Toxic epidermal necrolysis (TEN) is an acute life-threatening blistering disease characterized by involvement of the skin, multiple mucous membranes and internal organs. It is most commonly precipitated by the administration of medications like anticonvulsants. Neuroleptic malignant syndrome (NMS) is a rare complication of neuroleptic therapy characterized by catatonic behavior, generalized mus...

For several decades, intravenous Ig has been used as treatment for a variety of immune-related diseases, including immune thrombocytopenic purpura (ITP), autoimmune neuropathies, systemic lupus erythematosus, myasthenia gravis, Guillain-Barré syndrome, skin blistering syndromes, and Kawasaki disease. Despite years of use, its mechanism of immunomodulation is still unclear. Recent studies using ...

The clinical and immunopathologic features of children with acquired subepidermal blistering disorders show considerable overlap, and their classification frequently requires characterization of the targeted antigens. A 8-month-old boy developed a generalized subepidermal blistering disorder with striking palmoplantar involvement. The patient's serum contained antibodies reacting against the ep...

Bullous skin lesions are uncommon in children. While it is well known that Mycoplasma infections are associated with papular skin manifestations, bullous skin lesions are not commonly reported. Mycoplasma pneumoniae is a very common bacterial pathogen causing respiratory tract infection in children and adults. We report 2 children with serology-confirmed Mycoplasma infection who were hospitaliz...

Desmocollin 3 (DSC3) belongs to a subfamily of cadherins and is a major component of desmosomes in keratinocytes of stratified epithelia, such as the epidermis. Based on its amino acid sequence homology to classical cadherins, such as E-cadherin, it has been postulated that DSC3 functions as a cell-adhesion molecule. To test this hypothesis, we assessed the function of DSC3 in the development a...

Background: Autoimmune disorders are conditions in which autoantibodies are directed against a single organ or tissue resulting in localized tissue damage. Pemphigus includes a group of autoimmune blistering diseases of skin and mucous membranes characterized by intra dermal blisters and immunologically by finding of circulating immunoglobulin G antibody directed against the cell surface of ker...

Bullous pemphigoid is the most common autoimmune blistering disease in industrialized countries and particularly affects elderly. In this patient population, comorbid diseases are frequent may complicate management treatment of bullous pemphigoid. A better understanding why distinct more patients lead to new pathophysiological insights - as a consequence result care. The association with neurol...

Coronavirus disease 2019 (COVID-19) has been declared as an emergent pandemic. COVID-19 mainly present with fever and respiratory symptoms. Its severity ranges from asymptomatic infection, mild, moderate, severe to critical degrees which is related the degree of dysregulated inflammatory response systemic inflammation. Stevens-Johnson syndrome (SJS), on other hand a rare, severe, type IV hypers...

A group of autoimmune diseases is characterised by autoantibodies against epithelial adhesion structures and/or tissue-tropic lymphocytes driving inflammatory processes resulting in specific pathology at the mucosal surfaces and the skin. The most frequent site of mucosal involvement in autoimmune diseases is the oral cavity. Broadly, these diseases include conditions affecting the cell-cell ad...

BACKGROUND Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD; OMIM #226670) is an autosomal recessive disease, characterized mainly by skin blistering at birth or shortly thereafter, progressive muscle weakness, and rarely by alopecia. EBS-MD is caused by mutations in the PLEC gene (OMIM *601282), which encodes plectin, a structural protein expressed in several tissues, including ep...