keyword
https://read.qxmd.com/read/38443117/urticaria-pigmentosa-without-pruritus
#1
JOURNAL ARTICLE
William J Nahm, Denise J Corriveau, Chris A Mathe, W Austin Wyant, Carlos Vega, Anita Arthur, K Chris Min
Mastocytosis is a group of disorders characterized by the pathologic accumulation of mast cells in various tissues. One example of mastocytosis is urticaria pigmentosa, which presents with mastocytomas that can cause hives and, when irritated, pruritus. To our knowledge, we are describing the first case of urticaria pigmentosa without pruritus. The patient had a positive Darier's sign, stated that they never felt itchy, and denied ever using a topical steroid or antihistamine. Although our patient declined additional testing, patients like this may benefit from a detailed evaluation of their sensory system through both quantitative sensory testing and genetic analysis...
March 1, 2024: Journal of Drugs in Dermatology: JDD
https://read.qxmd.com/read/38313964/bullous-mastocytosis-a-rare-variant-of-diffuse-cutaneous-mastocytosis
#2
Shamma Khamis Almheiri, Jaheersha Pakran, Amani Abdulla AlFalasi, Reem El Bahtimi
Mastocytosis is a disease of the mast cells caused by an increase in the number of mast cells due to abnormal proliferation. The disease is associated with a mutation in the c-kit gene, which is a key factor in the development of mast cells. Mastocytosis is classified into two main groups, namely, cutaneous and systemic mastocytosis, based on the site of mast cell accumulation. In cutaneous mastocytosis, the cells purely gather in the skin. In contrast, systemic mastocytosis must affect an internal organ, including the bone marrow, lymph nodes, liver, spleen, and/or the gastrointestinal tract with or without skin involvement...
January 2024: Curēus
https://read.qxmd.com/read/38248039/challenges-in-the-diagnosis-of-cutaneous-mastocytosis
#3
REVIEW
Knut Brockow, Rebekka Karolin Bent, Simon Schneider, Sophie Spies, Katja Kranen, Benedikt Hindelang, Zsuzsanna Kurgyis, Sigurd Broesby-Olsen, Tilo Biedermann, Clive E Grattan
BACKGROUND: Mastocytosis is characterized by an accumulation of clonal mast cells (MCs) in tissues such as the skin. Skin lesions in mastocytosis may be clinically subtle or heterogeneous, and giving the correct diagnosis can be difficult. METHODS: This study compiles personal experiences together with relevant literature, discussing possible obstacles encountered in diagnosing skin involvement in mastocytosis and cutaneous mastocytosis (CM). RESULTS: The nomenclature of the term "CM" is ambiguous...
January 11, 2024: Diagnostics
https://read.qxmd.com/read/38243867/mastocytosis-in-the-skin-in-dogs-a-multicentric-case-series
#4
JOURNAL ARTICLE
Eleanor K Wyatt, Verena Affolter, Stefano Borio, Alexandra Guillen, Sara Verganti, Sue Murphy, Damiano Ballarini, Frane Banovic, Vanessa Schmidt, Jean-Benoit Tanis
Canine cutaneous mastocytosis (CM) is rare in contrast to canine mast cell tumours. In humans, CM commonly affects children and is usually indolent with possible spontaneous resolution. Systemic mastocytosis (SM) with bone marrow involvement typically affects adults, can have a poor outcome, and often includes skin lesions. 'Mastocytosis in the skin' (MIS) is the preferred term of skin lesions, if bone marrow evaluations are not available, which is often the cases in dogs. In human SM and CM, KIT mutations are often detected...
January 20, 2024: Veterinary and Comparative Oncology
https://read.qxmd.com/read/38111510/urticaria-pigmentosa-and-systemic-mastocytosis
#5
Jonathan Keow, Benjamin Chin-Yee, Cyrus C Hsia, Kara Robertson
KEY CLINICAL MESSAGE: Additional investigations for systemic involvement should be initiated once the diagnosis of cutaneous mastocytosis has been established in an adult patient. A serum tryptase can serve as a screening test for systemic mastocytosis, and persistent elevations should prompt further investigations, such as bone marrow studies. ABSTRACT: Urticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis, presenting as a wide variety of macroscopic appearances...
December 2023: Clinical Case Reports
https://read.qxmd.com/read/38076241/the-realistic-positioning-of-uva1-phototherapy-after-25%C3%A2-years-of-clinical-experience-and-the-availability-of-new-biologics-and-small-molecules-a-retrospective-clinical-study
#6
JOURNAL ARTICLE
Piergiacomo Calzavara-Pinton, Luca Bettolini, Francesco Tonon, Mariateresa Rossi, Marina Venturini
BACKGROUND: Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers. OBJECTIVE: To analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center. METHODS: We reviewed the medical files of 740 patients treated between 1998 and 2022...
2023: Frontiers in Medicine
https://read.qxmd.com/read/37222130/cutaneous-mastocytosis-in-8-young-dogs-and-review-of-literature
#7
JOURNAL ARTICLE
Ching Yang, Charles W Bradley, Diane Preziosi, Elizabeth A Mauldin
Cutaneous mastocytosis (CM) is a rare condition in young dogs characterized by multicentric cutaneous proliferation of neoplastic mast cells. Clinical data from 8 dogs that met inclusion criteria (age of onset less than 1.5 years, greater than 3 lesions) were obtained via a standardized survey. Biopsy samples were classified by the Kiupel/Patnaik grading systems and analyzed for c-KIT mutations. The median age of onset was 6 months (range: 2-17 months). Dogs had 5 to more than 50 lesions characterized as nodules, plaques, and papules...
May 24, 2023: Veterinary Pathology
https://read.qxmd.com/read/37140636/-mastocytosis-in-children
#8
REVIEW
Hanna Wassmer, Karin Hartmann
Mastocytosis in children is a rare disease characterized by an abnormal accumulation of tissue mast cells. Mastocytosis in children presents with typical skin alterations that are classified as maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis, or mastocytoma. Some patients also develop mast cell mediator symptoms, such as pruritus, flush, and anaphylaxis. In many children, the disease is characterized by a benign and usually self-limiting course; systemic mastocytosis with extracutaneous involvement and a chronic or progressive course is found only rarely...
May 2023: Dermatologie (Heidelb)
https://read.qxmd.com/read/36807903/mastocytosis-in-children-a-single-center-long-term-follow-up-study
#9
JOURNAL ARTICLE
Svetlana Popadic, Jovan Lalosevic, Branislav Lekic, Mirjana Gajić-Veljic, Branka Bonaci-Nikolic, Milos Nikolic
BACKGROUND: Mastocytosis is a heterogeneous group of rare disorders characterized by the accumulation of clonal mast cells in organs such as the skin and bone marrow. The diagnosis of cutaneous mastocytosis (CM) is based on clinical findings, positive Darier's sign, and histopathology, if necessary. METHODS: Medical records of 86 children with CM diagnosed during a 35-year long period were reviewed. Most patients (93%) developed CM during the first year of life (median age 3 months)...
February 20, 2023: International Journal of Dermatology
https://read.qxmd.com/read/36785740/delayed-diagnosis-of-indolent-systemic-mastocytosis-as-the-cause-of-unexplained-skin-rash-a-case-report
#10
Awni Alshurafa, Mohammad Abu-Tineh, Feryal A Ibrahim, Mahir Petkar, Mohamed A Yassin
Mastocytosis is a heterogeneous group of disorders in which mast cells exhibit clonal proliferation that infiltrates one or more organs. In cutaneous mastocytosis, the mast cells infiltrate the skin only, whereas systemic mastocytosis is diagnosed when at least one extra-cutaneous site is involved, with or without the skin being affected. Given the rarity of mastocytosis and the fact that skin rash can be a manifestation of different conditions and many clinicians are not familiar with this disorder, an accurate diagnosis may be delayed...
2023: Case Reports in Oncology
https://read.qxmd.com/read/36654513/clonal-mast-cell-disorders-and-hereditary-%C3%AE-tryptasemia-as-risk-factors-for-anaphylaxis
#11
REVIEW
Mark Kačar, Matija Rijavec, Julij Šelb, Peter Korošec
The association between Hymenoptera venom-triggered anaphylaxis (HVA) and clonal mast cell-related disorders (cMCD) has been known for decades. However, recent breakthroughs in peripheral blood screening for KIT p.D816V missense variant have revealed the true extent of this clinical association whilst adding to our understanding of the underlying aetiology. Thus, recent large studies highlighted the presence of KIT p.D816V among 18.2% and 23% of patients with severe Hymenoptera venom-triggered anaphylaxis. A significant proportion of those patients have normal serum basal tryptase (BST) levels, with no cutaneous findings such as urticaria pigmentosa or other systemic findings such as organomegaly that would have suggested the presence of cMCD...
January 18, 2023: Clinical and Experimental Allergy
https://read.qxmd.com/read/36578756/infantile-urticaria-pigmentosa-with-eosinophilic-infiltration-a-case-report
#12
JOURNAL ARTICLE
Masato Ishikawa, Yuka Hanami, Toshiyuki Yamamoto
No abstract text is available yet for this article.
2022: Indian Journal of Dermatology
https://read.qxmd.com/read/36569132/the-efficacy-of-adding-oral-sodium-cromoglycate-to-stable-treatment-for-controlling-bullous-pemphigoid-related-pruritus-a-retrospective-study
#13
JOURNAL ARTICLE
Noy Keller Rosenthal, Darby Boucher, Dedee F Murrell
INTRODUCTION: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease which mainly affects the elderly. It manifests as severe pruritus, urticarial plaques, and tense bullae and is associated with significant mortality. Therapy options for itch in BP patients are limited, mainly because the pathogenesis of itch in BP remains unclear. Sodium cromoglycate was commonly used in the past as an inhaled drug for the management of bronchial asthma and as an oral treatment for children with urticaria pigmentosa...
2022: Frontiers in Medicine
https://read.qxmd.com/read/36099235/dental-treatment-of-a-seven-year-old-child-with-mastocytosis
#14
JOURNAL ARTICLE
Filomeni Anagnostou, Zoi Daskalaki, Athina Chatzigianni, Konstantinos N Arapostathis
AIM: Mastocytosis is a rare disorder characterized by pathologic mast cell degranulation. This paper aims to present the dental management of a seven-year-old girl diagnosed with mastocytosis, in a hospital setting, yet without general anesthesia. CASE REPORT: A seven-year-old girl, diagnosed with urticaria pigmentosa, was referred to a dental practice. Her parents' main concern was a possible allergic reaction to local anesthetics and antibiotics. It was decided to perform treatment in the operating theatre of a clinic without general anesthesia...
July 1, 2022: Journal of Clinical Pediatric Dentistry
https://read.qxmd.com/read/36012900/dermoscopic-features-of-different-forms-of-cutaneous-mastocytosis-a-systematic-review
#15
REVIEW
Martyna Sławińska, Agnieszka Kaszuba, Magdalena Lange, Roman J Nowicki, Michał Sobjanek, Enzo Errichetti
The term mastocytosis refers to a heterogeneous group of disorders characterised by accumulation of clonal mast cells in different organs, most commonly in the skin. Little is known about the role of dermoscopy in the diagnostics of mastocytosis. To date, no systematic review on the dermoscopic features of cutaneous mastocytosis has been performed. The aim of this study was to summarise the current knowledge in the field as well as to identify the knowledge gaps to show possible directions for further studies, based on a systematic search of PubMed, Scopus, and Web of Science databases and related references published before 3 January 2022...
August 9, 2022: Journal of Clinical Medicine
https://read.qxmd.com/read/35991727/delayed-diagnosis-of-adult-onset-mastocytosis
#16
Annia Cavazos, Paul Subrt, Jaime A Tschen
Urticaria pigmentosa is most frequently observed in children. The associated symptomatology in mastocytosis is vague and not always diagnostic, and Darier's sign is often negative, making the diagnosis challenging. We describe a case of a 61-year-old man with extensive brown papules and macules in different locations of the body that slowly progressed in the last 10 years with a previous anaphylactic reaction after an ant bite. This suggests that urticaria pigmentosa in an adult should be furthered assessed for the possibility of systemic involvement for a correct diagnosis and treatment...
2022: Proceedings of the Baylor University Medical Center
https://read.qxmd.com/read/35656251/clinical-and-genetic-characteristics-of-ectodermal-dysplasia-in-four-indian-children
#17
Divya Kamat, Rahul Mahajan, Debajyoti Chatterjee, Jaivinder Yadav, Rakesh Kumar, Devi Dayal, Dipankar De, Sanjeev Handa
Introduction: Ectodermal dysplasias (EDs) affect structures derived from the ectoderm such as skin, its appendages, nail, and teeth. In this series, we describe four patients presenting with a clinical phenotype of dysplasia of one or more ectodermal structures who underwent next-generation sequencing for mutational analysis. Case Series: The clinical phenotype of three patients was hypohidrotic ectodermal dysplasia (HED) and one patient was diagnosed with autoimmune polyglandular syndrome (APS) type 1...
January 2022: Indian Journal of Dermatology
https://read.qxmd.com/read/35613833/treatment-of-capnocytophaga-sputigena-meningitis-in-a-neurosurgical-patient
#18
JOURNAL ARTICLE
Terry John Evans, Samitha Fernando, Micaela Uberti, Andrew J Martin, Marina Basarab
A woman in her 50s developed meningitis following an endoscopic, endonasal resection of a clival meningioma which was complicated by a cerebrospinal fluid (CSF) leak through the nose. CSF analysis showed a raised white cell count, and Capnocytophaga sputigena was isolated. This organism is an oral commensal and is implicated in periodontal disease; the CSF leak explains the portal of entry. C. sputigena is rarely isolated, and this is the first report of a central nervous system (CNS) infection caused by this organism...
May 25, 2022: BMJ Case Reports
https://read.qxmd.com/read/35412687/validation-of-dermatopathological-criteria-to-diagnose-cutaneous-lesions-of-mastocytosis-importance-of-kit-d816v-mutation-analysis
#19
JOURNAL ARTICLE
J Gebhard, H-P Horny, T Kristensen, S Broesby-Olsen, A Zink, T Biedermann, K Brockow
BACKGROUND: Cutaneous lesions of mastocytosis (CLM) are often subtle and may require biopsy. However, dermatohistopathological criteria for CLM remain undefined. OBJECTIVES: To establish criteria for CLM by validating histological and molecular parameters. METHODS: In skin samples from Caucasian patients with CLM and controls (atopic dermatitis, chronic urticaria, pruritus, tissue from tumor safety margin excisions), mast cell (MC) numbers, size, shape, distribution, immunostainability with a large panel of markers, pigmentation and presence of KIT D816V mutation were analysed...
August 2022: Journal of the European Academy of Dermatology and Venereology: JEADV
https://read.qxmd.com/read/35028497/cutaneous-mastocytosis-in-childhood
#20
REVIEW
Katja Nemat, Susanne Abraham
Mastocytoses are characterized by clonal proliferation of mast cells in various tissues. In childhood, cutaneous mastocytosis (CM) occurs almost exclusively. It is confined to the skin, and has a good prognosis. The most common form is the maculopapular cutaneous mastocytosis (MPCM), formerly called urticaria pigmentosa. A distinction is made between a monomorphic variant of MPCM with multiple small, roundish maculopapular skin lesions and the - more common - polymorphic variant with larger lesions of variable size...
2022: Allergologie Select
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