Since starting omalizumab, he is no longer taking doxepin and has reduced his dose of Advair 250/50 μg to 100/50 μg (GlaxoSmithKline, Research Triangle Park, NC). At age 48 years, he had another ...severe episode of anaphylaxis requiring an intensive care unit admission. Since age 48 years, he has had 15 to 16 episodes of anaphylaxis per year and during these episodes has associated wheezing, vomiting, and hives, but no angioedema.
Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass localized and disseminated forms. Although a classification and criteria for cutaneous mastocytosis (CM) have ...been proposed, there remains a need to better define subforms of cutaneous manifestations in patients with mastocytosis. To address this unmet need, an international task force involving experts from different organizations (including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology) met several times between 2010 and 2014 to discuss the classification and criteria for diagnosis of cutaneous manifestations in patients with mastocytosis. This article provides the major outcomes of these meetings and a proposal for a revised definition and criteria. In particular, we recommend that the typical maculopapular cutaneous lesions (urticaria pigmentosa) should be subdivided into 2 variants, namely a monomorphic variant with small maculopapular lesions, which is typically seen in adult patients, and a polymorphic variant with larger lesions of variable size and shape, which is typically seen in pediatric patients. Clinical observations suggest that the monomorphic variant, if it develops in children, often persists into adulthood, whereas the polymorphic variant may resolve around puberty. This delineation might have important prognostic implications, and its implementation in diagnostic algorithms and future mastocytosis classifications is recommended. Refinements are also suggested for the diagnostic criteria of CM, removal of telangiectasia macularis eruptiva perstans from the current classification of CM, and removal of the adjunct solitary from the term solitary mastocytoma.
Maculopapular Cutaneous Mastocytosis in a Diverse Population Kirshenbaum, Arnold S; Abuhay, Hanna; Bolan, Hyejeong ...
The journal of allergy and clinical immunology in practice (Cambridge, MA),
11/2019, Letnik:
7, Številka:
8
Journal Article
Des macules pigmentées Schneider, C.; Granel-Brocard, F.; Seiller, H. ...
La revue de medecine interne,
June 2023, 2023-06-00, Letnik:
44, Številka:
6
Journal Article
Mastocytosis is a clonal disorder of mast cells. Systemic symptoms are typically a result of mast-cell mediator release. Urticaria Pigmentosa is the most common type of children mastocytosis. We ...report a case of a 9-month-old boy who presented for a widespread eruption since the age of 2 months. Physical examination revealed numerous red to brown slightely hyperpigmented papules and plaques on the trunk, the neck, the proximal upper and lower extremities and the genitalia. Complete blood count, hepatic and renal functions, abdominal ultrasonography were normal. A skin biopsy was performed and the histological exam confirmed the diagnosis of urticaria pigmentosa. The avoidance of chemical and physical stimuli was provided. He had only residual pigmentation at the lesional sites with no new lesion formation. The prognosis of childhood UP is good.
Urticaria pigmentosa in monochorionic twins Yavuz, Goknur; Yavuz, Ibrahim; Bilgili, Serap ...
Indian journal of dermatology, venereology, and leprology,
09/2019, Letnik:
85, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Mastocytosis, a disease characterized by clonal proliferation of mast cells in one or multiple organs, may lead to various clinical manifestations. Erythema and urticarial wheals induced by physical ...trauma (e.g., rubbing, friction) is known as Darier's sign,8 which was positive in our case. Since the course of the disease is benign, the main objective of the therapy is to reduce the release of mast cell mediators and to inhibit their effects, thereby controlling signs and symptoms. ...although familial cases of urticaria pigmentosa, diffuse cutaneous mastocytosis and mastocytomas have been reported, to our knowledge, this is the first report of urticaria pigmentosa seen in twins at the same time, which suggests that this form of mastocytosis is also genetically determined.
Urticaria Pigmentosa Without Pruritus Nahm, William J; Corriveau, Denise J; Mathe, Chris A ...
Journal of drugs in dermatology,
2024-Mar-01, Letnik:
23, Številka:
3
Journal Article
Recenzirano
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. J Drugs Dermatol. 2024;23(3): doi:10.36849/JDD.7558e.
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