431 results match your criteria extracutaneous features

Clinicoprognostic implications of head and neck involvement by mycosis fungoides: A retrospective cohort study.

J Am Acad Dermatol 2021 Mar 23. Epub 2021 Mar 23.

Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. Electronic address:

Background: The clinicoprognostic implications of head and neck involvement of mycosis fungoides (MF) are poorly understood.

Objectives: To evaluate the association of head and neck involvement on the clinicoprognostic features of MF.

Methods: The clinical features and survival outcomes of patients with MF in a Korean academic medical center database were retrospectively evaluated according to the presence of head and neck involvement at diagnosis. Read More

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Extracutaneous Melanoma.

Hematol Oncol Clin North Am 2021 Feb 26;35(1):85-98. Epub 2020 Oct 26.

Division of Hematology/Oncology, Columbia University Irving Medical Center, Milstein Hospital Building, 177 Fort Washington Avenue, 6GN-435, New York, NY 10032, USA.

Extracutaneous melanomas (ECMs) represent a heterogeneous group of melanoma subtypes characterized by distinct clinical and biological features from cutaneous melanoma. These subtypes share an aggressive natural history with high mortalities compared with nonacral cutaneous melanoma (NACM). Although recent advances in NACM have made significant improvements in morbidity and mortality, ECMs continue to lag behind. Read More

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February 2021

Extracutaneous Involvement Is Common and Associated with Prolonged Disease Activity and Greater Impact in Juvenile Localized Scleroderma.

Rheumatology (Oxford) 2021 Mar 12. Epub 2021 Mar 12.

Department of Pediatrics, University School of Medicine, Riley Hospital for Children at Indiana University Health, Indianapolis,IN.

Objective: To evaluate factors associated with extracutaneous involvement (ECI) in juvenile localized scleroderma (jLS).

Methods: A prospective, multi-center, 6-month observational study was performed. Collected data included disease features, global assessments, and subject symptoms. Read More

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Mastocytosis in the skin accompanied by pseudo-Kaposi's sarcoma.

J Dermatol 2021 Mar 8. Epub 2021 Mar 8.

Spanish Reference Center of Mastocytosis, Instituto de Estudios de Mastocitosis de Castilla-La Mancha (CLMast), Hospital Virgen del Valle, Complejo Hospitalario de Toledo, Toledo, Spain.

Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of neoplastic mast cells in the skin and/or other extracutaneous tissues. Most patients with skin involvement can be subclassified into one of the three subtypes of cutaneous mastocytosis currently recognized by the World Health Organization (i.e. Read More

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Importance of extracutaneous organ involvement in determining the clinical severity and prognosis of incontinentia pigmenti caused by mutations in the IKBKG gene.

Exp Dermatol 2021 Mar 2. Epub 2021 Mar 2.

Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

Incontinentia pigmenti (IP) is a rare X-linked skin disease caused by mutations in the IKBKG gene, which is required for activation of the nuclear factor-kappa B signalling pathway. Multiple systems can be affected with highly variable phenotypic expressivity. We aimed to clarify the clinical characteristics observed in molecularly confirmed Korean IP patients. Read More

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Cutaneous, systemic features and laboratory characteristics of late- versus adult-onset systemic lupus erythematosus in 1006 Thai patients.

Lupus 2021 Apr 7;30(5):785-794. Epub 2021 Feb 7.

Division of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Background: Age at disease onset may modulate systemic lupus erythematosus (SLE), but its relation to cutaneous/extracutaneous manifestation remains understudied.

Objective: To compare the cutaneous, systemic features, laboratory characteristics, and disease severity between late- and adult-onset SLE patients.

Methods: Analyses of the cutaneous, systemic involvement, laboratory investigations, SLE disease activity index 2000 (SLEDAI-2K), and disease damage were performed to compare between groups. Read More

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A clinicopathological pattern of cutaneous tuberculosis and HIV concurrence in western Rajasthan.

Int J Mycobacteriol 2020 Oct-Dec;9(4):429-434

Department of Dermatology, Dr. Sampurnanand Medical College, Jodhpur, Rajasthan, India.

Background: Cutaneous tuberculosis (TB) forms a small subset of extrapulmonary TB and continues to be a significant diagnostic dilemma in routine practice. The present study is an attempt to find the incidence, clinical spectrum, and histopathological features of cutaneous TB in western Rajasthan. The relation of cutaneous TB with the human immunodeficiency virus (HIV) was also assessed. Read More

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December 2020

Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience).

Int J Dermatol 2020 Dec 10. Epub 2020 Dec 10.

Department of Pathology, Dr. Behcet Uz Children's Research and Training Hospital, Izmir, Turkey.

Background/objective: Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center.

Methods: Fourty-four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Read More

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December 2020

Rosai-Dorfman Disease Displays a Unique Monocyte-Macrophage Phenotype Characterized by Expression of OCT2.

Am J Surg Pathol 2021 01;45(1):35-44

Department of Laboratory Medicine and Pathology, Division of Hematopathology.

Rosai-Dorfman disease (RDD) is a rare histiocytosis with heterogenous clinical features. In this study, we characterized the histologic and phenotypic features in 33 RDD patients to better define the pathologic diagnosis. Cases included 24 patients with extracutaneous disease ("R" group), and 9 patients with lesions limited to the skin or subcutaneous tissue ("C" group). Read More

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January 2021

Hypertryptasemia and Mast Cell-Related Disorders in Severe Osteoporotic Patients.

Mediators Inflamm 2020 20;2020:5785378. Epub 2020 Oct 20.

Endocrinology and Diabetology Service, IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.

Purpose: Systemic mastocytosis (SM) is characterized by a clonal proliferation of neoplastic mast cells (MCs) in one or more extracutaneous organs including the bone marrow (BM). SM is often associated with osteoporosis (OP) and fractures. Hypertryptasemia usually occurs in SM. Read More

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October 2020

A retrospective study on clinical subtypes and management of morphea in 10 Italian Dermatological Units.

G Ital Dermatol Venereol 2020 Oct 16. Epub 2020 Oct 16.

Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola-Malpighi, Hospital, University of Bologna, Bologna, Italy.

Background: There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience.

Methods: A multicentre retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units. Read More

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October 2020

Cutaneous Lymphomas -Part II: Other Cutaneous Lymphomas.

Actas Dermosifiliogr 2021 Jan 10;112(1):24-31. Epub 2020 Oct 10.

Servicio de Dermatología, Hospital del Mar, Barcelona, España. Electronic address:

Primary cutaneous T-cell lymphomas other than mycosis fungoides, Sézary syndrome, and lymphoproliferative CD30 disorders are few, accounting for less than 5% of all cutaneous lymphomas. A cytotoxic phenotype is characteristic of these tumors, and their clinical behavior is usually aggressive. Patients often present with extracutaneous symptoms or develop them shortly after diagnosis. Read More

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January 2021

A clinical and histological comparison between acute cutaneous graft-versus-host disease and other maculopapular eruptions following hematopoietic stem cell transplantation: a retrospective cohort.

Int J Dermatol 2021 Jan 29;60(1):60-69. Epub 2020 Sep 29.

Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Background: Prompt management of acute graft-versus-host disease (GVHD) may prevent its morbidity and mortality. Maculopapular (MP) eruption is a presenting sign of acute GVHD, but differentiation from other causes of MP rash is challenging.

Methods: A retrospective study was conducted among patients developing MP eruptions after allogeneic hematopoietic stem cell transplantation. Read More

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January 2021

Epidermolysis bullosa.

Nat Rev Dis Primers 2020 09 24;6(1):78. Epub 2020 Sep 24.

Epidermolysis Bullosa Unit, Department of Dermatology, University Hospitals Birmingham National Health Service Foundation Trust, Birmingham, UK.

Epidermolysis bullosa (EB) is an inherited, heterogeneous group of rare genetic dermatoses characterized by mucocutaneous fragility and blister formation, inducible by often minimal trauma. A broad phenotypic spectrum has been described, with potentially severe extracutaneous manifestations, morbidity and mortality. Over 30 subtypes are recognized, grouped into four major categories, based predominantly on the plane of cleavage within the skin and reflecting the underlying molecular abnormality: EB simplex, junctional EB, dystrophic EB and Kindler EB. Read More

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September 2020

Malignant Melanoma: Skin Cancer-Diagnosis, Prevention, and Treatment.

Crit Rev Eukaryot Gene Expr 2020 ;30(4):291-297

Institute of Molecular Biology & Biotechnology, Bahauddin Zakariya University, Multan, Pakistan.

Melanoma is a skin cancer caused by a malignancy of melanocytes. Incidence of melanoma is rapidly increasing worldwide, which results in public health problems. Primary extracutaneous melanomas can be ocular, gastrointestinal, mucosal, leptomeningeal, genitourinary, and lymphatic. Read More

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January 2020

Melanoma in the setting of nevus of Ota: a review for dermatologists.

Int J Dermatol 2020 Aug 17. Epub 2020 Aug 17.

Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.

Nevus of Ota, also known as oculodermal melanocytosis or nevus fuscoceruleus ophthalmomaxillaris, is a benign dermal melanocytic nevus that most commonly affects Asian women. While the lesion is largely a cosmetic concern, it has the potential to undergo malignant transformation to melanoma. Patients with nevus of Ota often present to a dermatologist at some point for examination or removal with laser therapy. Read More

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Indeterminate dendritic cell tumor in the pancreas.

J Surg Case Rep 2020 Jul 16;2020(7):rjaa208. Epub 2020 Jul 16.

Department of General, Visceral, Endocrine and Transplantation Surgery, Kantonsspital St. Gallen, 9000 St. Gallen, Switzerland.

Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic neoplastic disorder with proliferation of indeterminate dendritic cells. In the vast majority of cases, IDCTs are restricted to the skin or lymph nodes. To our knowledge, we report the first case of IDCT in the pancreas. Read More

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Primary intra-abdominal melanoma arising in association with extracutaneous blue naevus: a report of two cases.

Histopathology 2021 Jan 16;78(2):281-289. Epub 2020 Oct 16.

Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.

Aims: Blue naevi are uncommon dermal melanocytic neoplasms characterised by GNAQ/GNA11 mutations, which very rarely progress to melanoma. Such melanomas also often have BAP1 mutations, and lack genetic events associated with conventional melanoma. Exceptionally, blue naevi arise in extracutaneous locations; one melanoma arising in this setting has been reported. Read More

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January 2021

Epidermolysis bullosa with congenital absence of skin: Review of the literature.

Pediatr Dermatol 2020 Sep 20;37(5):821-826. Epub 2020 Jul 20.

Department of Dermatology, University Hospital "Dr. José Eleuterio González", Universidad Autónoma de Nuevo León, Monterrey, México.

Background/objectives: Bart syndrome was initially described as association of congenital absence of skin (CAS), nail abnormalities, and epidermolysis bullosa (EB). Further reports of patients with CAS and EB have been made with wide clinical heterogeneity among them. Current guidelines recommend the elimination of eponyms and use of the descriptive term EB with CAS. Read More

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September 2020

Characterization of vascular stains associated with high flow.

J Am Acad Dermatol 2021 Mar 27;84(3):654-660. Epub 2020 Jun 27.

Department of Dermatology, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York; Department of Pediatrics, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York. Electronic address:

Background: High-flow vascular stains (HFVS) are lesions that have the appearance of capillary malformations/port wine stains but are associated with increased arterial flow.

Objective: To identify features of HFVS that differentiate them from typical "slow-flow" port wine stains.

Methods: Retrospective multicenter cohort study of HFVS evaluated across 7 centers was conducted. Read More

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[Reticular erythematous mucinosis-A special subtype of cutaneous lupus erythematosus?]

Z Rheumatol 2020 Oct;79(8):782-784

Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik Oberhausen, Universität Witten/Herdecke, Josefstr. 3, 46045, Oberhausen, Deutschland.

Reticular erythematous mucinosis (REM syndrome) is a rare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Read More

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October 2020

A genomic survey of sarcomas on sun-exposed skin reveals distinctive candidate drivers and potentially targetable mutations.

Hum Pathol 2020 08 12;102:60-69. Epub 2020 Jun 12.

Department of Pathology, University of Michigan, 2800 Plymouth Road, Ann Arbor, MI, 48109, USA; Department of Dermatology, University of Michigan, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109, USA; Rogel Cancer Center, University of Michigan, 1500 E. Medical Center Drive Ann Arbor, MI, 48109, USA. Electronic address:

Sarcomas on photodamaged skin vary in prognosis and management, but can display overlapping microscopic and immunophenotypic features. Improved understanding of molecular alterations in these tumors may provide diagnostic and therapeutic insights. We characterized 111 cutaneous sarcomatoid malignancies and their counterparts, including primary cutaneous angiosarcoma (n = 7), atypical fibroxanthoma (AFX) (n = 21), pleomorphic dermal sarcoma (PDS) (n = 17), extracutaneous undifferentiated pleomorphic sarcoma (n = 8), cutaneous leiomyosarcoma (LMS) (n = 5), extracutaneous LMS (n = 9), sarcomatoid squamous cell carcinoma (spindle cell squamous cell carcinoma) (S-SCC) (n = 24), and conventional cutaneous squamous cell carcinoma (SCC) (n = 20), by next-generation sequencing (NGS) using the StrataNGS panel for copy number variations, mutations, and/or fusions in more than 60 cancer-related genes. Read More

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Cutaneous manifestations in patients with COVID-19: a preliminary review of an emerging issue.

Br J Dermatol 2020 Sep 5;183(3):431-442. Epub 2020 Jul 5.

Dermatology and Venereology Private Practice, Bari and Barletta, Italy.

Background: The infection caused by the recently identified SARS-CoV-2, called coronavirus disease-19 (COVID-19), has rapidly spread throughout the world. With the exponential increase of patients worldwide, the clinical spectrum of COVID-19 is being better defined and new symptoms are emerging. Numerous reports are documenting the occurrence of different cutaneous manifestations in patients with COVID-19. Read More

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September 2020

Cutaneous polyautoimmunity in two unrelated dogs: pemphigus foliaceus and generalized discoid lupus erythematosus.

Vet Dermatol 2020 Aug 27;31(4):325-e84. Epub 2020 Apr 27.

Department of Clinical Sciences, College of Veterinary Medicine, NC State University, 1060 William Moore Drive, Raleigh, NC, 27607, USA.

Background: Polyautoimmunity, the concurrent expression of two or more distinct autoimmune diseases (ADs) in a single individual, is a known phenomenon in humans and has been rarely reported in dogs. To the best of the authors' knowledge, comorbid pemphigus foliaceus (PF) and generalized discoid lupus erythematosus (GDLE) has not been reported in dogs.

Hypothesis/objectives: To describe the clinical, histological and immunological features and treatment outcome of two unrelated dogs with comorbid PF and GDLE. Read More

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Cutaneous arteriolitis: A novel cutaneous small vessel vasculitis disorder clinicopathologically different from cutaneous polyarteritis nodosa and cutaneous venulitis.

J Cutan Pathol 2020 Sep 20;47(9):854-859. Epub 2020 Jul 20.

Meguro Chen Dermatology Clinic, Tokyo, Japan.

Cutaneous vasculitis can be classified into two types based on the affected vessel size: small vessel vasculitis predominantly affecting dermal venules, and muscular vessel vasculitis as found in cutaneous arteritis predominantly affecting arteries located at the dermal-subcutaneous junction. We describe two cases with a novel small vessel vasculitis disorder, which exclusively affected arterioles in the mid-dermis, and show clinical and pathological difference distinct from cutaneous polyarteritis nodosa and cutaneous venulitis. Both patients were male, and presented with painful infiltrative plaques, involving the palms, soles, and thighs without extracutaneous involvement except for fever and arthralgia. Read More

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September 2020

Novel use of Autoinflammatory Diseases Activity Index (AIDAI) captures skin and extracutaneous features to help manage pediatric DITRA: A case report and a proposal for a modified disease activity index in autoinflammatory keratinization disorders.

Pediatr Dermatol 2020 Jul 17;37(4):670-676. Epub 2020 Apr 17.

Department of Paediatrics, University of Calgary, Calgary, AB, Canada.

Generalized pustular psoriasis (GPP) is a severe form of psoriasis, which is rare in pediatric and adult patients. It is characterized by sterile pustular lesions that appear on erythematous skin, associated with systemic features. A recent identification of mutations in the IL36RN gene in some GPP patients has led to a diagnosis of new autoinflammatory disease, interleukin-36-receptor antagonist deficiency (DITRA). Read More

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Giant pyoderma gangrenosum in a patient with ulcerative colitis: A case report.

Medicine (Baltimore) 2020 Feb;99(6):e18795

Department of Dermatology, Qujing First People's Hospital affiliated to Kunming Medical University, Qujing, Yunnan, China.

Introduction: Pyoderma gangrenosum (PG) is a phenomenon of cutaneous ulceration with unknown etiology. About half the cases have associated extracutaneous manifestations or associated systemic diseases. The most commonly associated systemic disorders include inflammatory bowel disease (IBD), hematologic malignancies, autoimmune arthritis, and vasculitis. Read More

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February 2020

A novel nonsense mutation in the STS gene in a Pakistani family with X-linked recessive ichthyosis: including a very rare case of two homozygous female patients.

BMC Med Genet 2020 01 31;21(1):20. Epub 2020 Jan 31.

Faculty of Allied and Health Sciences, Imperial College of Business Studies, Lahore, Pakistan.

Background: X-linked ichthyosis (XLI; OMIM# 308100) is a recessive keratinization disorder characterized by the presence of dark brown, polygonal, adherent scales on different parts of the body surface. It almost exclusively affects males and the estimated prevalence ranges from 1:2000-6000 in males worldwide. Extracutaneous manifestations are frequent including corneal opacities, cryptorchidism, neuropsychiatric symptoms or others. Read More

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January 2020