![]() 8- Male child with cutaneous mastocytosis, characterized by maculopapular cutaneous lesions, polymorphic type (formerly known as urticaria pigmentosa or UP). 7- Female adult with smoldering systemic mastocytosis (SSM), and typical maculopapular, cutaneous lesions, monomorphic type (formerly called urticaria pigmentosa or UP) during a flare 6- Male child with cutaneous mastocytosis with polymorphic lesions and other rashes 5- Female adult with indolent systemic mastocytosis and confluent maculopapular, cutaneous lesions, monomorphic types (formerly known as urticaria pigmentosa or UP) ![]() ![]() 4- Female child with cutaneous mastocytoma on shoulder, which can present with an elevated lesion which is red or tannish brown 3- Female child with cutaneous mastocytosis and characteristic maculopapular, polymorphic skin lesions (formerly known as urticaria pigmentosa or UP) 2- Female adult athlete with maculopapular cutaneous lesions, monomorphic type (formerly known as urticaria pigmentosa or UP), during a flare when the lesions are swelling 1- Female adult with smoldering systemic mastocytosis and maculopapular cutaneous lesions, monomorphic type associated with this disease (formerly known as urticaria pigmentosa or UP) In children, some of these varieties will fade away, some will develop into indolent systemic mastocytosis and some will evolve into a newly described entity called well-differentiated systemic mastocytosis. It should be noted that the formerly used term “UP” encompasses a variety of clinical manifestations. Most cases of childhood-onset mastocytosis fall into one of the cutaneous mastocytosis categories listed above and may or may not include symptoms of systemic mast cell activation as a result of mediators released from the skin. While cutaneous mastocytosis can include maculopapular cutaneous mastocytosis (MPCM), formerly known as urticaria pigmentosa (UP), telangiectasia macularis eruptiva perstans (TMEP), diffuse cutaneous mastocytosis (DCM), and cutaneous mastocytoma, skin manifestations can also occur in systemic mastocytosis (SM), mast cell activation syndrome (MCAS) and idiopathic anaphylaxis patients as well. A majority of the pictures are of skin manifestations of mastocytosis. The following pages are a photo journal of examples of how mast cell diseases can present. Visual Guide to Skin Lesions in Mast Cell Diseases
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