Telangiectasia macularis eruptiva perstans

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Telangiectasia macularis eruptiva perstans
Other namesTMEP
SpecialtyMedical genetics Edit this on Wikidata

Telangiectasia macularis eruptiva perstans (TMEP) is persistent, pigmented, asymptomatic eruption of macules usually less than 0.5 cm in diameter with a slightly reddish-brown tinge.[1]: 616 [2]

Signs and symptoms[edit]

Small, irregular reddish-brown telangiectatic macules covering a tan to brown backdrop are the usual appearance of TMEP lesions.[3] The diameter of a single lesion is often between 2 and 4 mm.[4] During a diascopy, the telangiectatic lesions typically blanch. Lesions are usually non-pruritic and symmetrically distributed over the trunk and proximal extremities. Usually, the face, palms, and soles remain unharmed.[3] Darier's sign, a classic urticaria pigmentosa/maculopapular cutaneous mastocytosis finding, is usually negligible or absent in individuals with TMEP because the quantity of mast cells involved is comparatively lower.[4][5]

Causes[edit]

There have been reports of family clustering within families in certain TMEP cases.[6][7] Rare cases involving patients receiving radiation therapy, those suffering from malignant melanoma,[8] breast or kidney cancer,[9] or those with Sjogren's syndrome have also been reported.[10] Additional cases have been reported in individuals with leukemia[11] or myelofibrosis,[12][13] multiple myeloma,[14] and myeloproliferative diseases (including Polycythemia Rubra Vera).[15] Rare instances of urticaria pigmentosa and TMEP coexisting have also been reported.[16][17]

Diagnosis[edit]

Spindle-shaped mast cells found in skin biopsies validate the diagnosis of TMEP.[18] Histologically, TMEP is distinguished by a modest mononuclear infiltrate surrounding the superficial venous plexus capillaries that contains a considerable number of mast cells.[19] Special stains, such Giemsa or toluidine blue, are highly helpful in the diagnostic evaluation because they aid to highlight the presence of mast cells by exposing their metachromatic cytoplasmic granules.[20][21] TMEP lesions' dermoscopy reveals linear dots organized in a reticular pattern.[22]

Treatment[edit]

The presence of systemic involvement or clinical symptoms determines how individuals with TMEP are treated. There is no gold standard medication for the treatment of TMEP, thus it is critical to identify and avoid conditions that trigger mast cell destruction, such as sunshine, severe temperatures, alcohol, and narcotics. H1 antagonists are used to treat urticaria and pruritus. Psoralen combined with UV radiation by preventing mast cells from releasing histamine, improves symptoms and causes skin lesions to disappear.[19]

See also[edit]

References[edit]

  1. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. pp. 1845, 1848. ISBN 978-1-4160-2999-1.
  3. ^ a b Watkins, Casey E.; Bokor, Winston B.; Leicht, Stuart; Youngberg, George; Krishnaswamy, Guha (2011-07-29). "Telangiectasia Macularis Eruptiva Perstans: more than skin deep". Dermatology Reports. 3 (1). PAGEPress Publications: e12. doi:10.4081/dr.2011.e12. ISSN 2036-7406. PMID 25386256.
  4. ^ a b Wolff, Klaus; Komar, Michael; Petzelbauer, Peter (2001). "Clinical and histopathological aspects of cutaneous mastocytosis". Leukemia Research. 25 (7). Elsevier BV: 519–528. doi:10.1016/s0145-2126(01)00044-3. ISSN 0145-2126. PMID 11377676.
  5. ^ Vidal, C.; del Rio, E.; Suárez-Peñaranda, J.; Armisén, M. (2000). "Telangiectasia macularis eruptiva perstans presented as a pseudoallergic food reaction". Journal of Investigational Allergology & Clinical Immunology. 10 (4): 248–250. ISSN 1018-9068. PMID 11039843.
  6. ^ NERI, I.; GUARESCHI, E.; GUERRINI, V.; PATRIZI, A. (2005). "Familial Telangiectasia Macularis Eruptiva Perstans". Pediatric Dermatology. 22 (5). Wiley: 488–489. doi:10.1111/j.1525-1470.2005.00123.x. ISSN 0736-8046. PMID 16191009.
  7. ^ Chang, Anwell; Tung, Rebecca C.; Schlesinger, Todd; Bergfeld, Wilma F.; Dijkstra, Jacob; Kahn, Teri A. (2001). "Familial Cutaneous Mastocytosis". Pediatric Dermatology. 18 (4). Wiley: 271–276. doi:10.1046/j.1525-1470.2001.01939.x. ISSN 0736-8046. PMID 11576397.
  8. ^ Kowalzic, Lutz; Eickenscheidt, Lena; Seidel, Christoph; Kribus, Sabine; Ziegler, Heidrun; Komar, Michael (2009-03-30). "Telangiectasia macularis eruptiva perstans, a form of cutaneous mastocytosis, associated with malignant melanoma". JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 7 (4). Wiley: 360–362. doi:10.1111/j.1610-0387.2008.06941.x. ISSN 1610-0379. PMID 19054427.
  9. ^ Carlos Pascual, José; Bañuls, José; Albares, Ma Pilar; Vergara, Gloria; Belinchón, Isabel; Francisco Silvestre, Juan; Betlloch, Isabel (2003-10-01). "Presentation of Telangiectasia Macularis Eruptiva Perstans as a Long-Standing Solitary Plaque Associated with Renal Carcinoma". Journal of Cutaneous Medicine and Surgery: Incorporating Medical and Surgical Dermatology. 7 (5). SAGE Publications: 399–402. doi:10.1007/s10227-002-0144-z. ISSN 1203-4754. PMID 14973646.
  10. ^ Kalay Erbay, Shebnem; Stewart, Carol; Hassanein, Ashraf; Fletcher, Andrew; Bhattacharyya, Indraneel; Cohen, Donald; Wesson, Stanton K.; Weinstein, Jason; Lyons, Robert; Reeves, Westley H. (2006). "Cutaneous mastocytosis in a patient with primary Sjögren's syndrome". The Journal of Rheumatology. 33 (8): 1697–1700. ISSN 0315-162X. PMID 16881127.
  11. ^ Mahadeo, Kris M.; Wolgast, Lucia; McMahon, Christine; Cole, Peter D. (2011-02-04). "Systemic Mastocytosis in a Child With t(8;21) Acute Myeloid Leukemia". Pediatric Blood & Cancer. 57 (4). Wiley: 684–687. doi:10.1002/pbc.23026. ISSN 1545-5009. PMID 21671435.
  12. ^ Turchin, Irina; Barankin, Benjamin; Schloss, Eric (2006). "Unusual cutaneous findings of urticaria pigmentosa and telangiectasia macularis eruptiva perstans associated with marked myelofibrosis". International Journal of Dermatology. 45 (10). Wiley: 1215–1217. doi:10.1111/j.1365-4632.2006.02648.x. ISSN 0011-9059. PMID 17040445.
  13. ^ Martin, Lucy K.; Romanelli, Paolo; Ahn, Yeon Soong; Kirsner, Robert S. (2004-08-09). "Telangiectasia macularis eruptiva perstans with an associated myeloproliferative disorder". International Journal of Dermatology. 43 (12). Wiley: 922–924. doi:10.1111/j.1365-4632.2004.02249.x. ISSN 0011-9059. PMID 15569020.
  14. ^ Bachmeyer, Claude; Guillemette, Jacqueline; Blum, Laurent; Turc, Yves; Dhôte, Robin; Fermand, Jean-Paul; Aractingi, Selim (2000). "Telangiectasia macularis eruptiva perstans and multiple myeloma". Journal of the American Academy of Dermatology. 43 (5). Elsevier BV: 972–974. doi:10.1067/mjd.2000.109254. ISSN 0190-9622. PMID 11044837.
  15. ^ Suzuki, Kenji; Konishi, Noriko; Tokura, Yoshiki; Takigawa, Masahiro (2002). "Telangiectasia macularis eruptiva perstans in polycythemia rubra vera". European Journal of Dermatology: EJD. 12 (2): 201–203. ISSN 1167-1122. PMID 11872425.
  16. ^ Parks, A. (1988-03-01). "Reddish-brown macules with telangiectasia and pruritus. Urticaria pigmentosa-telangiectasia macularis eruptiva perstans (TMEP) variant, with systemic mastocytosis". Archives of Dermatology. 124 (3). American Medical Association (AMA): 429b–430. doi:10.1001/archderm.124.3.429b. ISSN 0003-987X. PMID 3345092.
  17. ^ Weber, F. Parkes (2024-03-14). "Telangiectasia Macularis Eruptiva Perstans—probably a telangiectatic variety of urticaria pigmentosa in an adult". Proceedings of the Royal Society of Medicine. 24 (2). Royal Society of Medicine Press: 96–97. doi:10.1177/003591573002400204. PMC 2182058. PMID 19987891.
  18. ^ Cengizlier, R.; Hücümenoğlu, S.; Özen, A.; Tülin Şayli, R. (2009). "Treatment of telangiectasia macularis eruptiva perstans with montelukast". Allergologia et Immunopathologia. 37 (6). Codon Publications: 334–336. doi:10.1016/j.aller.2009.03.010. ISSN 0301-0546. PMID 19913347.
  19. ^ a b Costa, Dailana Louvain Marinho; Moura, Heloisa Helena; Rodrigues, Rosangela; Pineiro-Maceira, Juan; Ramos-e-Silva, Marcia (2024-03-14). "Telangiectasia Macularis Eruptiva Perstans: A Rare Form of Adult Mastocytosis". The Journal of Clinical and Aesthetic Dermatology. 4 (10). Matrix Medical Communications: 52–54. PMC 3196299. PMID 22010057.
  20. ^ Gibbs, Neil F.; Friedlander, Sheila Fallon; Harpster, Elaine F. (2000). "Telangiectasia Macularis Eruptiva Perstans". Pediatric Dermatology. 17 (3). Wiley: 194–197. doi:10.1046/j.1525-1470.2000.01750.x. ISSN 0736-8046. PMID 10886750.
  21. ^ Sotiriou, Eleni; Apalla, Zoi; Ioannides, Demetris (2010). "Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy". Photodermatology, Photoimmunology & Photomedicine. 26 (1). Wiley: 46–47. doi:10.1111/j.1600-0781.2009.00480.x. ISSN 0905-4383. PMID 20070840.
  22. ^ Akay, Bengü Nisa; Kittler, Harald; Sanlı, Hatice; Harmankaya, Kaan; Anadolu, Rana (2008-12-06). "Dermatoscopic Findings of Cutaneous Mastocytosis". Dermatology. 218 (3). S. Karger AG: 226–230. doi:10.1159/000182260. ISSN 1018-8665. PMID 19060465.

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