63 results match your criteria Ulerythema


[Keratosis pilaris].

Pan Afr Med J 2019 30;33:274. Epub 2019 Jul 30.

Hôpital Civil Tétouan, Tétouan, Maroc.

Keratosis pilaris is characterized by small bumps around hair follicles. Keratosis pilaris simplex results in gray and keratotic papules mainly on the arms, thighs and buttocks. It mainly affects women. Read More

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November 2019

Dermatological manifestations in Noonan syndrome: a prospective multicentric study of 129 patients positive for mutation.

Br J Dermatol 2019 06 18;180(6):1438-1448. Epub 2019 Jan 18.

Department of Clinical Genetics, Arnaud de Villeneuve Hospital, Montpellier, France.

Background: Data on dermatological manifestations of Noonan syndrome (NS) remain heterogeneous and are based on limited dermatological expertise.

Objectives: To describe the dermatological manifestations of NS, compare them with the literature findings, and test for dermatological phenotype-genotype correlations with or without the presence of PTPN11 mutations.

Methods: We performed a large 4-year, prospective, multicentric, collaborative dermatological and genetic study. Read More

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And next… Adnexa: Ulerythema ophryogenes and keratosis pilaris.

Eur J Dermatol 2018 Aug;28(4):566-567

Department of Dermatology and Venereology, Hospital de Santo António dos Capuchos - Centro Hospitalar de Lisboa Central, Alameda de Santo António dos Capuchos, 1169-050 Lisbon, Portugal.

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Dermatological manifestations in cardiofaciocutaneous syndrome: a prospective multicentric study of 45 mutation-positive patients.

Br J Dermatol 2019 01 30;180(1):172-180. Epub 2018 Sep 30.

Department of Genetic Biochemistry, Robert-Debré Hospital, AP-HP and University of Paris-Diderot, Paris, France.

Background: Data on dermatological manifestations of cardiofaciocutaneous syndrome (CFCS) remain heterogeneous and almost without expert dermatological classification.

Objectives: To describe the dermatological manifestations of CFCS; to compare them with the literature findings; to assess those discriminating CFCS from other RASopathies, including Noonan syndrome (NS) and Costello syndrome (CS); and to test for dermatological phenotype-genotype correlations.

Methods: We performed a 4-year, large, prospective, multicentric, collaborative dermatological and genetic study. Read More

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January 2019

Lichenoid folliculitis: A unifying concept.

J Cutan Pathol 2017 Jul 5;44(7):647-654. Epub 2017 May 5.

Department of Dermatology, Uniformed Services University of the Health Sciences, Los Angeles, Maryland.

Skin diseases presenting with keratotic papules, atrophy, cicatricial alopecia and/or "lichenoid" histopathologic changes have been described under at least 30 names. This family of diseases contains 2 subgroups, largely based on clinical features: keratosis pilaris atrophicans (KPA; including keratosis pilaris atrophicans faciei/ulerythema ophryogenes, atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans); and the lichen planopilaris (LPP) subgroup (including LPP, frontal fibrosing alopecia, Graham-Little-Piccardi-Lassueur Syndrome and fibrosing alopecia in a pattern distribution). An interface dermatitis with lichenoid inflammation is characteristic of the LPP group of disorders, but the literature provides scant information about the histopathology of the KPA group. Read More

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Red face revisited: Disorders of hair growth and the pilosebaceous unit.

Clin Dermatol 2014 Nov-Dec;32(6):784-99. Epub 2014 Mar 1.

Sector of Dermatology and Post-Graduation Course, University Hospital, and School of Medicine, Federal University of Rio de Janeiro, Rua Dona Mariana 143/C-32, Botafogo 22280-020, Rio de Janeiro, Brazil.

This paper reviews the recent literature on the diseases of the hair and pilosebaceous unit that may cause a red face. We discuss the epidemiology, clinicals, pathogenesis, and therapy of lichen planopilaris with its variants, discoid lupus erythematosus, folliculitis decalvans, dissecting folliculitis, acne keloidalis nuchae, pseudofolliculitis barbae, tinea capitis, tinea barbae, folliculitis of diverse causative factors and inflammatory follicular keratotic syndromes, ulerythema ophryogenes, atrophoderma vermiculatum, keratosis follicularis spinulosa decalvans, and folliculitis spinulosa decalvans. Read More

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Red face and fungi infection.

Clin Dermatol 2014 Nov-Dec;32(6):734-8. Epub 2014 Mar 2.

Department of Dermatology, Dr. Jose Eleuterio Gonzalez University Hospital, Universidad Autonoma de Nuevo Leon, Monterrey, Mexico.

Red face syndrome is characterized by an erythematous dermatitis that is produced by different entities. These include rosacea, seborrheic dermatitis, contact dermatitis, atopic dermatitis, psoriasis, cutaneous lupus, photodermatosis, post-topical steroid dermatosis, demodicosis, borderline borderline (BB) leprosy, mastocytosis, carcinoid, postneoplasia flushing, cutaneous lymphoma, tineas, ulerythema ophryogenes, and psychosomatic flushing. Red face is a relatively common dermatologic manifestation. Read More

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Trias of keratosis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis syndrome.

J Dermatol 2014 May;41(5):371-6

Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, Germany.

Keratosis pilaris and ulerythema ophryogenes (keratosis pilaris atrophicans faciei) are part of a group of hereditary disorders of hair follicle keratinization involving follicular inflammation and subsequent atrophy. Monosomy 18p refers to a chromosomal disorder resulting from the deletion of all or part of the short arm of chromosome 18. This trias was first described in a patient by Zouboulis et al. Read More

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Ulerythema ophryogenes: updates and insights.

Cutis 2014 Feb;93(2):83-7

Department of Dermatology, Rutgers New Jersey Medical School, Medical Science Building, H-576, Newark, NJ 07103, USA.

Ulerythema ophryogenes is a rare cutaneous atrophic disorder that occasionally is associated with Noonan syndrome, de Lange syndrome, Rubinstein-Taybi syndrome, and cardiofaciocutaneous (CFC) syndrome. Often presenting in pediatric patients, the pathogenesis of ulerythema ophryogenes remains unclear, though several genetic causes have been suggested. Treatment recommendations remain anecdotal, but clearance has been noted as the patient ages. Read More

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February 2014

Ulerythema ophryogenes, a rarely reported cutaneous manifestation of noonan syndrome: case report and review of the literature.

J Cutan Med Surg 2013 May-Jun;17(3):212-8

University of Calgary, Calgary, AB, Canada.

Background: Ulerythema ophryogenes (also known as keratosis pilaris atrophicans faciei) is a rarely reported cutaneous manifestation of Noonan syndrome.

Objective: Recognizing ulerythema ophryogenes as a cutaneous association in Noonan syndrome may aid in the diagnosis of this relatively common genetic condition.

Methods: We present a case of a patient with Noonan syndrome and ulerythema ophryogenes associated with a SOS1 mutation and review the literature on this association. Read More

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Ulerythema ophryogenes and Noonan syndrome.

Dermatol Online J 2013 Feb 15;19(2):14. Epub 2013 Feb 15.

We describe a patient with Noonan syndrome (NS) presenting with ulerythema ophrygenes (UO)--an association initially suggested in a single case series of five patients by Pierini and Pierini in 1979. Recognition of the association of UO with NS by pediatric dermatologists is important because of the high incidence of cardiovascular anomalies in NS. Therefore, in infants and children presenting with UO, clinicians should maintain a high index of suspicion for NS and refer for further workup. Read More

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February 2013

Keratosis pilaris and ulerythema ophryogenes in a woman with monosomy of the short arm of chromosome 18.

An Bras Dermatol 2011 Jul-Aug;86(4 Suppl 1):S42-5

Serviço de Dermatologia, Complexo Hospitalar Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brasil.

Partial monosomy of the short arm of chromosome 18 (18p- syndrome) is characterized mainly by speech delay, mild to moderate mental retardation and short stature. We describe a patient with the 18p- syndrome and widespread severe keratosis pilaris and ulerythema ophryogenes. This is the fourth case in which such an association has been reported. Read More

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Dermatological phenotype in Costello syndrome: consequences of Ras dysregulation in development.

Br J Dermatol 2012 Mar;166(3):601-7

Department of Dermatology and Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road TBRC, 2nd Floor, Suite C2010, Milwaukee, WI 53226, USA.

Background: The RASopathies are a class of human genetic syndromes caused by germline mutations in genes that encode protein components of the Ras/mitogen-activated protein kinase (MAPK) pathway. Costello syndrome (CS) is a RASopathy caused by mutations in the HRAS gene, a key regulator of signal transduction.

Objective: To quantify the specific cutaneous phenotype observed in 46 individuals with Costello syndrome with confirmed HRAS mutations. Read More

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Ulerythema ophryogenes, a rare and often misdiagnosed syndrome: analysis of an idiopathic case.

Int J Immunopathol Pharmacol 2011 Apr-Jun;24(2):523-7

Keratosis pilaris (KP) is a follicular hyperkeratosis disorder which is frequently detected in the adult population (44%), mostly in female adolescents (80%). It is a genetic autodominant dermatosis with variable penetrance, but no specific gene association has been determined, even though association to the presence of chromosome 18p deletion has been reported in some cases. We report the case of a 51-year-old Caucasian woman affected by keratosis pilaris gradually progressing with age and with a story of multiple abortions. Read More

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Dermatological findings in 61 mutation-positive individuals with cardiofaciocutaneous syndrome.

Br J Dermatol 2011 Mar 28;164(3):521-9. Epub 2011 Jan 28.

Department of Dermatology and Pediatrics, Oregon Health and Science University, Portland, USA.

Background: The RASopathies are a class of human genetic syndromes that are caused by germline mutations in genes which encode components of the Ras/mitogen-activated protein kinase (MAPK) pathway. Cardiofaciocutaneous (CFC) syndrome is characterized by distinctive craniofacial features, congenital heart defects, and abnormalities of the skin and hair.

Objectives: Systematically to characterize the spectrum of dermatological findings in mutation-positive individuals with CFC syndrome. Read More

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Palmoplantar keratoderma, pseudo-ainhum, and universal atrichia: A new patient and review of the palmoplantar keratoderma-congenital alopecia syndrome.

Am J Med Genet A 2010 Aug;152A(8):2043-7

Medical Genetics, Department of Experimental Medicine, Sapienza - University of Rome, San Camillo-Forlanini Hospital, Rome, Italy.

Palmoplantar keratoderma (PPK) may concur with congenital alopecia (CA) in various genodermatoses. We report on a 10-year-old girl with generalized atrichia and a severe form of PPK causing pseudo-ainhum, sclerodactyly, and contractures, a phenotype not consistent with any well-defined condition. Non-specific additional findings comprised mild nail dystrophy and widespread keratosis pilaris including ulerythema ophryogenes. Read More

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[Keratosis pilaris and keratosis pilaris atrophicans faciei].

J Dtsch Dermatol Ges 2006 Apr;4(4):319-23

Dermatology Clinic, University Hospital, Basel, Switzerland.

Keratosis pilaris and ulerythema ophryogenes (keratosis pilaris atrophicans faciei) are hereditary disorders with altered follicular keratinization that show follicular, horny papules surrounded by an erythematous halo. Ulerythema ophryogenes is an uncommon variant of keratosis pilaris characterized by erythematous follicular papules of the eyebrows and cheeks followed by a gradual loss of hair. On the background of 15-year-old boy who presented with keratosis pilaris and ulerythema ophryogenes, we discuss the various clinical manifestations of keratosis pilaris. Read More

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Hereditary woolly hair and keratosis pilaris.

J Am Acad Dermatol 2006 Feb;54(2 Suppl):S35-9

Division of Dermatology, University of Washington, Seattle, Washington, USA.

We describe a family with woolly hair and ulerythema ophryogenes spanning four generations. Both woolly hair and ulerythema ophryogenes have been associated with Noonan syndrome and cardiofaciocutaneous syndrome (CFC), two disorders with considerable phenotypic overlap. This family did not exhibit any of the other findings characteristic of either Noonan syndrome or CFC, similar to a previously described pedigree with hereditary woolly hair. Read More

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February 2006

Interstitial deletion of chromosome 12q: genotype-phenotype correlation of two patients utilizing array comparative genomic hybridization.

Am J Med Genet A 2005 Nov;138(4):349-54

Department of Pediatrics, Division of Medical Genetics, University of California San Francisco, San Francisco, California 94115, USA.

Interstitial deletions of chromosome 12q are rare, with only 11 reported cases in the literature. We recently described two cases with cytogenetically identical interstitial deletions of the long arm of chromosome 12. Here, we report on a third patient, a 26-month-old male with a cytogenetically-identical interstitial deletion: 46,XY,del(12)(q21. Read More

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November 2005

Cardiofaciocutaneous syndrome (CFC) with congenital peripheral neuropathy and nonorganic malnutrition: an autopsy study.

Am J Med Genet A 2005 Aug;137(1):1-8

Pediatric Pathology, University of South Alabama Children's and Women's Hospital, Mobile, 36604, USA.

Many phenotypic manifestations have been reported in cardiofaciocutaneous (CFC) syndrome, but none, to date, are pathognomonic or obligatory. Previous histopathological studies reported findings in skin and hair; no autopsy studies have been published. We report the clinical and autopsy findings of a 7-year-old boy with severe CFC syndrome and malnutrition of psychosocial origin. Read More

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Self-healing congenital verruciform hyperkeratosis.

Am J Med Genet A 2004 Oct;130A(3):303-6

Unité de Dermatologie Pédiatrique et, Hôpital Pellegrin-Enfants, Place Amelie Raba-Leon, Bordeaux, France.

We report on an apparently previously undescribed neonatal diffuse congenital hyperkeratosis with spontaneous improvement. The child, born to consanguinous parents, presented at birth with a verrucous hyperkeratosis involving face, trunk, and limbs, but sparing palms and soles. No visceral or skeletal abnormality was associated and neurosensory status was normal. Read More

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October 2004

Ulerythema ophryogenes and keratosis pilaris.

Eur J Dermatol 2002 Nov-Dec;12(6):572

Department of Clinical and Experimental Medicine, Division of Dermatology, University of Bologna, Italy.

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[Atrophic pilar keratosis of the face: case report].

Med Pregl 2001 Sep-Oct;54(9-10):486-9

Klinicki centar Novi Sad, Klinika za kozno-venericne bolesti, 21000 Novi Sad, Hajduk Veljkova 1-3.

Introduction: Keratosis pilaris atrophicans faciei (KPAF), previously called ulerythema ophryogenes, belongs to a group of follicular syndromes with inflammation and atrophy. The disease often starts at birth or during the first months of life with autosomal dominant inheritance.

Case Report: We report a case of a 24-year-old woman, who noticed the first lesion two years ago. Read More

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Ulerythema ophryogenes in Cornelia de Lange syndrome.

Pediatr Dermatol 2002 Jan-Feb;19(1):42-5

Department of Dermatology, Complejo Hospitalario Universitario, Faculty of Medicine, Santiago de Compostela, Spain.

A 17-year-old woman with Cornelia de Lange syndrome had asymptomatic skin lesions since the age of 4 years. These were multiple, follicular, horny papules, present on both cheeks, and surrounded by erythematous skin. Similar lesions were present on the external aspect of the arms, but amidst skin of normal coloration. Read More

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Treatment of keratosis pilaris atrophicans with the pulsed tunable dye laser.

J Cutan Laser Ther 2000 Sep;2(3):151-6

Department of Dermatology, University Hospital of Wales, Cardiff, Wales, UK.

Background: Few therapies are currently available to treat keratosis pilaris atrophicans (KPA), a spectrum of disorders which includes ulerythema ophryogenes and atrophoderma vermiculata.

Objective: To evaluate the response of KPA to treatment with the pulsed dye laser (PDL) with regard to improvements in erythema and skin roughness, treatment tolerability, and side effects.

Methods: Treatment of all facial areas involved with KPA with the PDL at 585 nm was evaluated in 12 patients. Read More

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September 2000

Noonan syndrome: a case with recurrent keloid formation.

Cutis 2001 Apr;67(4):315-6

Department of Dermatology, Baskent Universitesi Tip Fakültesi, Dermatoloji Anabilim Dali, 12. sokak, No 7/6, Bahçelievler 06490, Ankara, Turkey.

We describe a 6-year-old boy who presented with erythema and horny, follicular papules on the lateral aspects of the eyebrows and extensor surfaces of the arms. The condition was diagnosed as ulerythema ophryogenes and keratosis pilaris atrophicans faciei. The patient had the characteristic features of Noonan syndrome, including dysmorphic facial appearance, congenital heart disease, pectus excavatum, and cubitus valgus, accompanied by a tendency for keloid formation. Read More

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Keratosis pilaris and ulerythema ophryogenes associated with an 18p deletion caused by a Y/18 translocation.

Am J Med Genet 1999 Jul;85(2):179-82

Institute of Medical Genetics, Tomsk, Russia.

We present a patient with partial monosomy of the short arm of chromosome 18 caused by de novo translocation t(Y;18) and a generalized form of keratosis pilaris (keratosis pilaris affecting the skin follicles of the trunk, limbs and face-ulerythema ophryogenes). Two-color FISH with centromere-specific Y and 18 DNA probes identified the derivative chromosome 18 as a dicentric with breakpoints in p11.2 on both involved chromosomes. Read More

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Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy.

Pediatr Dermatol 1999 Mar-Apr;16(2):134-6

Department of Dermatology, General Hospital of Galicia, Faculty of Medicine, Santiago de Compostela, Spain.

Rubinstein-Taybi syndrome is characterized by the presence of a peculiar facies, mental retardation, and broad thumbs and great toes. Several associated cutaneous abnormalities have been reported with this syndrome. Ulerythema ophryogenes is a form of follicular keratosis associated occasionally with other ectodermal defects and congenital anomalies. Read More

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September 1999