Current allergy and asthma reports
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Mastocytosis is characterized by pathologic mast cell accumulation and activation in tissues. Establishment of objective histopathologic and molecular criteria for diagnosis of mastocytosis has allowed sensitive detection of mast cells with aberrant features in patients presenting with suspected mast cell activation symptoms. ⋯ In vitro experiments, however, showed that mast cells carrying the D816V c-kit mutation were resistant to the prototypical tyrosine kinase inhibitor imatinib. Efficacy of newer generation tyrosine inhibitors in mast cell disease is currently being evaluated.
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Since their first descriptions in 1922 and 1948, respectively, Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) have become recognized as manifestations--with different severity--of the same disease process along a spectrum of illness. Even today, decades after their description, there is still disagreement about when a particular bullous disease evolves from erythema multiforme to SJS/TEN. ⋯ Many cases are misdiagnosed, especially in their early stages. In this paper we address our current understanding of this disease spectrum and discuss both accepted and more controversial modes of therapy.