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Urticaria and Angioedema
Published in Pudupakkam K Vedanthan, Harold S Nelson, Shripad N Agashe, PA Mahesh, Rohit Katial, Textbook of Allergy for the Clinician, 2021
Jenny M Stitt, Stephen C Dreskin
Three important conditions that are on the differential diagnosis of urticaria are urticarial vasculitis, urticaria pigmentosa and systemic mastocytosis. Urticarial vasculitis is characterized by hives that are typically painful or burning. The lesions can be non-blanching, typically last longer than 24 hours and often have associated ecchymosis, and resolve with residual hyperpigmentation of the skin or frank scar formation (Davis and Brewer 2004). Urticaria pigmentosa presents with brownish-red cutaneous lesions that become erythematous, pruritic and edematous after gentle stroking, called ‘Darier’s sign’. This physical stimulation activates the nest of mast cells that comprise the lesions of urticaria pigmentosa and is pathognomonic for urticaria pigmentosa (Brockow 2004). Of note, this sign may be absent if a patient is taking antihistamines. Systemic mastocytosis is a rare disorder of mast cell hyperplasia and proliferation. Mast cells may infiltrate the skin, bone marrow and other organs. Classification and severity are based in part on the extent of mast cell burden. Patients may have urticaria pigmentosa but also may experience episodic flushing, urticaria, angioedema, gastrointestinal distress, anaphylaxis and neuropsychiatric abnormalities (Lim et al. 2009, Metcalfe 2008).
Benign tumours, moles, birthmarks and cysts
Published in Ronald Marks, Richard Motley, Common Skin Diseases, 2019
This term is used to describe a group of disorders in which there may be excess mast cells in many tissues, but which is mainly manifest in the skin. The term urticaria pigmentosa was formerly employed because it is not uncommon for the individual lesions to become pigmented. In the juvenile form, numerous pink or red–brown papules develop over the trunk and limbs (Fig. 14.37). In some young patients, the lesions are intensely itchy and they experience discomfort and erythema when bathing. Juvenile mastocytosis usually remits spontaneously during adolescence (Fig. 14.38).
Mastocytosis
Published in Dongyou Liu, Tumors and Cancers, 2017
CM accounts for 90% of mastocytosis cases and may appear as maculopapular CM (urticaria pigmentosa), diffuse CM, or solitary mastocytoma. Urticaria pigmentosa lesions are usually reddish brown macules or papules. Diffuse CM lesions may manifest as diffuse xanthogranulomas similar to yellow-orange subcutaneous nodules or diffuse red bullae. A solitary mastocytoma has a single or multiple brown nodules, which may display flushing and systemic symptoms (e.g., hypotension after exposure to friction). Histopathologically, CM shows mast cell accumulation along the papillary dermis and reticular dermis and inside the subcutaneous adipose tissue, without bone marrow or other organ infiltration. Serum tryptase is usually not elevated in CM [7,8].
Recent advances in our understanding of mast cell activation – or should it be mast cell mediator disorders?
Published in Expert Review of Clinical Immunology, 2019
Theoharis C. Theoharides, Irene Tsilioni, Huali Ren
In this subcategory, affected skin most commonly presents as urticaria pigmentosa (UP) or diffuse CM, and less frequently as bullous CM or solitary mastocytoma [96,116]. Different from adults with CM, bone marrow biopsy is not recommended for children [117,118]. It is generally considered that pediatric CM regresses by puberty in most children [116], but there have not been any systematic longitudinal studies to prove this point. However, increased serum baseline total tryptase identifies children at risk for SM [119], as does the presence of maculopapular cutaneous mastocytosis [120] [121] or detection of KIT D816V in peripheral blood [122]. It is interesting that KIT D816V is not present in most skin lesions suggesting that there may be committed stem cells outside the bone marrow. In this case, the molecules triggering mast cell proliferation in the skin may be NGF [123], RANTES [124], or even CRH acting on precursors in the hair follicle mesenchyme [125]. It is interesting that new UP lesions in children often occur in areas of trauma.
Anti-TNF agent etanercept augments UV-induced skin cancer development in SKH-1 mice
Published in Journal of Dermatological Treatment, 2021
Ercan Calıskan, Mehmet Gamsızkan, Aslan Yurekli, Aysenur Botsali, Mehmet Esref Kabalar, Murat Demiriz, Mustafa Tunca
Another interesting observation in our study is the emergence of three mastocytosis lesions exclusively in the anti-TNF treatment groups. Throughout our literature search, we have encountered only one case report defining onset of urticaria pigmentosa after ETN treatment in an ankylosing spondylitis patient (23). Apart from immunosuppression, a specific explanation for the induction of mastocytosis lesions might be dysregulation of tumor necrosis factor-related apoptosis-inducing ligand receptor activity by anti-TNF treatments in mice with a vulnerable genetic background.
Delayed diagnosis of adult-onset mastocytosis
Published in Baylor University Medical Center Proceedings, 2022
Annia Cavazos, Paul Subrt, Jaime A. Tschen
Cutaneous mastocytosis is a rare disease, mostly manifested in adults as urticaria pigmentosa, diffuse and erythrodermic mastocytosis, and telangiectasia macularis eruptiva perstans. The affected skin is characterized by Darier’s sign. Extracutaneous involvement should be carefully considered in adults with cutaneous manifestations.1