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Academic Pathology 2021http://journals.sagepub.com/doi/10.1177/2374289517715040..
http://journals.sagepub.com/doi/10.1177/2374289517715040..
PubMed: 33889719
DOI: 10.1177/23742895211006844 -
Frontiers in Immunology 2019Several lines of evidence indicate that the immune system, inflammation, and coagulation are simultaneously activated in autoimmune and immune-mediated skin diseases.... (Review)
Review
Several lines of evidence indicate that the immune system, inflammation, and coagulation are simultaneously activated in autoimmune and immune-mediated skin diseases. Pro-inflammatory cytokines such as interleukin-6 and tumor necrosis factor-alpha induce the expression of the main initiator of coagulation, i.e., tissue factor. The proteases of coagulation in turn act on protease-activated receptors inducing the expression of various pro-inflammatory cytokines triggering inflammation. The cross-talk among immune system, inflammation, and coagulation amplifies and maintains the activation of all three pathways. This review focuses on three skin disorders as chronic spontaneous urticaria (CSU), angioedema, and bullous pemphigoid (BP), in which the relationships among the three systems have been investigated or their clinical consequences are relevant. Markers of thrombin generation, fibrinolysis, and inflammation have been reported to be increased in the plasma during flares of CSU and angioedema, as well as in the active phase of BP, with the marker levels reverting to normal during remission. The coagulation activation seems to be important only at local level in CSU and angioedema while both at local and systemic levels in BP which is the only condition associated with an increased thrombotic risk. The prothrombotic state in autoimmune skin diseases raises the question of the indication of anticoagulant treatment, particularly in the presence of other cardiovascular risk factors.
Topics: Autoimmune Diseases; Autoimmunity; Fibrinolysis; Humans; Interleukin-6; Skin; Skin Diseases; Tumor Necrosis Factor-alpha
PubMed: 31281319
DOI: 10.3389/fimmu.2019.01407 -
Clinical, Cosmetic and Investigational... 2015Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and... (Review)
Review
Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and immunopathological findings. According to several evidences, DH is considered the specific cutaneous manifestation of celiac disease, and the most recent guidelines of celiac disease have stated that, in celiac patients with a proven DH, a duodenal biopsy is unnecessary for the diagnosis. In this review, the most recent data about the diagnosis and the management of DH have been reported and discussed. In particular, in patients with clinical and/or histopathological findings suggestive for DH, the finding of granular IgA deposits along the dermal-epidermal junction or at the papillary tips by direct immunofluorescence (DIF) assay, together with positive results for anti-tissue transglutaminase antibody testing, allows the diagnosis. Thereafter, a gluten-free diet should be started in association with drugs, such as dapsone, that are able to control the skin manifestations during the first phases of the diet. In conclusion, although DH is a rare autoimmune disease with specific immunopathological alterations at the skin level, its importance goes beyond the skin itself and may have a big impact on the general health status and the quality of life of the patients.
PubMed: 25999753
DOI: 10.2147/CCID.S69127 -
International Journal of Women's... Oct 2022Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in... (Review)
Review
Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III-V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.
PubMed: 35923586
DOI: 10.1097/JW9.0000000000000034 -
Gastroenterology Jun 2024Celiac disease (CeD) is a chronic immune-mediated condition triggered by gluten consumption in genetically predisposed individuals. Approximately 1% of the general... (Review)
Review
Celiac disease (CeD) is a chronic immune-mediated condition triggered by gluten consumption in genetically predisposed individuals. Approximately 1% of the general population is affected by the disorder. Disease presentation is heterogeneous and, despite growing awareness among physicians and the public, it continues to be underestimated. The most effective strategy for identifying undiagnosed CeD is proactive case finding through serologic testing in high-risk groups. We reviewed the most recent evidence on the association between CeD and more than 20 conditions. In light of this review, CeD screening is recommended in individuals with (1) autoimmune disease and accompanying symptoms suggestive of CeD; (2) diseases that may mimic CeD (eg, irritable bowel syndrome [IBS], inflammatory bowel disease [IBD], and microscopic colitis); and (3) among patients with conditions with a high CeD prevalence: first-degree relatives, idiopathic pancreatitis, unexplained liver enzyme abnormalities, autoimmune hepatitis, primary biliary cholangitis, hyposplenism or functional asplenia with severe bacterial infection, type 1 diabetes mellitus, Hashimoto's thyroiditis and Graves' disease, Sjögren's syndrome, dermatitis herpetiformis, recurrent aphthous syndrome and enamel defects, unexplained ataxia, peripheral neuropathy, delayed menarche or premature menopause, Down syndrome, Turner syndrome, Williams syndrome, chronic fatigue syndrome, IgA nephropathy, and IgA deficiency. CeD serology should be the initial step in the screening process. However, for patients with any of the aforementioned disorders who are undergoing upper endoscopy, biopsies should be performed to rule out CeD.
Topics: Humans; Celiac Disease; Autoimmune Diseases; Diagnosis, Differential; Inflammatory Bowel Diseases; Serologic Tests; Risk Factors; Prevalence; Genetic Predisposition to Disease
PubMed: 38460606
DOI: 10.1053/j.gastro.2024.02.044 -
Frontiers in Medicine 2018Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes.... (Review)
Review
Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.
PubMed: 30450358
DOI: 10.3389/fmed.2018.00296 -
Cureus Sep 2023Gluten sensitivity is defined as a chronic intolerance to gluten ingestion in genetically predisposed individuals. The etiology is thought to be immune-mediated and has... (Review)
Review
Gluten sensitivity is defined as a chronic intolerance to gluten ingestion in genetically predisposed individuals. The etiology is thought to be immune-mediated and has a variable dermatologic presentation. Celiac disease (CD) is one of the most common forms of gluten intolerance and encompasses a wide range of extra-intestinal pathology, including cutaneous, endocrine, nervous, and hematologic systems. Psoriasis, another long-term inflammatory skin condition, has been linked to significant symptomatic improvement with a gluten-free diet (GFD). Palmoplantar pustulosis (PP), a variant of psoriasis, and aphthous stomatitis, which causes recurrent oral ulcers, have also exhibited beneficial results after the dietary elimination of gluten. In addition to this, dermatitis herpetiformis (DH), another immune-mediated skin disorder, is genetically similar to CD and has, therefore, shown tremendous improvement with a GFD. Another highly prevalent long-term skin condition called atopic dermatitis (AD), however, has revealed inconsistent results with gluten elimination and would require further research in the future to yield concrete results. Hereditary angioedema (HA) has shown an association with gluten intolerance in some patients who had symptomatic benefits with a GFD. Similarly, vitiligo and linear IgA bullous dermatosis have also shown some clinical evidence of reversal with a GFD. On the contrary, rosacea enhances the risk of developing CD. This narrative review emphasizes the potential impact of gluten intolerance on different cutaneous conditions and the potential therapeutic effect of a GFD on various symptomatic manifestations. There is a need for additional clinical and observational trials to further expand on the underlying pathophysiology and provide conclusive and comprehensive recommendations for possible dietary interventions.
PubMed: 37790051
DOI: 10.7759/cureus.44549 -
ChemistryOpen Mar 2018Gluten-related disorders are a complex group of diseases that involve the activation of the immune system triggered by the ingestion of gluten. Among these, celiac... (Review)
Review
Gluten-related disorders are a complex group of diseases that involve the activation of the immune system triggered by the ingestion of gluten. Among these, celiac disease, with a prevalence of 1 %, is the most investigated, but recently, a new pathology, named nonceliac gluten sensitivity, was reported with a general prevalence of 7 %. Finally, there other less-prevalent gluten-related diseases such as wheat allergy, gluten ataxia, and dermatitis herpetiformis (with an overall prevalence of less than 0.1 %). As mentioned, the common molecular trigger is gluten, a complex mixture of storage proteins present in wheat, barley, and a variety of oats that are not fully degraded by humans. The most-studied protein related to disease is gliadin, present in wheat, which possesses in its sequence many pathological fragments. Despite a lot of effort to treat these disorders, the only effective method is a long-life gluten-free diet. This Review summarizes the actual knowledge of gluten-related disorders from a translational chemistry point of view. We discuss what is currently known from the literature about the interaction of gluten with the gut and the critical host responses it evokes and, finally, connect them to our current and novel molecular understanding of the supramolecular organization of gliadin and the 33-mer gliadin peptide fragment under physiological conditions.
PubMed: 29531885
DOI: 10.1002/open.201700197 -
Dermatologie (Heidelberg, Germany) Dec 2022Wheat sensitivity is a collective term for several, especially gastrointestinal, diseases that occur as part of a hypersensitivity reaction after wheat consumption.... (Review)
Review
BACKGROUND
Wheat sensitivity is a collective term for several, especially gastrointestinal, diseases that occur as part of a hypersensitivity reaction after wheat consumption. The symptoms, which are mostly similar to those of irritable bowel syndrome, are often accompanied by skin lesions. In addition to celiac disease and dermatitis herpetiformis, the cutaneous manifestation of celiac disease, wheat sensitivity also includes nonceliac gluten sensitivity (NCGS), allergic nickel contact mucositis, wheat allergy, amylase-trypsin inhibitor intolerance, and fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAP) intolerance.
OBJECTIVES
This review article aims to provide an overview of the clinical, especially dermatological and gastrointestinal features of the different forms of wheat sensitivity. Diagnosis and therapeutic management are also discussed.
MATERIALS AND METHODS
A selective literature search was carried out with evaluation of our own clinical data.
RESULTS
The skin lesions in dermatitis herpetiformis are very disease-specific. In contrast, wheat allergy often shares signs and symptoms with many other diseases. Other forms of wheat sensitivity cause primarily gastrointestinal abnormalities, but extra-intestinal manifestations can also occur. Their diagnosis is often complex and requires cross-disciplinary collaboration with experts in gastroenterology. The therapy consists of a wheat- or gluten-free diet.
CONCLUSIONS
Knowledge of the different and frequently occurring dermatological signs of wheat sensitivity is of great importance, because dermatological manifestations associated with gastrointestinal pathology, intolerance reactions, and allergies appear more and more frequently.
Topics: Humans; Celiac Disease; Glutens; Wheat Hypersensitivity; Dermatitis Herpetiformis; Diet, Gluten-Free; Amylases
PubMed: 37882829
DOI: 10.1007/s00105-023-05243-1