Premium
Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine
Author(s) -
Lock A. D.,
McNamara C. J.,
Rustin M. H. A.
Publication year - 2015
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/ced.12488
Subject(s) - systemic mastocytosis , cladribine , urticaria pigmentosa , medicine , cutaneous mastocytosis , tryptase , dermatology , bone marrow , tyrosine kinase inhibitor , cd117 , rash , immunology , mast cell , stem cell , cd34 , cancer , biology , genetics
Summary Systemic mastocytosis ( SM ) is a myeloproliferative disorder, characterized by a clonal proliferation of abnormal mast cells accumulating in internal organs and sometimes in the skin, leading to cutaneous and systemic symptoms. Mutations within the gene KIT , which encodes the receptor tyrosine kinase ( KIT ) on mast cells, is found in most patients with SM . We report a case of a 62‐year‐old woman presenting with a pruritic rash on her limbs and trunk. Several years later she developed gastrointestinal symptoms, associated with raised serum tryptase. Skin and bone marrow biopsies confirmed a diagnosis of SM , initially presenting with urticaria pigmentosa. Responses to multiple therapies, including potent topical steroids, oral antihistamines, phototherapy and the tyrosine kinase inhibitor, nilotinib, were inadequate. Treatment with cladribine (2‐chlorodeoxyadenosine) produced a marked and sustained reduction in her symptoms and serum tryptase level.