1/2. Occurrence of platelet-activating factor (PAF) and an endogenous inhibitor of platelet aggregation in diffuse cutaneous mastocytosis.We have identified PAF in the blister fluid from a patient with bullous mastocytosis, a rare form of mast-cell disease. We have found a novel endogenous inhibitor of platelet aggregation which obscured the presence of the PAF in unprocessed blister fluid and in ethanol or lipid extracts. The PAF was characterized by the demonstration of chromatographic, mass spectral and biological properties identical to those of authentic PAF. Thus this is the first demonstration of PAF in biological fluid from a patient with mastocytosis. High levels of immunoreactive prostaglandin d2 (PGD2) and histamine were also present in the blister fluid. The interaction between PAF and the inhibitor of platelet aggregation in patients with systemic mastocytosis may provide an explanation for some of the manifestations of the disease, in particular the episodic hypotension, cutaneous flushing and pallor.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/2. Bullous urticaria pigmentosa.urticaria pigmentosa is the most common manifestation of cutaneous mastocytosis. Three-quarters of all cases occur during infancy or early childhood. Occasionally, vesicles or bullae may appear in persons with urticaria pigmentosa. We describe an unusual case of bullous urticaria pigmentosa with prominent scalp involvement. prognosis and therapy are reviewed.- - - - - - - - - - ranking = 0.14285714285714keywords = mastocytosis (Clic here for more details about this article) |