Publications by authors named "Hazem A Juratli"

13 Publications

  • Page 1 of 1

Autoreactive Peripheral Blood T Helper Cell Responses in Bullous Pemphigoid and Elderly Patients With Pruritic Disorders.

Front Immunol 2021 25;12:569287. Epub 2021 Mar 25.

Department of Dermatology and Allergology, Philipps-Universität Marburg, Marburg, Germany.

Bullous pemphigoid (BP) is a prototypic autoimmune disorder of the elderly, characterized by serum IgG autoantibodies, namely anti-BP180 and anti-BP230, directed against components of the basal membrane zone that lead to sub-epidermal loss of adhesion. Pruritus may be indicative of a pre-clinical stage of BP, since a subset of these patients shows serum IgG autoantibodies against BP230 and/or BP180 while chronic pruritus is increasingly common in the elderly population and is associated with a variety of dermatoses. Clinical and experimental evidence further suggests that pruritus of the elderly may be linked to autoimmunity with loss of self-tolerance against cutaneous autoantigens. Thus, the objective of this study was to determine autoreactive T cell responses against BP180 in elderly patients in comparison to patients with BP. A total of 22 elderly patients with pruritic disorders, 34 patients with bullous or non-bullous BP and 34 age-matched healthy controls were included in this study. The level of anti-BP180 and anti-BP230 IgG serum autoantibodies, Bullous Pemphigoid Disease Area Index (BPDAI), and pruritus severity were assessed for all patients and controls. For characterization of the autoreactive T cell response, peripheral blood mononuclear cells were stimulated with recombinant BP180 proteins (NH- and COOH-terminal domains) and the frequencies of BP180-specific T cells producing interferon-γ, interleukin (IL)-5 or IL-17 were subsequently determined by ELISpot assay. Patients with BP showed a mixed Th1/Th2 response against BP180 while autoreactive Th1 cells were identified in a minor subset of elderly patients with pruritic disorders. Furthermore, our T cell characterization revealed that therapeutic application of topical clobetasol propionate ointment in BP patients significantly reduced peripheral blood BP180-specific T cells, along with clinically improved symptoms, strongly suggesting a systemic immunosuppressive effect of this treatment.
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http://dx.doi.org/10.3389/fimmu.2021.569287DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8027500PMC
March 2021

Lepidopterismus: Erfahrungen mit dem Eichenprozessionsspinner in Marburg.

J Dtsch Dermatol Ges 2021 Jul;19 Suppl 1:44-46

Klinik für Dermatologie und Allergologie, Universitätsklinikum Marburg UKGM, Marburg.

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http://dx.doi.org/10.1111/ddg.14483DOI Listing
July 2021

Three novel pathogenic NEK9 variants in patients with nevus comedonicus: a case series.

J Am Acad Dermatol 2021 Apr 2. Epub 2021 Apr 2.

Institute of Human Genetics, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2021.03.096DOI Listing
April 2021

Porokeratosis Plantaris, Palmaris et Disseminata Caused by Con- genital Pathogenic Variants in the MVD Gene and Loss of Hetero-zygosity in Affected Skin.

Acta Derm Venereol 2021 Feb 16;101(2):adv00397. Epub 2021 Feb 16.

Institute of Human Genetics, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, DE-79106 Freiburg, Germany.

Porokeratoses are a heterogeneous group of keratinization disorders. For linear porokeratosis and disseminated superficial actinic porokeratosis, a heterozygous pathogenic germline variant in a mevalonate pathway gene and a postzygotic second hit mutation present in affected skin have been shown to be the patho-genetic mechanism for the development of the lesions. However, the molecular mechanism leading to development of porokeratosis plantaris, palmaris et disseminata is not known. This study analysed a cohort of 4 patients with linear porokeratosis and 3 patients with porokeratosis plantaris, palmaris et disseminata, and performed mutation analyses of DNA extracted from blood samples and skin biopsies. All of the study patients carried the heterozygous germline variant c.70+5G>A in the MVD gene. Loss of heterozygosity due to a second hit mutation was found in affected skin of 3 patients with linear porokeratosis and 2 patients with porokeratosis plantaris, palmaris et disseminata. These results suggest that porokeratosis plantaris, palmaris et disseminata shares the same pathogenetic mechanism as other porokeratosis subtypes and belongs to the phenotypic spectrum of MVD-associated porokeratosis.
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http://dx.doi.org/10.2340/00015555-3753DOI Listing
February 2021

Amyopathic and anti-TIF1 gamma-positive dermatomyositis: analysis of a monocentric cohort and proposal to update diagnostic criteria.

Eur J Dermatol 2020 Jun;30(3):279-288

Department of Dermatology and Allergology.

Dermatomyositis (DM) is a group of autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. An amyopathic form of DM (ADM) has been described, and more recently, an anti-TIF-1 gamma-positive subtype, characterized by poikiloderma and associated with a relatively high risk of cancer. To characterise a cohort of DM patients. A cohort of 29 DM patients was followed between January 2004 and March 2019, and investigated for clinical characteristics, pathological features based on electromyography and MRI, laboratory data, and auto-antibody profile. Based on the investigations, DM was shown to be heterogeneous. However, we identified a subgroup of anti-TIF-1 gamma-positive patients who all shared heliotrope erythema. Furthermore, we observed a positive correlation between serum glutamicoxaloacetic transaminase (GOT) and creatine kinase (CK) concentrations in patients with anti-TIF-1 gamma antibodies, which is not found in patients with anti-MDA-5 antibodies. Based on the findings of this study, we propose an update of the Sontheimer et al. diagnostic criteria to improve the sensitivity of diagnosis for ADM. In addition, we describe a significant association between serum GOT and CK levels in DM patients with anti-TIF-1 gamma antibodies, and further highlight the significance of heliotrope rash as a clinical hallmark for this particular subset of patients.
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http://dx.doi.org/10.1684/ejd.2020.3766DOI Listing
June 2020

The polymorphous spectrum of dermatomyositis: classic features, newly described skin lesions, and rare variants.

Eur J Dermatol 2020 Jun;30(3):229-242

Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.

Dermatomyositis belongs to a group of rare autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. The clinically diagnostic hallmarks of dermatomyositis are heliotrope rash, Gottron's papules and weakness of the proximal muscles. Along with pathognomonic, characteristic, and compatible cutaneous features, several uncommon and rare skin manifestations have been reported. In addition, new skin lesions have been described in dermatomyositis patients. Furthermore, rare clinical subtypes of dermatomyositis have been reported in the literature, including Wong-type dermatomyositis, characterised by the coexistence of dermatomyositis and pityriasis rubra pilaris with hyperkeratotic, erythematous, follicular confluent papules on the back of the hands along the bony prominences. In addition, plenty of autoantibody subsets have been recently described that are related to distinct clinical features and systemic involvement, such as anti-MDA5 autoantibodies. We reviewed the English- and German-language scientific literature using the key words "dermatomyositis", "autoantibodies", and "clinical features", alone or in combination, focusing on particular cutaneous symptoms and their association with defined autoantibody profiles. Furthermore, we focused on rare subtypes of dermatomyositis, unusual clinical features, and recently described skin lesions.
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http://dx.doi.org/10.1684/ejd.2020.3761DOI Listing
June 2020

Eine Reisedermatose mit Spätfolgen: marine Kontaktdermatitis durch Korallen mit verzögerter lichenoider Reaktion.

J Dtsch Dermatol Ges 2020 May;18(5):495-497

Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg, Philipps Universität Marburg, Marburg.

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http://dx.doi.org/10.1111/ddg.14086_gDOI Listing
May 2020

Travel-associated dermatosis with late sequelae: Coral contact dermatitis presenting with a lichenoid reaction.

J Dtsch Dermatol Ges 2020 May 20;18(5):493-495. Epub 2020 Apr 20.

Department of Dermatology and Allergology, University Medical Center of Giessen and Marburg, Philipps University, Marburg, Germany.

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http://dx.doi.org/10.1111/ddg.14086DOI Listing
May 2020

A Family with Palmar and Plantar Hyperkeratosis: A Quiz.

Acta Derm Venereol 2020 Feb 27;100(4):adv00064. Epub 2020 Feb 27.

Department of Dermatology and Allergology, Philipp University, DE-35043 Marburg, Germany.

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http://dx.doi.org/10.2340/00015555-3419DOI Listing
February 2020

Sarkoidose mit begleitender Hepatitis-C-Infektion erfolgreich mit Adalimumab behandelt.

J Dtsch Dermatol Ges 2019 Sep;17(9):936-939

Klinik für Dermatologie und Allergologie, Universitätsklinikum Marburg, Marburg, Deutschland.

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http://dx.doi.org/10.1111/ddg.13884_gDOI Listing
September 2019

Sarcoidosis with concomitant hepatitis C infection treated successfully with adalimumab.

J Dtsch Dermatol Ges 2019 09 26;17(9):936-938. Epub 2019 Jun 26.

Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.

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http://dx.doi.org/10.1111/ddg.13884DOI Listing
September 2019

Bullöse Autoimmundermatosen.

J Dtsch Dermatol Ges 2018 Nov;16(11):1339-1360

Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg GmbH, Standort Marburg.

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http://dx.doi.org/10.1111/ddg.13688_gDOI Listing
November 2018

Bullous autoimmune dermatoses.

J Dtsch Dermatol Ges 2018 Nov;16(11):1339-1358

Department of Dermatology and Allergology, Marburg University Medical Center, Marburg, Germany.

Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases. The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Predominantly affecting elderly individuals, there has been a significant increase in its incidence in recent years. While mucosal lesions occur in less than 30 % of bullous pemphigoid patients, the second most common bullous autoimmune dermatosis, pemphigus vulgaris, typically presents with oral erosions as the predominant and - frequently - initial symptom. Its onset is usually in the 4 to 6 decade of life. Scarring is typically found in subepidermal blistering disorders such as epidermolysis bullosa acquisita or mucous membrane pemphigoid. Diagnosis is based on clinical and histological findings as well as direct and indirect immunofluorescence and detection of circulating autoantibodies. Although a number of controlled clinical trials have been conducted in recent years, treatment of bullous autoimmune disorders is still primarily based on clinical experience. Therapeutic options include topical and systemic corticosteroids as well as adjuvant immunosuppressants. Recalcitrant cases may require treatment with immunoadsorption, intravenous immunoglobulins, or the monoclonal anti-CD20 antibody rituximab.
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http://dx.doi.org/10.1111/ddg.13688DOI Listing
November 2018