2011
DOI: 10.1182/blood.v118.21.5176.5176
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Telangiectasia Macularis Eruptiva Perstans: Report of Three Cases

Abstract: 5176 Introduction Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis seen commonly in adults. TMEP may show systemic manifestations and may be associated with myelodysplasia, myeloproliferative disorders, acute myeloid leukemia, and/or lymphoproliferative disease, but is not well recognized by hematologists. We present two cases of this rare but fascinating disease that illustrate the wide range of associated findings t… Show more

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“…Although UP is generally well‐recognized when presenting with its classical appearance, unusual macroscopic presentations of cutaneous mastocytosis are often misdiagnosed given the potential variability in clinical presentation and subtlety with establishing the histological diagnosis in the context of a low pre‐test probability. 14 Adult patients with UP often have a high likelihood of an underlying systemic mast cell disorder. 15 One case series described systemic involvement in up to 47% of cases with cutaneous mastocytosis 16 with aggressive (non‐indolent) systemic mastocytosis in up to 9% of those cases.…”
Section: Discussionmentioning
confidence: 99%
“…Although UP is generally well‐recognized when presenting with its classical appearance, unusual macroscopic presentations of cutaneous mastocytosis are often misdiagnosed given the potential variability in clinical presentation and subtlety with establishing the histological diagnosis in the context of a low pre‐test probability. 14 Adult patients with UP often have a high likelihood of an underlying systemic mast cell disorder. 15 One case series described systemic involvement in up to 47% of cases with cutaneous mastocytosis 16 with aggressive (non‐indolent) systemic mastocytosis in up to 9% of those cases.…”
Section: Discussionmentioning
confidence: 99%
“…Microscopically, TMEP characteristically reveals scanty ovoid to spindle shaped mast cells in papillary dermis that can be ignored by the pathologist in the absence of clinical suspicion. Special stains such as Giemsa and toluidine blue stains, as well as CD117 immunohistochemistry, are useful for highlighting the mast cells …”
Section: Discussionmentioning
confidence: 99%
“…To our knowledge, this is the first reported case of highly vascularized MPCM presenting solely in the palms and soles and successfully treated with topical PUVA. Three reports have described cases with lesions present solely in the palms, while in another case, lesions started in the face and extended to the chest, arms, and hands including palms and fingers . Another report described a case with lesions starting in the hands and feet and extending to the entire body…”
mentioning
confidence: 99%