Mast cells have been linked to rheumatoid arthritis (RA) and are essential to the pathogenesis of RA-like disease in a mouse model. We describe a 34-year-old woman who developed Sjögren's syndrome concurrently with telangiectasia macularis eruptiva perstans (TMEP), a rare form of cutaneous mastocytosis. The patient had sicca symptoms with an abnormal minor salivary gland biopsy and decreased salivary flow, peripheral neuropathy, an 80 pound weight loss, and a macular erythematous rash that exhibited superficial perivascular mast cell infiltrates on biopsy of lesional skin. This case further underscores the link between mast cells and the development of autoimmunity.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Exploring Pediatric Dermatology in Skin of Color: Focus on Dermoscopy.
Life (Basel)
December 2024
Institute of Dermatology, Department of Medical Area, University of Udine, 33100 Udine, Italy.
This literature review aims to comprehensively evaluate the clinical and dermoscopic presentations of common pediatric diseases among children with skin of color (SoC) while also addressing potential variations based on racial backgrounds. This review encompasses various conditions, such as nevi subtypes, viral infections, infestations, and inflammatory dermatoses, as well as hair diseases and abnormal vascular formations, occurring in pediatric populations. Overall, we identified 7 studies on nevi subtypes, 24 studies on skin infections, 6 on inflammatory dermatoses, 10 on hair diseases and disorders, and 14 on miscellaneous disorders that also satisfied our SoC- and race-specific criteria.
View Article and Find Full Text PDFEvaluation of allergic diseases in children diagnosed with cutaneous mastocytosis.
Allergy Asthma Proc
January 2025
Department of Pediatric Allergy and Immunology, Health Sciences University, Ankara City Hospital, Ankara, Turkey.
Cutaneous mastocytosis (CM) is the most common type of mastocytosis in children. The atopy frequency in these patients is typically similar to that in the general population, but a higher incidence of anaphylaxis is reported. This study aimed to evaluate the presence of allergic diseases in children diagnosed with CM and its impact on clinical manifestations.
View Article and Find Full Text PDFRecalcitrant Pustular Dermatosis Successfully Treated With Prolonged Isotretinoin Therapy.
Cureus
November 2024
Dermatology, Imperial Dermatology, Hollywood, USA.
Isotretinoin (13-cis-retinoic acid) is a well-established systemic treatment for moderate to severe acne vulgaris, renowned for its ability to target multiple contributors to acne pathogenesis. However, its therapeutic potential extends beyond conventional acne management. This case report highlights its efficacy in treating recalcitrant pustular dermatosis, a condition that proved resistant to standard therapies and posed significant diagnostic challenges.
View Article and Find Full Text PDFThe Mastocytosis Control Test: A Patient-Reported Outcome Measure Assessing Disease Control.
J Allergy Clin Immunol Pract
December 2024
Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Insititute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.
Background: Mastocytosis is characterized by expanding neoplastic mast cells in organs such as the skin, bone marrow, and gastrointestinal tract. The release of mast cell mediators triggers cutaneous, gastrointestinal, and other symptoms. Currently, no validated mastocytosis-specific patient-reported outcome measure (PROM) exists to assess disease control.
View Article and Find Full Text PDFMast cell sarcoma with KIT p.D816V mutation and concurrent systemic mastocytosis.
J Hematop
December 2024
Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, USA.
Mast cell sarcoma (MCS) is an extremely rare and aggressive form of mastocytosis characterized by highly atypical mast cells with local invasion, metastatic potential, and poor prognosis. MCS is predominantly a de novo process without recurrent molecular findings or predisposing lesions including various myeloid neoplasms. However, there have been rare case reports of MCS with preceding or concurrent systemic mastocytosis (SM) or cutaneous mastocytosis (CM), which is suggestive of an uncommon progression from SM/CM to MCS.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!