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Diagnosing Skin Disease
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
The unique accessibility of the skin allows for the utilization of a variety of tests that can be readily carried out in the clinical setting. Manipulation of the skin by a clinician is one such test that can yield useful diagnostic information. In the case of Darier’s sign, vigorous rubbing of the patient’s skin can support the diagnosis of mastocytosis when it results in significant swelling, itch, and erythema. The presence of Auspitz’s sign, which is the appearance of punctate bleeding after the scraping of scaly lesions, can be indicative of psoriasis. Additionally, the shearing of skin with rubbing, known as the Nikolsky sign, can be a clinically useful diagnostic finding in the evaluation of blistering skin disorders, such as pemphigus and toxic epidermal necrolysis.
Mastocytosis
Published in Dimitris Rigopoulos, Alexander C. Katoulis, Hyperpigmentation, 2017
The upper and lower extremities are most commonly affected, followed by the thorax and abdomen. In adults, sun-exposed areas, such as the palms, soles, face and scalp, generally remain free of lesions. In children, the face and scalp may be involved. Lesions on the head often exhibit a particularly prominent Darier’s sign. Pruritus associated with MPCM may be exacerbated by changes in temperature; exercise; hot showers; local friction; ingestion of hot beverages, spicy food, or ethanol; emotional stress; or certain drugs, such as nonsteroidal anti-inflammatory drugs (NSAIDs).
Contemporary Diagnosis and Management of Systemic Mastocytosis
Published in Richard T. Silver, Ayalew Tefferi, Myeloproliferative Disorders, 2007
Peter Valent, Cem Akin, Hans-Peter Horny, Dean D. Metcalfe
In CM, MC infiltration is confined to the skin (39,64). Most patients are children. By contrast, in most adults with skin lesions, a systemic variant of mastocytosis is diagnosed. Thus, the skin lesions of CM cannot be distinguished from that found in SM, either by macroscopic examination or by histology or molecular tests. Most patients with CM and most with SM have a characteristic maculopapular rash (39,64). In a smaller group of patients, usually those with CM, skin lesions are diffuse or nodular (39,64,65). A positive Darier’s sign (urtication on stroking of lesional skin) is a typical finding. Blistering of the skin may also be observed, but is unusual. The diagnosis of CM is thus based on typical skin lesions, a skin biopsy, and lack of SM criteria (50,51,69). The serum tryptase usually is below < 20 ng/mL. For further information about subtypes, prognosis, and the manage-ment of CM, we refer the reader to the available literature (39,64,69).
Mastocytosis and related entities: a practical roadmap
Published in Acta Clinica Belgica, 2023
Michiel Beyens, Jessy Elst, Marie-Line van der Poorten, Athina Van Gasse, Alessandro Toscano, Anke Verlinden, Katrien Vermeulen, Marie-Berthe Maes, J. N. G. Hanneke Oude Elberink, Didier Ebo, Vito Sabato
The diagnosis of CM relies mainly on recognition of (typical) skin lesions. A positive Darier’s sign serves as a major criterium. This involves a local wheal and flare reaction when lesions are stroked at moderate pressure. Darier’s sign differs from dermographism, since the latter also applies to nonlesional skin. Obviously, intake of antihistamines might result in a false-negative Darier’s sign. It is dissuaded to test Darier’s sign in patients with mastocytoma or the nodular variant of polymorphic MPCM as this can provoke flushing or even hypotension. However, this sign can be negative in adults with (cutaneous) mastocytosis but will often be positive in children [14]. The first two minor criteria are based on the skin biopsy. The first being an increased number (four- to eightfold) of MCs on histology. It is of note that the normal range value of MCs in skin is highly dependent on the site of biopsy and that some patients with CM do not have an increased number of MCs in the skin [15]. The second minor criterion is the presence of an (activating) KIT mutation in lesional skin tissue.
An evaluation of masitinib for treating systemic mastocytosis
Published in Expert Opinion on Pharmacotherapy, 2019
Mariarita Laforgia, Ilaria Marech, Patrizia Nardulli, Concetta Calabrò, Cosimo Damiano Gadaleta, Girolamo Ranieri
Lortholary et al. in a placebo-controlled phase 3 study [62] assessed safety and efficacy of masitinib in 135 severely symptomatic patients who were unresponsive to standard symptomatic treatments. By 24 weeks, masitinib was associated with a cumulative response, in at least one of four severe baseline symptoms of MC mediator release (pruritus, flushes, depression, or asthenia), of 19% compared with 7% for placebo (p = 0.0076). At week 24, the mean change of tryptase level from baseline in the modified ITT population was a decrease of 18% in the masitinib arm versus an increase of 2% in the placebo arm (p < 0.0001). The response to masitinib of urticaria pigmentosa lesions differed when compared with placebo (p = 0.0210) as evidenced by a decrease in average body surface area of 12% for masitinib versus an increase of 16% for placebo. The response to masitinib included one c-kit D816V-positive patient who had a complete response at week 24 (from baseline body surface area of 18%). This observation was also supported by the disappearance of Darier’s sign in 19% of patients treated with masitinib versus 3% treated with placebo (p = 0.0187).
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