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The Journal of Investigative Dermatology Jan 2021
Review
Topics: Celiac Disease; Child; Dermatitis Herpetiformis; Dermatitis, Atopic; Humans; Skin
PubMed: 32540248
DOI: 10.1016/j.jid.2020.05.091 -
Nutrients Jun 2018Celiac disease (CD) is an immune-mediated, gluten-induced enteropathy that affects predisposed individuals of all ages. Many patients with CD do not report... (Review)
Review
Celiac disease (CD) is an immune-mediated, gluten-induced enteropathy that affects predisposed individuals of all ages. Many patients with CD do not report gastrointestinal symptoms making it difficult to reach an early diagnosis. On the other hand, CD is related to a wide spectrum of extra-intestinal manifestations, with dermatitis herpetiformis (DH) being the best characterized. These associated conditions may be the clue to reaching the diagnosis of CD. Over the last few years, there have been multiple reports of the association between CD and several cutaneous manifestations that may improve with a gluten-free diet (GFD). The presence of some of these skin diseases, even in the absence of gastrointestinal symptoms, should give rise to an appropriate screening method for CD. The aim of this paper is to describe the different cutaneous manifestations that have been associated with CD and the possible mechanisms involved.
Topics: Alopecia Areata; Celiac Disease; Dermatitis Herpetiformis; Dermatitis, Atopic; Diet, Gluten-Free; Gastrointestinal Diseases; Glutens; Humans; Mucous Membrane; Psoriasis; Rosacea; Skin; Skin Diseases; Stomatitis, Aphthous; Urticaria; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 29933630
DOI: 10.3390/nu10070800 -
Best Practice & Research. Clinical... Jul 2015Skin manifestations during pregnancy are common and diversified. This review will focus on the most important entities to be recognized by obstetricians. These are, on... (Review)
Review
Skin manifestations during pregnancy are common and diversified. This review will focus on the most important entities to be recognized by obstetricians. These are, on the one hand, physiological changes, where unnecessary investigations should be avoided, and on the other, the specific dermatoses of pregnancy. These develop electively in pregnancy, and they are currently grouped into three disorders: polymorphic eruption of pregnancy, atopic eczema of pregnancy, and pemphigoid gestationis. Arguments for recognition of these are presented including detection of anti-BP180 antibodies. Follow-up and treatment depend on the precise diagnosis. Risks in fetal prognosis may occur in rare pemphigoid gestationis cases.
Topics: Dermatitis, Atopic; Female; Humans; Hyperpigmentation; Pemphigoid Gestationis; Pregnancy; Pregnancy Complications; Skin Diseases; Skin Diseases, Vascular; Skin Physiological Phenomena
PubMed: 25862358
DOI: 10.1016/j.bpobgyn.2015.03.005 -
Der Pathologe Jul 2020Blisters and erosions of skin and mucous membranes are key features of the clinically heterogeneous group of autoimmune bullous diseases (AIBDs). These can be divided... (Review)
Review
Blisters and erosions of skin and mucous membranes are key features of the clinically heterogeneous group of autoimmune bullous diseases (AIBDs). These can be divided into pemphigoid diseases with autoantibodies against structural proteins of the dermal-epidermal junction, pemphigus diseases with autoantibodies against desmosomal proteins, and dermatitis herpetiformis with autoantibodies against transglutaminases 1 and 2. A differentiation based only on clinical features is often not sufficient. After researching the literature in PubMed, the current diagnostic tools for AIBDs are summarized.AIBD diagnostics are performed using histology, direct and indirect immunofluorescence, as well as ELISA and immunoblotting. For serological diagnosis, the conventional multistep approach or multivariant assays for the analysis of autoantibodies against several target antigens in parallel can be applied. These allow a precise classification of AIBD and therefore a tailored use of different therapeutic regimens, e.g., for bullous pemphigoid or pemphigus foliaceus/vulgaris, as well as identification of disease entities with a known association with neoplasia.Direct immunofluorescence is still the diagnostic mainstay of AIBDs. However, novel serological assays, such as target-antigen-specific ELISA or indirect immunofluorescence systems using BIOCHIP™ mosaic technology, allow serologic diagnosis in most AIBD patients and the exact classification of the disease entity at the molecular level.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Pemphigoid, Bullous; Pemphigus; Skin Diseases, Vesiculobullous
PubMed: 32542511
DOI: 10.1007/s00292-020-00795-8 -
Scandinavian Journal of Gastroenterology Jul 2019Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease. Bone fracture risk is increased in coeliac disease, but little knowledge exists about... (Comparative Study)
Comparative Study
Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease. Bone fracture risk is increased in coeliac disease, but little knowledge exists about bone complications in DH. This study aimed to evaluate the risk of hip and other hospital-treated fractures in DH and coeliac disease in a high prevalence area with good adherence to a gluten-free diet. Hip, proximal humerus, wrist and ankle fractures in 368 treated DH and 1076 coeliac disease patients between 1970 and 2015 were reviewed from the National Hospital Discharge Register. Hip fracture incidence rates for DH and coeliac disease patients were compared to those for the general population. The overall fracture risk for DH was compared to coeliac disease. The hip fracture incidence rates for DH and coeliac disease patients did not differ from the general population. In females aged 80-89, the hip fracture incidence was higher in DH than in coeliac disease, but the risk for any hospital-treated fracture was lower in DH compared to coeliac disease (adjusted HR 0.620, 95% CI 0.429-0.949). The DH and coeliac disease patients with hospital-treated fractures were diagnosed at an older age, but the degree of small bowel mucosal damage did not significantly differ between patients with and without fractures. The incidence of hip fracture is not increased in treated DH or coeliac disease in an area with high awareness and dietary compliance rates. However, patients with DH seem to have a lower risk for fractures overall compared to coeliac disease.
Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Celiac Disease; Dermatitis Herpetiformis; Female; Finland; Hip Fractures; Humans; Incidence; Male; Middle Aged; Prevalence; Registries; Risk; Sex Factors; Young Adult
PubMed: 31280614
DOI: 10.1080/00365521.2019.1636132 -
Dermatology Practical & Conceptual May 2022Autoimmune bullous diseases (AIBDs) are a group of skin-related disorders that involve damage to structures maintaining cell-cell adhesion, such as desmosomes and... (Review)
Review
Autoimmune bullous diseases (AIBDs) are a group of skin-related disorders that involve damage to structures maintaining cell-cell adhesion, such as desmosomes and hemidesmosomes. Key AIBDs include pemphigus related diseases, pemphigoid related conditions, acquired epidermolysis bullosa (EBA), and dermatitis herpetiformis (DH). Each group of conditions exhibits characteristic clinical lesion patterns and is associated with specific autoantibodies targeting epidermal and dermal structures involved in cell-cell adhesion and skin integrity. Pemphigus diseases primarily target desmoglein (Dsg) 3 and Dsg1 proteins but several non-Dsg autoantibodies have also been linked to pemphigus. Pemphigoid diseases typically target bullous pemphigoid (BP)180 and BP230; EBA is associated with antibodies directed against anti-type VII collagen and DH by IgA autoantibodies against tissue transglutaminase and deaminated gliadin. Investigation into the serological biomarkers found in AIBDs have allowed the development of diagnostic assessments (i.e. tissue antibody detection and serological testing) based on the unique autoantibody profiles of a particular disease group. The methods for the detection and quantification of disease-associated autoantibodies continue to evolve and improve.
PubMed: 35646449
DOI: 10.5826/dpc.1202a116 -
Journal of Investigative Medicine High... 2021Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in the Western world affecting mainly the elderly population. The diagnosis is based on...
Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in the Western world affecting mainly the elderly population. The diagnosis is based on clinical assessment along with specific immunopathologic findings on skin biopsy. Risk factors include genetic factors, environmental exposures, and several infections including hepatitis B, hepatitis C, , , and cytomegalovirus. A variety of drugs have been associated with BP including but not limited to dipeptidyl peptidase-4 inhibitors, loop diuretics, spironolactone, and neuroleptics. Associated neurologic disorders (dementia, Parkinson's disease, bipolar disorder, previous stroke history, and multiple sclerosis) have also been described. Common clinical presentation consists of extremely pruritic inflammatory plaques that resemble eczematous dermatitis or urticaria, followed by formation of tense bullae with subsequent erosions. Typical distribution involves the trunk and extremities. Mucosa is typically spared affecting only 10% to 30% of patients. Several unusual clinical presentations of BP have been described such as nonbullous forms with erythematous excoriated papules, plaques, and nodules. Other reported findings include urticarial lesions, prurigo-like nodules, multiple small vesicles resembling dermatitis herpetiformis or pompholyx, vegetating and purulent lesions localized in intertriginous areas, and even exfoliative erythroderma. Recognition and management of such cases can present a diagnostic challenge to clinicians. In this article, we describe another variant which to our knowledge is the first case to present with a cellulitis-like presentation in a patient with a known history of BP.
Topics: Aged; Blister; Cellulitis; Humans; Pemphigoid, Bullous; Skin
PubMed: 33847152
DOI: 10.1177/23247096211008585 -
Scandinavian Journal of Gastroenterology 2023The current knowledge on the associations between coeliac disease and different skin diseases is contradictory and the patient's perspective on the burden of these is...
OBJECTIVES
The current knowledge on the associations between coeliac disease and different skin diseases is contradictory and the patient's perspective on the burden of these is lacking. This study aimed to investigate patient-reported frequency, severity and quality of life effects of skin disorders in coeliac disease patients compared to controls and moreover to study the impacts of gluten-free diet on these skin diseases.
MATERIALS AND METHODS
A study questionnaire designed for the purposes of this study and a validated Dermatology Life Quality Index (DLQI) questionnaire were posted to 600 adult members of the Finnish Coeliac Society and 1173 matched controls. Responses from 327 coeliac disease patients and 382 non-coeliac controls were compared.
RESULTS
Coeliac disease patients were shown to be at no increased risk of atopic dermatitis, acne, rosacea, psoriasis, alopecia areata, vitiligo or chronic urticaria. The severity of these skin diseases did not differ between study groups, but the risk for at least moderate effects on quality of life caused by dermatological diseases was increased among those with coeliac disease. Positive response from gluten-free diet was most commonly experienced by coeliac disease patients with atopic dermatitis.
CONCLUSIONS
Even though the risk for skin diseases was shown not to be increased among coeliac disease patients, there is still an increased burden related to experienced skin symptoms among these patients, which non-dermatologists treating coeliac disease patients should acknowledge.
Topics: Adult; Humans; Celiac Disease; Dermatitis Herpetiformis; Dermatitis, Atopic; Quality of Life; Patient Reported Outcome Measures
PubMed: 37477901
DOI: 10.1080/00365521.2023.2236263 -
The Journal of Investigative Dermatology Mar 2019Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are autoimmune bullous skin diseases. DH has been described to evolve into BP and the two diseases can have...
Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are autoimmune bullous skin diseases. DH has been described to evolve into BP and the two diseases can have overlapping clinical appearances and diagnostic findings, but the association between DH and BP has not previously been studied in a large population. To evaluate DH and celiac disease as risk factors for BP, we conducted a retrospective case-control study of patients with BP and matched controls with basal cell carcinoma diagnosed in Finland between 1997 and 2013. A total of 3,397 patients with BP and 12,941 controls were included in the study. Forty-one (1.2%) BP patients and 7 (0.1%) controls had preceding DH. Diagnosed DH increased the risk of BP 22-fold (odds ratio = 22.30; 95% confidence interval = 9.99-49.70) and celiac disease 2-fold (odds ratio = 2.54; 95% confidence interval = 1.64-3.92) compared to controls. Eighteen (43.9%) of the patients who had DH and subsequent BP had bought dapsone during the 2 years prior to their BP diagnosis. Mean time between diagnosed DH and BP was 3 years. We conclude that diagnosis of DH is associated with a striking increase in the risk for BP.
Topics: Adult; Aged; Aged, 80 and over; Case-Control Studies; Celiac Disease; Dapsone; Dermatitis Herpetiformis; Female; Finland; Humans; Leprostatic Agents; Male; Middle Aged; Pemphigoid, Bullous; Retrospective Studies; Risk
PubMed: 30612975
DOI: 10.1016/j.jid.2018.10.010 -
JAMA Dermatology Nov 2023Autoimmune bullous diseases (AIBDs) are chronic relapsing-remitting conditions with significant morbidity. Skin-related quality of life (SRQL) may vary by AIBD subtype...
IMPORTANCE
Autoimmune bullous diseases (AIBDs) are chronic relapsing-remitting conditions with significant morbidity. Skin-related quality of life (SRQL) may vary by AIBD subtype and disease type. Disease severity and flare severity can be difficult to define; SRQL can offer a key insight.
OBJECTIVES
To investigate the Skindex-16 score as an SRQL measure in AIBD subtypes during flare and nonflare states and to evaluate Skindex-16 construct validity.
DESIGN, SETTING, AND PARTICIPANTS
This retrospective cross-sectional study was conducted from September 1, 2016, to February 1, 2020, among 192 patients at the University of Utah Health autoimmune dermatology clinic with pemphigoid, pemphigus, dermatitis herpetiformis, and linear immunoglobulin A disease. Patients had an encounter-associated diagnosis, Skindex-16 scores, and self-reported flare status. Statistical analysis was performed from March 2022 to June 2023.
EXPOSURE
Autoimmune bullous disease subtype and patient-reported flare status.
MAIN OUTCOMES AND MEASURES
Skindex-16 domain scores (emotions, symptoms, and functioning; range, 0-100, where 0 indicates no effect on SRQL and 100 maximum effect) and individual item scores were described by disease and flare status. Flare scores were expected to be higher by at least the standard error of measurement (SEm). Convergent validity was assessed using Spearman correlation among Skindex-16 scores, serologic titers, and other patient-reported outcome measures. Floor or ceiling domain scores (<20% of sample scoring either lowest or highest possible domain scores, respectively) were assessed for Skindex-16. Structural validity was assessed using confirmatory factor analysis (CFA).
RESULTS
The study included 192 patients with 212 visits (median age, 68 years [IQR, 58-76 years]; 123 of 212 women [58.0%]) with Skindex-16 scores (64 in flare state and 148 in nonflare state). Median Skindex-16 domain scores were higher for all disease categories among patients in the flare state compared with those in the nonflare state (pemphigoid [emotions: flare, 52.4 (IQR, 38.1-69.0); nonflare, 7 (IQR, 0-17); symptoms: flare, 37.5 (IQR, 29.2-58.0); nonflare, 13 (IQR, 0-25); functioning: flare, 26.7 (IQR, 10.0-56.7); nonflare, 0 (IQR, 0-3)]; pemphigus [emotions: flare, 54.8 (IQR, 31.0-81.0; nonflare, 0 (IQR, 0-19); symptoms: flare, 58.3 (IQR, 41.7-70.8); nonflare, 4 (IQR, 0-12.5); functioning: flare, 26.7 (IQR, 13.3-83.3); nonflare, 0 (IQR, 0-3.33)]; dermatitis herpetiformis [emotions: flare, 72.6 (IQR, 34.7-90.5); nonflare, 14.3 (IQR, 2.4-26.2); symptoms: flare, 69 (IQR, 31.3-85.4); nonflare, 12.5 (IQR, 0-29.2); functioning: flare, 38.3 (IQR, 5.0-63.2); nonflare, 0 (IQR, 0-13.3)]. This difference exceeded SEm cut points. Cronbach α was greater than 0.80 for all domains and AIBDs. Moderate or low correlations were seen with desmoglein 1 and bullous pemphigoid 180 titers. Moderate correlation existed between Skindex-16 and Patient-Reported Outcomes Measurement Information System Depression scores (emotions: ρ = 0.40; symptoms: ρ = 0.41; functioning: ρ = 0.48), and strong correlation existed between Skindex-16 and patient-reported disease severity (emotions: ρ = 0.71; symptoms: ρ = 0.73; functioning: ρ = 0.66). Floor domain scores greater than 20% were seen among patients in the nonflare state, but ceiling domain scores were rare (<10% for all domains); CFA model fit was poor.
CONCLUSIONS AND RELEVANCE
In this cross-sectional study, SRQL was highly associated with flare of AIBDs. Skin-related quality of life was worse during periods without flare among patients with pemphigoid and dermatitis herpetiformis compared with pemphigus, highlighting residual SRQL morbidity. Skindex-16 showed good construct validity, but the poor CFA model fit needs further research. Clinical measurement of SRQL in AIBDs can add critical disease-severity information.
Topics: Humans; Female; Aged; Pemphigus; Quality of Life; Pemphigoid, Bullous; Retrospective Studies; Cross-Sectional Studies; Dermatitis Herpetiformis; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Disease Progression
PubMed: 37703003
DOI: 10.1001/jamadermatol.2023.3121