Visual Guide to Skin Lesions in Mast Cell DiseasesThe following pages are a photo journal of examples of how mast cell diseases can present. A majority of the pictures are of skin manifestations of mastocytosis. 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.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. It should be noted that the formerly used term “UP” encompasses a variety of clinical manifestations. 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.Pic. 1- Female adult with smoldering systemic mastocytosis and maculopapular cutaneous lesions, monomorphic type associated with this disease (formerly known as urticaria pigmentosa or UP)Pic. 2- Female adult athlete with maculopapular cutaneous lesions, monomorphic type (formerly known as urticaria pigmentosa or UP), during a flare when the lesions are swellingPic. 3- Female child with cutaneous mastocytosis and characteristic maculopapular, polymorphic skin lesions (formerly known as urticaria pigmentosa or UP)Pic. 4- Female child with cutaneous mastocytoma on shoulder, which can present with an elevated lesion which is red or tannish brownPic. 5- Female adult with indolent systemic mastocytosis and confluent maculopapular, cutaneous lesions, monomorphic types (formerly known as urticaria pigmentosa or UP)Pic. 6- Male child with cutaneous mastocytosis with polymorphic lesions and other rashesPic. 7- Female adult with smoldering systemic mastocytosis (SSM), and typical maculopapular, cutaneous lesions, monomorphic type (formerly called urticaria pigmentosa or UP) during a flarePic. 8- Male child with cutaneous mastocytosis, characterized by maculopapular cutaneous lesions, polymorphic type (formerly known as urticaria pigmentosa or UP). Note that in some children, during a flare in response to a trigger, lesions may become bullous or blistered.Pic. 9- Male child with cutaneous mastocytosis during flare causing blisters in his maculopapular cutaneous lesionsPic. 10- Male child with mast cell activation syndrome, during flushing episodePic. 11- Male child with the maculopapular cutaneous lesions, polymorphic type, consistent with cutaneous mastocytosis (formerly called urticaria pigmentosa or UP)Pic. 12- Adult female with maculopapular, cutaneous lesions, monomorphic type during a flarePic. 13- Cutaneous mastocytoma, normal and inflamedPic. 14- Female child with maculopapular, polymorphic lesions of cutaneous mastocytosisPic. 15- Female with idiopathic anaphylaxis, hives (urticaria) and dermatographismFor further descriptions of Cutaneous Mastocytosis Variants, please click HERE.For more information on skin manifestations in mastocytosis (including a large selection of photos) and to review the source of our website’s descriptions of cutaneous mastocytosis variants, please see the following full text article, which is freely available online:Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 2016 Jan;137(1):35-45.
