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JAAD Case Reports Aug 2021
PubMed: 34337119
DOI: 10.1016/j.jdcr.2021.03.054 -
Clinical Case Reports Jul 2021Herpetiformis dermatitis is the best characterized extraintestinal manifestation of celiac disease (CD). However, other chronic heterogeneous skin lesions have been...
Herpetiformis dermatitis is the best characterized extraintestinal manifestation of celiac disease (CD). However, other chronic heterogeneous skin lesions have been associated with CD and should be considered in the differential diagnosis.
PubMed: 34306684
DOI: 10.1002/ccr3.4441 -
PloS One 2021Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to...
OBJECTIVES
Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan.
METHODS
This was a retrospective cross-sectional study conducted at Khartoum Dermatological and Venereal Diseases Teaching Hospital. We used routinely collected health care data, and included all patients with an autoimmune bullous disease who presented to the hospital between 2001 and 2016.
RESULTS
Out of the 4736 patients who were admitted to the hospital during the study period, 923 (19.5%) had an autoimmune bullous disease. The average rate of patients at the hospital was 57.7 per year representing 1.3 per 100,000 population per year. After exclusion of patients where the final diagnosis was missing, 585 were included in the further analysis. Pemphigus vulgaris was the most common disease (50.9%), followed by bullous pemphigoid (28.2%), linear IgA disease/chronic bullous disease of childhood (8.4%), and pemphigus foliaceous (8.2%). Pemphigoid gestationis and IgA pemphigus constituted 1.4% and 1.2% of the cohort, respectively. Paraneoplastic pemphigus, mucous membrane pemphigoid, lichen planus pemphigoidis, bullous systemic lupus erythematosus, and dermatitis herpetiformis were rare. None of the patients had epidermolysis bullosa acquisita.
CONCLUSIONS
The clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoimmune Diseases; Child; Child, Preschool; Cross-Sectional Studies; Epidermolysis Bullosa Acquisita; Female; Humans; Male; Middle Aged; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Retrospective Studies; Sudan; Young Adult
PubMed: 34255799
DOI: 10.1371/journal.pone.0254634 -
Cureus May 2021Introduction A vesiculobullous lesion of the skin encompasses a group of dermatological disorders with protean clinicopathological features. They usually occur as a part...
Introduction A vesiculobullous lesion of the skin encompasses a group of dermatological disorders with protean clinicopathological features. They usually occur as a part of the spectrum of various infectious, inflammatory, drug-induced, genetic, and autoimmune disorders. Therefore, accurate diagnosis of these lesions is essential for appropriate management and to reduce the associated morbidity and mortality. The conventional skin punch biopsy is the mainstay in the diagnosis of dermatological diseases, especially when combined with confirmatory tests, such as direct immunofluorescence (DIF). Our study evaluated the clinicopathological spectrum of vesiculobullous lesions. Methods We studied 150 cases of vesiculobullous lesions at the Department of Histopathology, Liaquat National Hospital and Medical College Karachi, Pakistan. Written and informed consent was taken from the patients followed by skin punch procedure in which three biopsies were obtained, which included one biopsy from the lesion and two peri-lesional biopsies. One peri-lesional biopsy was sent in cryomatrix for DIF studies, whereas the other two were sent in formalin to follow the standard tissue-processing protocol. Results Our results showed that most patients belonged to the geriatric age group of more than 50 years (44.7%), and 54.7% of the patients were females. Total 74.7% of the patients had generalized lesions, followed by lower limbs (9.3%) and trunk (7.3%) involvement. Most patients were diagnosed with bullous pemphigoid (31.3%), followed by pemphigus vulgaris (27.3%), dermatitis herpetiformis (15.3%), Darier's disease (14.7%), pemphigus foliaceus (4.7%), epidermolysis bullosa (2%), linear immunoglobulin A dermatosis (2%), paraneoplastic pemphigus (0.7%), and drug reactions (0.7%). DIF studies were applied on 60 cases, out of which complement protein C3c was the most commonly deposited protein (53.3%). Conclusion Our study emphasized the diagnostic role of skin punch biopsy in the proper evaluation of vesiculobullous skin lesions. Histopathology is the cornerstone diagnostic tool in this regard, with DIF being a useful adjunct.
PubMed: 34235011
DOI: 10.7759/cureus.15330 -
Indian Journal of Dermatology,...
Topics: Dermatitis Herpetiformis; Female; Humans; Immunoglobulin A; Male; Middle Aged; Staining and Labeling; Young Adult
PubMed: 34114424
DOI: 10.25259/IJDVL_287_20 -
Clinical Medicine (London, England) May 2021Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of...
Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of immunobullous disorders with characteristic morphology that relates to the structural properties of the target protein. In this article we will describe the pathogenesis, clinical features and treatment of the most common immunobullous disorders.
Topics: Epidermolysis Bullosa Acquisita; Humans; Pemphigoid, Bullous; Skin; Skin Diseases, Vesiculobullous
PubMed: 34001564
DOI: 10.7861/clinmed.2021-0232 -
Dermatology Online Journal Apr 2021
Topics: Autoantigens; Dermatitis Herpetiformis; Enzyme-Linked Immunosorbent Assay; Epidermis; Humans; Immunoglobulin A; Transglutaminases
PubMed: 33999587
DOI: No ID Found -
Frontiers in Immunology 2021The reason why only few coeliac patients develop the cutaneous manifestation of the disease, named dermatitis herpetiformis (DH), is still unknown. Epidermal... (Clinical Trial)
Clinical Trial
The reason why only few coeliac patients develop the cutaneous manifestation of the disease, named dermatitis herpetiformis (DH), is still unknown. Epidermal transglutaminase (TG3) has been described as the main autoantigen of humoral immunity in DH but the mechanisms leading to this autoimmune response remain obscure. Here we characterized T cells from skin, gut and peripheral blood of DH and coeliac disease (CD) patients, evaluated the impact of the gluten-free diet on circulating T lymphocytes' phenotype and investigated antigen specific T cell response toward epidermal and tissue transglutaminase (TG2). DH patients showed an increased frequency of skin-derived T cells producing TNFα when compared to CD patients. Moreover, circulating T cells producing TNFα and IL-17A positively correlated with clinical score of skin disease activity and decreased after gluten-free diet. Finally, TG2 and TG3-specific T cells resulted more reactive to antigens stimulation in DH patients and showed cross reactivity toward the two autoantigens in both the group of patients. Our data suggest a role of TNFα and IL-17A producing cells in the development of DH and, for the first time, show the existence of a crossed T cell response toward the two transglutaminases isoforms, thus suggesting new insights on T cells role in skin damage.
Topics: Adolescent; Adult; Aged; Celiac Disease; Child; Dermatitis Herpetiformis; Female; GTP-Binding Proteins; Humans; Interleukin-17; Male; Middle Aged; Protein Glutamine gamma Glutamyltransferase 2; T-Lymphocytes; Transglutaminases; Tumor Necrosis Factor-alpha
PubMed: 33959126
DOI: 10.3389/fimmu.2021.645143 -
JAAD Case Reports May 2021
PubMed: 33948472
DOI: 10.1016/j.jdcr.2021.03.035