Subject(s)
Hair Follicle , Hair Removal/adverse effects , Patch Tests/adverse effects , Urticaria/etiology , Female , Humans , Middle AgedSubject(s)
Dermatitis, Allergic Contact/diagnosis , Dermatitis, Occupational/diagnosis , Pressure/adverse effects , Protective Clothing/adverse effects , Shoes/adverse effects , Skin Diseases, Vesiculobullous/diagnosis , Urticaria/diagnosis , Dermatitis, Allergic Contact/etiology , Dermatitis, Occupational/etiology , Diagnosis, Differential , Humans , Male , Middle Aged , Skin Diseases, Vesiculobullous/etiology , Urticaria/etiologySubject(s)
Dermatitis, Allergic Contact/etiology , Dermatitis, Exfoliative/chemically induced , Psoriasis/drug therapy , Quinolines/adverse effects , Adrenal Cortex Hormones/therapeutic use , Dermatitis, Allergic Contact/drug therapy , Dermatitis, Exfoliative/drug therapy , Humans , Male , Middle Aged , Patch Tests , Plant Oils/adverse effects , Psoriasis/complicationsABSTRACT
BACKGROUND: Systemic mast cell activation disorders (MCADs) are characterized by severe and systemic mast cell (MC) mediators-related symptoms frequently associated with increased serum baseline tryptase (sBt). OBJECTIVE: To analyze the clinical, biological, and molecular characteristics of adult patients presenting with systemic MC activation symptoms/anaphylaxis in the absence of skin mastocytosis who showed clonal (c) versus nonclonal (nc) MCs and to provide indication criteria for bone marrow (BM) studies. METHODS: Eighty-three patients were studied. Patients showing clonal BM MCs were grouped into indolent systemic mastocytosis without skin lesions (ISMs(-); n = 48) and other c-MCADs (n = 3)-both with CD25(++) BM MCs and either positive mast/stem cell growth factor receptor gene (KIT) mutation or clonal human androgen receptor assay (HUMARA) tests-and nc-MCAD (CD25-negative BM MCs in the absence of KIT mutation; n = 32) and compared for their clinical, biological, and molecular characteristics. RESULTS: Most clonal patients (48/51; 94%) met the World Health Organization criteria for systemic mastocytosis and were classified as ISMs(-), whereas the other 3 c-MCAD and all nc-MCAD patients did not. In addition, although both patients with ISMs(-) and patients with nc-MCAD presented with idiopathic and allergen-induced anaphylaxis, the former showed a higher frequency of men, cardiovascular symptoms, and insect bite as a trigger, together with greater sBt. Based on a multivariate analysis, a highly efficient model to predict clonality before BM sampling was built that includes male sex (P = .01), presyncopal and/or syncopal episodes (P = .009) in the absence of urticaria and angioedema (P = .003), and sBt >25 microg/L (P = .006) as independent predictive factors. CONCLUSIONS: Patients with c-MCAD and ISMs(-) display unique clinical and laboratory features different from nc-MCAD patients. A significant percentage of c-MCAD patients can be considered as true ISMs(-) diagnosed at early phases of the disease.
