1/14. Telangiectasia macularis eruptiva perstans.Telangiectasia macularis eruptiva perstans (TMEP) is a form of mastocytosis. It is an uncommon condition, particularly in children. The disorder is characterized by telangiectatic macules and generally has a good prognosis, with little tendency to urticate or cause constitutional symptoms. We report a girl who presented at age 2 with a 6-month history of erythematous macules on her arms and legs. The lesions were larger and possessed a more angular configuration than classic telangiectases. They slowly spread to her torso and face; there were no associated constitutional symptoms. Histopathologic examination showed dilated capillaries and increased numbers of mast cells, consistent with TMEP. mastocytosis should be considered in patients presenting with telangiectatic lesions that are atypical in appearance, distribution, or onset.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/14. Telangiectasia macularis eruptiva perstans presented as a pseudoallergic food reaction.Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis observed in less than 1% of cases of mastocytosis. Clinically, anaphylaxis may appear as a result of increased mast cell degranulation in different circumstances. A case of TMEP presented as pseudoallergic reactions to foods is reported in which the appearance of typical lesions on the trunk and their histological analysis together with a negative food allergy study confirmed the diagnosis.- - - - - - - - - - ranking = 2keywords = mastocytosis (Clic here for more details about this article) |
3/14. Telangiectasia macularis eruptiva perstans and multiple myeloma.The association of mast cell diseases and some hematologic malignancies, usually myeloproliferative disorders, myelodysplastic syndromes, and acute leukemia is well recognized. We report the case of a patient with telangiectasia macularis eruptiva perstans, a rare form of cutaneous mastocytosis, and multiple myeloma, an association that has been described only twice in the literature. Parallel improvement of both conditions was observed under chemotherapy regimens for multiple myeloma. Pathogenesis remains unclear, although the abnormalities in the c-kit pathway may play a role in the proliferation of cells from both lineages.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
4/14. Familial cutaneous mastocytosis.Cutaneous mastocytosis appears to occur sporadically; however, familial inheritance has been reported in 50 families since the mid-1880s. We report four cases of telangiectasia macularis eruptiva perstans (TMEP) occurring in three generations of a family. Whereas most patients with TMEP manifest in adulthood, all of the members of this family developed initial lesions during childhood. This is the third documented instance of familial mastocytosis affecting members of three generations, and the first report of familial TMEP. Although the inheritance pattern is unknown, the presentation of disease in this family further supports the hypothesis of an autosomal dominant mode of transmission with incomplete penetrance.- - - - - - - - - - ranking = 6keywords = mastocytosis (Clic here for more details about this article) |
5/14. Telangiectasia macularis eruptiva perstans in polycythemia rubra vera.We describe a 65-year-old Japanese man with a 20-year history of telangiectasia macularis eruptiva perstans, who developed polycythemia rubra vera and duodenal ulcer 10 and 12 years respectively after the onset of mastocytosis. Involvement of mast cells was found in neither bone marrow nor gastrointestinal tract. Immunohistochemical staining revealed that the mast cell was positive for both tryptase and chymase, indicating the nature of cutaneous mast cells. Despite the coexistence of a hematologic disorder, our case is suggested to have cutaneous but not systemic mastocytosis presenting as telangiectasia macularis eruptiva perstans.- - - - - - - - - - ranking = 2keywords = mastocytosis (Clic here for more details about this article) |
6/14. Presentation of telangiectasia macularis eruptiva perstans as a long-standing solitary plaque associated with renal carcinoma.BACKGROUND: mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. OBJECTIVE AND methods: We report a case of TMEP with an unusual clinical presentation as a solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. CONCLUSIONS: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
7/14. Systemic mastocytosis treated with histamine H1 and H2 receptor antagonists.A case of systemic mastocytosis with skin characteristics of telangiectasia macularis eruptiva perstans is reported. Systemic involvement was confirmed as a combination of highly increased urinary excretion of methyl imidazole acetic acid (Melm AA) and increased amounts of mast cells in skin, liver and colon transversum. Treatment with cimetidine (H2 receptor antagonist) resulted in diarrhea, however, the addition of cyproheptadine (H1 receptor antagonist) was beneficial in amelioration of the cutaneous symptoms of mastocytosis. The excretion of Melm AA was unchanged during treatment. After 7 months of continuous cimetidine and cyproheptadine treatment no side effects were observed.- - - - - - - - - - ranking = 6keywords = mastocytosis (Clic here for more details about this article) |
8/14. Telangiectasia macularis eruptiva perstans with an associated myeloproliferative disorder.Telangiectasia macularis eruptiva perstans (TMEP) is a cutaneous form of mastocytosis. It has been rarely associated with an underlying myeloproliferative disorder. We report the case of a patient, while receiving treatment for thrombocytosis, with both platelet production and function inhibitors presented with TMEP. TMEP is often refractory to therapy; however, our patient responded to treatment with PUVA.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
9/14. Telangiectasia macularis eruptiva perstans.A 45-year-old man presented with lesions clinically and histologically consistent with telangiectasis macularis eruptiva perstans (TMEP). TMEP is a rare form of mastocytosis, which is localized to the skin, although systemic involvement has been reported. A mutation in the c-kit proto-oncogene is thought to be involved in mast cell hyperplasia in some patients with mastocytosis. Treatment depends on the presence of systemic involvement or clinical symptoms. In case reports, therapeutic benefit has been reported in TMEP with the use of the 585-flashlamp pumped dye lasers and of electron beam radiation.- - - - - - - - - - ranking = 2keywords = mastocytosis (Clic here for more details about this article) |
10/14. Two cases of telangiectasia macularis eruptiva perstans demonstrated by immunohistochemistry for c-kit (CD 117).Telangiectasia macularis eruptiva perstans (TMEP) is an uncommon form of cutaneous mastocytosis that occurs exclusively in adults. Histologically, TMEP presents with scattered mast cells lined up around the dilated capillaries and venules of the superficial vascular plexus. In some cases, the number of mast cells falls within the range observed in normal skin and therefore cannot be detected by routine histologic examination. We used immunohistochemical staining for c-kit (CD 117) for the definitive diagnosis in two patients with TMEP. One of them was successfully treated with topical application of pimecrolimus.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
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