Pediatric ward M. Djamil hospital with chief complain redness and peeling of the skin since 2 days before hospitalized. Culture of the skin, eyes and nose was Staphylococcus aureus, and patients was given ampicillin and gentamycin for seven days.

<abstract> <p>Autoimmune blistering skin disorders are rare. According to direct immunofluorescence studies, three categories are described: pemphigus group, pemphigoid group and dermatitis herpetiformis. Among these diseases, bullous pemphigoid is the most common. Patients with typical bullous pemphigoid disease are usually elderly and have many comorbidities. Considering that topical and systemic corticosteroids are the first choice therapy, these patients also have increased morbidity and risk of death. The main characteristic of bullous pemphigoid as an acquired autoimmune blistering disease is the formation of autoantibodies against hemidesmosomal antigens BP180 and BP230. Although IgG autoantibodies predominate within the plasma and skin of BP patients, some features of the disease cannot be explained solely by IgG-mediated mechanisms. Epitope spreading phenomena, immunoglobulin class switch and the relevance of IgM and IgE autoantibodies are discussed in this article.</p> </abstract>

Keratin intermediate filaments constitute the primary cytoskeletal component of epithelial cells. Numerous human disease phenotypes related to keratin mutation remain mechanistically elusive. Our recent crystal structures of the helix 1B heterotetramer from keratin 1/10 enabled further investigation of the effect of pathologic 1B domain mutations on keratin structure. We used our highest resolution keratin 1B structure as a template for homology-modeling the 1B heterotetramers of keratin 5/14 (associated with blistering skin disorders), keratin 8/18 (associated with liver disease), and keratin 74/28 (associated with hair disorder). Each structure was examined for the molecular alterations caused by incorporating pathogenic 1B keratin mutations. Structural modeling indicated keratin 1B mutations can harm the heterodimer interface (R265PK5, L311RK5, R211PK14, I150VK18), the tetramer interface (F231LK1, F274SK74), or higher-order interactions needed for mature filament formation (S233LK1, L311RK5, Q169EK8, H128LK18). The biochemical changes included altered hydrophobic and electrostatic interactions, and altered surface charge, hydrophobicity or contour. Together, these findings advance the genotype-structurotype-phenotype correlation for keratin-based human diseases.

Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses on mice that lack the desmosomal cadherins desmoglein 3 or desmocollin 3 in stratified epithelia. A comparison of the phenotypes observed in these mouse lines is provided and the relationship between the mutant mouse phenotypes and human diseases, in particular pemphigus vulgaris, is discussed. Furthermore, we will discuss the advantages and potential limitations of genetically engineered mouse lines in our ongoing quest to understand blistering skin diseases.