2,114 results match your criteria Keratosis Follicularis Darier Disease


Keratosis Pilaris in an Ovarian Cancer Patient Treated with Bevacizumab and Doxorubicin.

Skinmed 2022 30;20(2):139-140. Epub 2022 Apr 30.

Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, PA.

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Response of Darier Disease Following Treatment With Baricitinib.

JAMA Dermatol 2022 Apr 27. Epub 2022 Apr 27.

Department of Dermatology and Venereology, La Paz University Hospital, Madrid, Spain.

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[Translated article] Coment on "Darier Disease: A Case Series of 20 Patients and Review of the Literature".

Authors:
J Redondo Mateo

Actas Dermosifiliogr 2022 Mar 7. Epub 2022 Mar 7.

Académico Emérito AEDV. Electronic address:

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Darier disease successfully treated with a topical agent containing vitamin A (retinyl palmitate), vitamin E, and urea.

J Dermatol 2022 Apr 19. Epub 2022 Apr 19.

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

Darier disease (DD), also called keratosis follicularis, is an autosomal dominant hereditary keratinization disorder that manifests as keratotic papules with plaques in seborrheic areas. There are no validated curative treatments for DD, with the majority of cases treated symptomatically. We report the efficacy of a topical over-the-counter agent which contains retinyl palmitate, vitamin E, and urea for a patient with DD. Read More

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Subcutaneous sarcoidosis (Darier-Roussy sarcoidosis) with extensive disease on positron emission tomography: A case report and review of the literature.

Respirol Case Rep 2022 May 10;10(5):e0949. Epub 2022 Apr 10.

Department of Respiratory Medicine Sir Charles Gairdner Hospital Nedlands Western Australia Australia.

Cutaneous manifestations of sarcoidosis are common, but subcutaneous nodules are rare, originally described in 1904 by Darier and Roussy and thought to represent isolated skin disease. We present a 61-year-old male who presented with 3 months of subcutaneous nodules on the forearms and knees. Biopsy confirmed sarcoidosis. Read More

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A case of segmental form of type 1 mosaic Darier's disease.

Acta Dermatovenerol Alp Pannonica Adriat 2022 03;31(Suppl):S33-S35

Department of Dermatovenereology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.

Darier's disease is a rare genetic disorder with autosomal dominant inheritance. It is characterized by hyperkeratotic papules in seborrheic areas. Associated abnormalities include nail abnormalities and changes in the mucous membranes. Read More

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Whole-transcriptome sequencing identifies postzygotic ATP2A2 mutations in a patient misdiagnosed with herpes zoster, confirming the diagnosis of very late-onset segmental Darier disease.

Exp Dermatol 2022 Mar 5. Epub 2022 Mar 5.

Department Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, and Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

An 82-year-old female patient presented with a recent onset of painful skin lesions in unilateral distribution on the abdominal area following the lines of Blaschko; the initial diagnosis of Varicella-Zoster infection was made. However, because the individual lesions appeared as hyperkeratotic papules and were unresponsive to antiviral therapy, a skin biopsy was performed, which revealed hyperkeratosis, suprabasal acantholysis and dyskeratosis with corps ronds and grains, consistent with acantholytic dyskeratotic acanthoma. Since this entity has been associated with Darier disease, whole-transcriptome sequencing by RNA-Seq was performed on RNA isolated from a lesion and from adjacent normal appearing skin, and a recently developed bioinformatics pipeline that can identify both genomic sequence variants and the presence of any of 926 viruses was applied. Read More

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Persistent perianal "warts".

JAAD Case Rep 2022 Mar 19;21:116-118. Epub 2022 Jan 19.

National Skin Centre, Singapore, Singapore.

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Follicular keratosis of the face in pediatric patients of color.

Pediatr Dermatol 2022 Mar 17;39(2):231-235. Epub 2022 Feb 17.

Department of Medicine, University of Chicago, Chicago, Illinois, USA.

Background/objectives: Follicular keratosis (FK) is a poorly understood disorder presenting with multiple, grouped hyperkeratotic follicular papules typically affecting the chin or jawline. This study describes the clinical presentation, histopathology, management, and outcomes of a series of pediatric patients of color with FK of the face, thought to be related to rubbing or friction on the skin.

Methods: Retrospective review of 20 pediatric patients with FK of the face who presented to our pediatric dermatology practice between April 2019 and October 2021. Read More

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Clinicopathological correlation of transient acantholytic dermatosis: A case report.

Exp Ther Med 2022 Feb 28;23(2):173. Epub 2021 Dec 28.

Department of Dermatology, University of Medicine and Pharmacy 'Grigore T. Popa', 700115 Iasi, Romania.

Transient acantholytic dermatosis (TAD) is a benign, non-familial, non-immune mediated acantholytic disorder of unknown etiology. The presence of polymorphous, unorganized, pruritic lesions on the trunk, associated with focal acantholysis and dyskeratosis, resembles a wide variety of dermatoses. The etiology of TAD (also known as Grover's disease) is unknown, and the success of treatment relies on the correct identification of the disease; however, some cases are refractory to all forms of therapy. Read More

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February 2022

Noonan Syndrome Presenting with Stunted Growth: A Case Report.

Mymensingh Med J 2022 Jan;31(1):263-266

Dr Ershad Mondal, Medical Officer, Department of Medicine, Mymensingh Medical College Hospital (MMCH), Mymensingh, Bangladesh; E-mail:

Noonan syndrome is a genetic disorder of autosomal dominant inheritance that prevents normal development in various parts of the body. A spontaneous mutation without any family history may also result in the condition. Noonan syndrome can affect normal growth. Read More

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January 2022

Keratosis pilaris in collagen type VI-related disorders.

Pediatr Dermatol 2022 Jan 5;39(1):133-134. Epub 2022 Jan 5.

Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, South Carolina, USA.

Keratosis pilaris is a common skin condition associated with a number of syndromes, including collagen type VI-related disorders. Our patient, recently diagnosed with Ullrich congenital muscular dystrophy, presented with severe keratosis pilaris, hypotonia, and velvety skin on the palms and soles. We present this case to highlight the importance of including cutaneous findings, such as keratosis pilaris, to aid in the diagnosis when evaluating patients with syndromic features. Read More

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January 2022

[Established Genital and Extragenital Lichen Sclerosus:A Clinicopathological Analysis].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2021 Dec;43(6):849-855

Graduate School,CAMS and PUMC,Beijing 100730,China.

Objective To investigate the clinicopathological characteristics of established genital and extragenital lichen sclerosus(LS)and compare the differences between them. Method The clinicopathological data of 55 patients with established genital and extragenital LS diagnosed by pathological examination in the Department of Dermatology of Beijing Hospital were retrospectively analyzed. Results The 55 patients included 11 males and 44 females. Read More

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December 2021

Characterization of Classical Flexural and Nummular Forms of Atopic Dermatitis in Childhood with Regard to Anamnestic, Clinical and Epidermal Barrier Aspects.

Acta Derm Venereol 2022 Mar 8;102:adv00664. Epub 2022 Mar 8.

Department of Dermatology, Venereology and Allergology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller Str. 3, DE-24105 Kiel, Germany.

Nummular (coin-shaped) and classical (flexural) atopic dermatitis differ morphologically, but no other distinguishing features are known. The aim of this study was to determine differences and similarities of both variants in children. Detailed interviews, clinical examinations, biophysical measurements and electron microscopic analyses were performed on 10 children with nummular atopic dermatitis, 14 with classical atopic dermatitis and 10 healthy controls. Read More

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Familial Leukodermic Darier Disease with Faun Tail Nevus in a Female Child - An Uncommmon Coexistence.

Indian Dermatol Online J 2021 Nov-Dec;12(6):936-938. Epub 2021 Nov 22.

Department of Dermatology, Venereology and Leprosy, Katuri Medical College and Hospital, Guntur, Andhra Pradesh, India.

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November 2021

Linear Darier's Disease.

N Engl J Med 2021 Dec 11;385(25):e91. Epub 2021 Dec 11.

Geisinger Medical Center, Danville, PA

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December 2021

Atypical keratosis pilaris-like lesions in a patient with Bethlem myopathy.

Pediatr Dermatol 2022 Mar 9;39(2):309-311. Epub 2021 Dec 9.

Department of Dermatology, University of California, San Diego, California, USA.

Bethlem myopathy is a collagen VI-related myopathy. Collagen VI is primarily not only associated with the extracellular matrix of skeletal muscle, but is also found in the skin, blood vessels, and other organs. Dermatologic findings described for Bethlem myopathy include follicular hyperkeratosis and abnormal scar formation, although clinical and histopathologic photographs remain elusive in the literature. Read More

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[Ulerythema ophryogenes as an entity associated with 18p- syndrome in a pediatric patient].

Arch Argent Pediatr 2021 12;119(6):e636-e638

Servicio de Dermatología del Complejo Hospitalario Universitario de Pontevedra, Pontevedra, España.

Ulerythema ophryogenes is a benign and rare skin disorder commonly presenting in childhood. It is characterized by erythematous and keratotic follicular papules located on the side of the eyebrows, and which over time tends to evolve into scarred alopecia. This entity may appear as an isolated clinical manifestation or associated with several congenital syndromes (18p-, Cornelia de Lange, Noonan, Rubinstein- Taybi, among others). Read More

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December 2021

A detailed regimen of isotretinoin for the successful treatment of severe keratosis pilaris.

Authors:
A Kirchner S Hoyer

Clin Exp Dermatol 2022 Mar 16;47(3):619-621. Epub 2021 Dec 16.

Department of Dermatology, University of Illinois College of Medicine, Chicago, IL, USA.

Although a well-known recommended treatment option, there are currently no studies that describe the detailed regimen of isotretinoin for the treatment of primary keratosis pilaris. Based on previous studies involving other hyperkeratotic disorders, this report describes a safe and effective treatment course of isotretinoin for severe keratosis pilaris. Read More

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Darier disease, radiation therapy, and herpesvirus -- an unfortunate triad.

Dermatol Online J 2021 Aug 15;27(8). Epub 2021 Aug 15.

University of Utah Health, Salt Lake City, UT.

Darier disease (DD) is a rare autosomal dominant keratinizing disorder often characterized by brown scaly pruritic papules over the face, neck, and trunk. Herein is reported a patient who developed secondary cutaneous herpes simplex virus (HSV) following exacerbation of his DD as a result of radiation therapy. In November 2020, a 78-year-old man presented to clinic for a pruritic rash on his back consistent with DD. Read More

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Clinicopathological Profile of Childhood Onset Cutaneous Mastocytosis from a Tertiary Care Center in South India.

Indian Dermatol Online J 2021 Sep-Oct;12(5):706-713. Epub 2021 Sep 10.

Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Background: Mastocytosis is characterized by clonal proliferation of mast cells in various organs and can have isolated cutaneous or systemic involvement. Childhood-onset mastocytosis (COM) is usually cutaneous and regresses spontaneously, while adult-onset mastocytosis (AOM) is often persistent with systemic involvement. There is limited data on COM from India. Read More

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September 2021

Darier and Hailey-Hailey disease: update 2021.

J Dtsch Dermatol Ges 2021 10;19(10):1478-1501

Department of Dermatology, Venereology and Allergology, University Würzburg, Würzburg, Germany.

The autosomal-dominant genodermatoses Darier disease and Hailey-Hailey disease present special challenges to dermatologists. Despite their similar pathogenesis featuring impaired adhesion of suprabasal keratinocytes as a result of defective ATPases in epidermal calcium channels, the two diseases differ considerably in clinical presentation and therapeutic options. Darier disease is characterized by reddish brown, keratotic papules in seborrheic and intertriginous areas, which may coalesce into extensive lesions. Read More

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October 2021

Correcting a 30-year diagnosis: A report of vesiculobullous Darier disease previously diagnosed as pemphigus vulgaris.

Int J Womens Dermatol 2021 Sep 1;7(4):492-493. Epub 2021 Mar 1.

Department of Dermatology, University of California-Irvine, Irvine, California, United States.

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September 2021

Darier's Disease Flare following COVID-19 Vaccine.

Case Rep Dermatol 2021 May-Aug;13(2):432-436. Epub 2021 Aug 18.

Department of Dermatology, Zealand University Hospital, Roskilde, Denmark.

Darier's disease is a rare genodermatosis characterized clinically by dyskeratotic papules in the seborrheic and intertriginous areas and nail abnormalities. Dyskeratosis and acantholysis are typical histological findings. Darier's disease is not known to be inflammatory by nature as inflammation occurs primarily due to local infections, and it may therefore differ from inflammatory dermatoses such as psoriasis and cutaneous lupus in response to antigen stimulation. Read More

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Utilities of Botulinum Toxins in Dermatology and Cosmetology.

Authors:
Piyu Parth Naik

Clin Cosmet Investig Dermatol 2021 21;14:1319-1330. Epub 2021 Sep 21.

Department of Dermatology, Saudi German Hospital and Clinic, Dubai, United Arab Emirates.

Botulinum toxin (BoNT) is a neurotoxin produced by the bacterium with a well-known efficacy and safety profile in the focal idiopathic hyperhidrosis treatment. BoNT comprises seven different neurotoxins; however, only toxins A and B are clinically employed. BoNT is lately practiced in off-label therapies for a variety of skin diseases. Read More

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September 2021

Maculopapular Cutaneous Mastocytosis Successfully Treated with Omalizumab.

Acta Dermatovenerol Croat 2021 Apr;291(1):60-61

Theodora Kanni, MD, PhD, Department of Dermatology and Venereology Andreas Sygros Hospital , 5 Ionos Dragoumi Str, 161 21 Athens, Greece;

Dear Editor, Maculopapular cutaneous mastocytosis (MPCM), formerly telangiectasia macularis eruptiva perstans (TMEP), is an uncommon form of cutaneous mastocytosis first described on 1930 (1). It is more frequent in adults, and early diagnosis is crucial since it has been reported to be associated with serious underlying systemic disorders, such as myeloproliferative diseases and severe manifestations like anaphylaxis (2,3). Treatment of MPCM depends on the presence of systemic involvement and/or the clinical symptoms of the disease itself. Read More

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An Uncommon Presentation of Darier-White Disease with Hystrix-like Palmoplantar Keratoderma.

Acta Dermatovenerol Croat 2021 Apr;291(1):39-41

Ilan Goldberg, MD, Department of Dermatology Tel-Aviv Sourasky Medical Center, 6 Weizmann Street, Tel-Aviv, Israel;

Darier-White disease is a relatively common autosomal dominant genodermatosis caused by mutation in the ATP2A2 gene. It is characterized by multiple warty papules coalescing into plaques in the seborrheic areas and by specific histological skin changes. Palm and sole involvement in Darier-White disease is usually mild, mainly featuring discrete and small keratotic papules. Read More

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Eruptive vellus hair cyst syndrome or exuberant atypical keratosis pilaris?

Rev Assoc Med Bras (1992) 2021 03;67(3):353-354

Universidade Federal da Bahia, Complexo Hospitalar Universitário Professor Edgard Santos, Departamento de Dermatologia - Salvador (BA) Brazil.

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Familial occurrence of follicular keratosis of the chin.

Authors:
Kiyofumi Egawa

J Dermatol 2021 Nov 25;48(11):1793-1796. Epub 2021 Aug 25.

Amakusa Dermatology and Internal Medicine Clinic, Kamiamakusa, Kumamoto, Japan.

Follicular keratosis of the chin is a rare and poorly understood pediatric disorder with a characteristic presentation of multiple follicular papules on the chin. Although prolonged friction or pressure over the chin is considered an etiology, such history is not present in most cases. It sometimes occurs within families without any evidence of physical trauma, suggesting a genetic predisposition of the patients to develop the disease. Read More

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November 2021

Kaposi-Juliusberg Varicelliform Eruption complicating Darier disease: May cannabinoid abuse be an underestimated risk factor?

Clin Case Rep 2021 Aug 16;9(8):e04589. Epub 2021 Aug 16.

Di.S.Sal., University of Genoa Genoa Italy.

Cannabinoid abuse may facilitate disseminated skin infection by herpes viruses in predisposed patients. These patients should be counselled about that. Read More

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