2,048 results match your criteria Keratosis Follicularis Darier Disease


Patients with Darier Disease Exhibit Cognitive Impairment while Patients with Hailey-Hailey Disease Do Not: An Experimental, Matched Case-control Study.

Acta Derm Venereol 2021 Apr 30. Epub 2021 Apr 30.

Dermatology and Venereology Division, Department of Medicine (Solna), Karolinska Institutet, SE-17176 Stockholm, Sweden.

Darier disease and Hailey-Hailey disease are severe, monogenetic dermatological disorders with mutations affecting all cells, making them liable to exhibit extra-dermal symptoms. The aim of this study is to assess broad cognitive function in individuals with these diseases, using an experimental, case-control set-up comparing cognition in patients with that in healthy controls matched for age, sex and level of education. Cognition was assessed with the Cambridge Neuropsycho-logical Test Automated Battery. Read More

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Use of H-1 Antihistamine in Dermatology: More than Itch and Urticaria Control: A Systematic Review.

Dermatol Ther (Heidelb) 2021 Apr 12. Epub 2021 Apr 12.

Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan.

H-1 antihistamines are commonly used in dermatological practice for itch and urticaria control. The widespread expression of H-1 receptor on different cells in the skin and various biologic functions of H-1 antihistamines indicate the possible treatment potentials of H-1 antihistamines in dermatology. A literature search was performed on PubMed and Embase, targeting articles reporting use of antihistamine for purposes other than itch and urticaria control in dermatological practice. Read More

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Basal cell carcinoma associated with non-neoplastic cutaneous conditions: a comprehensive review.

Authors:
Philip R Cohen

Dermatol Online J 2021 Feb 15;27(2). Epub 2021 Feb 15.

San Diego Family Dermatology, National City, CA Touro University California College of Osteopathic Medicine, Vallejo, CA.

Basal cell carcinoma (BCC) can be a component of a collision tumor in which the skin cancer is present at the same cutaneous site as either a benign tumor or a malignant neoplasm. However, BCC can also concurrently occur at the same skin location as a non-neoplastic cutaneous condition. These include autoimmune diseases (vitiligo), cutaneous disorders (Darier disease), dermal conditions (granuloma faciale), dermal depositions (amyloid, calcinosis cutis, cutaneous focal mucinosis, osteoma cutis, and tattoo), dermatitis, miscellaneous conditions (rhinophyma, sarcoidal reaction, and varicose veins), scars, surgical sites, systemic diseases (sarcoidosis), systemic infections (leischmaniasis, leprosy and lupus vulgaris), and ulcers. Read More

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February 2021

Ophthalmic Assessment in Patients With Darier Disease.

Am J Ophthalmol 2021 Mar 15;227:139-142. Epub 2021 Mar 15.

From the Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel (H.H., E.A., A.Y., D.B., M.Z., R.P.D.-G.); Department of Dermatology, Emek Medical Center, Afula, Israel (H.H., A.Y., M.Z., R.P.D.-G.); Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada (R.P.D.-G.).. Electronic address:

Purpose: To assess the prevalence of ophthalmic findings in patients with Darier disease, an autosomal dominant genetic skin disorder, in an effort to evaluate the need for eye examinations in the management of the disease.

Design: Prospective observational case series.

Methods: Thirty-six individuals with Darier disease were evaluated by both ocular assessment questionnaire and a comprehensive ophthalmic examination (visual acuity, refraction, external examination, and slit-lamp examination) with emphasis on the eyelids, conjunctiva, and cornea. Read More

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An Update on the Cutaneous Manifestations of Darier Disease.

J Cutan Med Surg 2021 Mar 9:1203475421999331. Epub 2021 Mar 9.

26747 Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.

Background: Knowledge about the clinical features of Darier disease, an orphan autosomal-dominant genetic disorder, is sparse and has been evaluated only in few studies.

Objectives: To investigate the clinical features of a large group of patients with Darier disease, and to explore for associations between disease characteristics and severity of the disease.

Methods: Seventy-six individuals with Darier disease were evaluated utilizing a structured questionnaire-based interview, a physical examination, and a retrospective assessment of their medical records. Read More

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Darier sign in mastocytoma.

Med J Aust 2021 03;214(5):206

Liverpool Hospital, Sydney, NSW.

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Darier Disease: A Case Series of 20 Patients and Review of the Literature.

Actas Dermosifiliogr 2021 Mar 3. Epub 2021 Mar 3.

Servicio de Dermatología, Hospital Universitario Doctor Peset de Valencia, Valencia, España.

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Darier Disease.

Mayo Clin Proc 2021 03;96(3):688-689

Kaplan-Amonette Department of Dermatology, University of Tennessee Health Science Center, Memphis.

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Darier Disease - A Multi-organ Condition?

Acta Derm Venereol 2021 Apr 15;101(4):adv00430. Epub 2021 Apr 15.

Department of Medicine Solna, Karolinska Institutet, SE-171 76 Stockholm, Sweden. E-mail:

Darier disease is a severe, rare autosomal dominant inherited skin condition caused by mutations in the ATP2A2 gene encoding sarcoendoplasmic reticulum Ca2+-ATPase isoform 2 in the endoplasmic reticulum. Since sarcoendoplasmic reticulum Ca2+-ATPase isoform 2 is expressed in most tissues, and intracellular calcium homeostasis is of fundamental importance, it is conceivable that other organs besides the skin may be involved in Darier disease. This review focusses on the association of Darier disease with other organ dysfunctions and diseases, emphasizing their common molecular pathology. Read More

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Botulinum toxin injections as an effective treatment for patients with intertriginous Hailey-Hailey or Darier disease: an open-label 6-month pilot interventional study.

Orphanet J Rare Dis 2021 02 18;16(1):93. Epub 2021 Feb 18.

Reference Centre for Rare Skin Diseases, Dermatology Department (CRMRP), Larrey University Hospital, 24, Chemin de Pouvourville TSA 30030, 31059, Toulouse, France.

Background: Patients with Hailey-Hailey and Darier diseases present with disabling inflammatory lesions located in large skin folds, which are often exacerbated or induced by sweating. Quality of life is highly impaired because of pain and recurrent skin infections. An improvement in skin lesions after botulinum toxin A injections has previously been reported in some patients but no prospective interventional studies are available. Read More

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February 2021

Management of Darier disease: A review of the literature and update.

Indian J Dermatol Venereol Leprol 2021 Jan-Feb;87(1):14-21

Department of Dermatology, Saint George Hospital University Medical Center, Beirut, Lebanon.

Darier disease (DD) is a rare type of inherited keratinizing disorder with no definitive therapeutic approach. The objective of this study is to provide a detailed literature review of all the available treatment modalities of Darier disease, including those that are both surgical and non surgical, to compare their efficacies and to propose a novel therapeutic approach. A complete search of the literature for all articles describing the different treatments of Darier disease, with no restrictions on patients' ages, gender or nationalities, was performed with the use of PubMed. Read More

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November 2019

Darier's disease with epilepsy in an elderly patient after surgery for aortic dissection.

J Dermatol 2021 Apr 7;48(4):e169-e170. Epub 2021 Feb 7.

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

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Exuberant oral manifestation of Darier disease.

Int J Dermatol 2021 Jun 2;60(6):775-777. Epub 2021 Feb 2.

Laboratory of Morphology and Experimental Pathology, Institute of Technology and Research, Tiradentes University (UNIT), Aracaju, SE, Brazil.

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Laser Treatment of Darier Disease: Report of Two Cases and Systematic Review of the Literature.

J Lasers Med Sci 2020 3;11(4):395-404. Epub 2020 Oct 3.

Georgetown University School of Medicine, Washington, DC, USA.

Darier disease (DD) is a chronic disease with high morbidity and limited treatment options. Laser efficacy in the treatment of DD remains understudied. A literature search conducted between 07/21/2017 and 05/05/2018 identified all original cases of DD treated with laser therapy. Read More

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October 2020

Acquired Darier disease in a patient with metastatic prostate cancer: a paraneoplastic process?

Dermatol Online J 2020 Nov 15;26(11). Epub 2020 Nov 15.

Department of Dermatology, Aneurin Bevan University Health Board, Newport.

Darier disease is considered a hereditary acantholytic dermatosis that is inherited in an autosomal dominant manner and usually manifests in the first or second decade of life. We describe a 62-year-old man with an acquired variant of Darier disease after a recent diagnosis of metastatic prostate cancer. We discuss and postulate a novel mechanism for this unusual presentation. Read More

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November 2020

Darier sign in a teenager with primary cutaneous marginal zone lymphoma.

Pediatr Dermatol 2021 Mar 18;38(2):494-496. Epub 2020 Dec 18.

Department of Dermatology, University of Massachusetts Medical School, Worcester, MA, USA.

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B-cell lymphoma that arises in the skin. An adolescent male presented with dermal nodules on the arms, legs, and back with a positive Darier sign, ultimately diagnosed as PCMZL. The nodules demonstrated a partial response to doxycycline in the setting of prior Lyme disease followed by a complete response to rituximab. Read More

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The Whey Acidic Protein WFDC12 Is Specifically Expressed in Terminally Differentiated Keratinocytes and Regulates Epidermal Serine Protease Activity.

J Invest Dermatol 2021 May 4;141(5):1198-1206.e13. Epub 2020 Nov 4.

Department of Dermatology, Medical University of Vienna, Vienna, Austria. Electronic address:

WFDC proteins such as peptidase inhibitor 3 and SLPI inhibit proteases in the epidermis and other tissues. In this study, we tested the hypothesis that further WFDC protein family members might contribute to epidermal homeostasis. We found that in addition to peptidase inhibitor 3 and SLPI, WFDC5 and WFDC12 were expressed in human epidermis. Read More

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A Peculiar Case of Darier Disease in Blaschkoid Distribution.

Dermatol Pract Concept 2020 Oct 26;10(4):e2020078. Epub 2020 Oct 26.

Department of Surgical, Medical, Dental and Morphological Sciences, Transplant, Oncological and Regenerative Medicine; Dermatology Unit, University of Modena and Reggio Emilia, Modena, Italy.

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October 2020

Darier disease complicated by severe Kaposi varicelliform eruption resistant to acyclovir.

Dermatol Ther 2020 11 9;33(6):e14500. Epub 2020 Nov 9.

Department of Dermatovenereology, Third Faculty of Medicine, Charles University and Kralovske Vinohrady University Hospital, Prague, Czech Republic.

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November 2020

Darier disease restricted to the buttocks.

J Eur Acad Dermatol Venereol 2021 Apr 21;35(4):e268-e269. Epub 2020 Nov 21.

Clinical Trials and Cosmetics Testing Center, Hospital for Skin Diseases (Institute of Dermatology), Chinese Academy of Medical Sciences and Peking Union Medical Colleges, Nanjing, China.

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Type 1 Segmental Darier Disease: Case Report and Discussion of the Treatment Options.

Case Rep Dermatol 2020 Sep-Dec;12(3):159-167. Epub 2020 Sep 11.

Dermatology Teaching Assistant, King Abdul Aziz University, Jeddah, Saudi Arabia.

Darier disease is a rare type of autosomal dominant genodermatosis, and it is caused by a mutation in the gene coding for the endoplasmic reticulum membrane calcium pump Ca-ATPase type 2, leading to compromised intercellular adhesion. Moreover, this condition is characterized by multiple keratotic greasy papules with a seborrheic distribution and is worsened by heat and sun exposure, sweating, and friction. Occasionally, it may be associated with nail abnormalities and may involve the mucosa. Read More

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September 2020

Linear comedonal Darier disease: A rare variant.

Australas J Dermatol 2020 Oct 9. Epub 2020 Oct 9.

Department of Pathology, AIIMS, Bhubaneswar, India.

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October 2020

A 73-Year-Old With a Linear Rash.

Mayo Clin Proc 2020 10;95(10):2060-2061

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN; Department of Dermatology, Mayo Clinic, Rochester, MN. Electronic address:

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October 2020

Improvement in malodor with topical metronidazole gel in Darier disease.

JAAD Case Rep 2020 Oct 3;6(10):1027-1028. Epub 2020 Aug 3.

Department of Dermatology, University of California, Irvine, California.

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October 2020

[Surgical treatment of verrucous Darier disease].

Hautarzt 2020 Jul;71(Suppl 1):60-62

Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, Frauenlobstr. 9-11, 80337, München, Deutschland.

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Erythrodermic Darier disease associated with metastatic lung adenocarcinoma.

Dermatol Online J 2020 Jul 15;26(7). Epub 2020 Jul 15.

Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, AK.

We present a patient with erythrodermic Darier disease occurring in temporal association with diagnosis of metastatic lung adenocarcinoma. The patient presented with eroded and crusted keratotic papules and plaques over greater than 90% body surface area with extensive secondary impetiginization. Given a longstanding history of pruritic eruption, this presentation represents a paraneoplastic flare of an underlying genodermatosis. Read More

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The Use of Allograft Skin for the Treatment of Darier Disease.

Adv Skin Wound Care 2020 Oct;33(10):1-4

At the Burns Centre and Emilia Romagna Regional Skin Bank, M. Bufalini Hospital, in Cesena, Italy, Carmine D'Acunto, MD, and Catuscia Orlandi, MD, are dermatologists; Valeria Purpura, PhD, is a biotechnologist; and Davide Melandri, MD, is a dermatologist and plastic surgeon. The authors have disclosed no financial relationships related to this article. Submitted July 29, 2019; accepted in revised form October 11, 2019.

Darier disease is an autosomal dominant skin disorder characterized by keratotic papules. After their appearance, these lesions tend to grow over time, producing large and exudative plaques that compromise the general condition of the affected patient's skin. The authors report the clinical case of a patient affected by Darier disease with superinfected de-epithelialized areas over 30% of his body. Read More

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October 2020

Light and Laser Treatments for Keratosis Pilaris: A Systematic Review.

Dermatol Surg 2020 11;46(11):1397-1402

Department of Dermatology, Hotel Dieu de France University Hospital, Beirut, Lebanon.

Background: Keratosis pilaris (KP) is a common hereditary keratinization disorder. Keratosis pilaris rubra and KP atrophicans faciei are less frequent variants of the disease. Topical treatments often yield ineffective and temporary results. Read More

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November 2020

Darier Disease.

JAMA Dermatol 2020 10;156(10):1125

Department of Internal Medicine, Division of Dermatology, The Ohio State University Wexner Medical Center.

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October 2020

Successful Darier disease treatment in Russian patients.

Dermatol Ther 2020 11 11;33(6):e14028. Epub 2020 Aug 11.

Department of Dermatology and Venereology, Sechenov University, Moscow, Russian Federation.

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November 2020