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Telangiectasia macularis eruptiva perstans

MedGen UID:
78094
Concept ID:
C0263402
Disease or Syndrome
Synonyms: Paucicellular mastocytosis; Telangiectasia macularis eruptive perstans; Telangiectatic cutaneous mastocytosis
SNOMED CT: Telangiectasia macularis eruptiva perstans (8214000); TMEP - telangiectasia macularis eruptiva perstans (8214000); Paucicellular mastocytosis (8214000)
 
HPO: HP:0007583
Monarch Initiative: MONDO:0019576
Orphanet: ORPHA90389

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTelangiectasia macularis eruptiva perstans
Follow this link to review classifications for Telangiectasia macularis eruptiva perstans in Orphanet.

Conditions with this feature

Cutaneous mastocytosis
MedGen UID:
210143
Concept ID:
C1136033
Finding
Mastocytosis, or mast cell disease, is a heterogeneous group of clinical disorders characterized by the abnormal accumulation of mast cells in various tissues, especially in the skin and hematopoietic organs. Mastocytosis usually appears in infancy or early adulthood. In most pediatric cases, the disease is limited to the skin, but it can be associated with systemic symptoms due to the release of mediators from mast cells, even when there is no systemic infiltration. It usually has a good prognosis, with substantial improvement or spontaneous resolution before puberty. In rare cases, the disease may remain active through adolescence as a systemic adult mastocytosis. Cutaneous mastocytosis is characterized by macules, papules, nodules, or diffuse infiltration of the skin, often associated with localized hyperpigmentation. Gentle rubbing of the lesions induces histamine release from mechanically activated mast cells, causing local wheals, erythema, and often pruritus, a phenomenon termed the 'Darier sign.' In contrast to childhood-onset mastocytosis, adult-onset mastocytosis often persists for the lifetime of the patient and is also more likely to be a severe and systemic disease involving numerous organs. In some cases, it is associated with a clonal hematologic non-mast-cell lineage disease, such as a myelodysplastic or myeloproliferative disorder. Adult-onset mastocytosis can also lead to the rare mast cell leukemia, which carries a high risk of mortality (summary by Bodemer et al., 2010 and Kambe et al., 2010).

Recent clinical studies

Etiology

Michelerio A, Grassi S, Elena C, Croci GA, Boveri E, Bossi G, Brazzelli V
Eur J Dermatol 2019 Apr 1;29(2):174-178. doi: 10.1684/ejd.2019.3532. PMID: 30973331
Moon HR, Kim YJ, Jung JM, Won CH, Lee MW, Choi JH, Chang SE
J Dermatol 2017 Nov;44(11):1276-1280. Epub 2017 Jun 23 doi: 10.1111/1346-8138.13928. PMID: 28646532
Severino M, Chandesris MO, Barete S, Tournier E, Sans B, Laurent C, Apoil PA, Lamant L, Mailhol C, Laroche M, Fraitag S, Hanssens K, Dubreuil P, Hermine O, Paul C, Bulai Livideanu C
J Am Acad Dermatol 2016 May;74(5):885-91.e1. Epub 2016 Feb 19 doi: 10.1016/j.jaad.2015.10.050. PMID: 26899198
Kowalzic L, Eickenscheidt L, Seidel C, Kribus S, Ziegler H, Komar M
J Dtsch Dermatol Ges 2009 Apr;7(4):360-2. Epub 2008 Nov 20 doi: 10.1111/j.1610-0387.2008.06941.x. PMID: 19054427
Turchin I, Barankin B, Schloss E
Int J Dermatol 2006 Oct;45(10):1215-7. doi: 10.1111/j.1365-4632.2006.02648.x. PMID: 17040445

Diagnosis

Castaño-Fernandez JL, Rodriguez-Cuadrado FJ, Sanchez-Gutierrez I, Najera-Botello L, Salguero Fernandez I, Roustan Gullon G, Rosell-Diaz AM
Eur J Dermatol 2022 Jul 1;32(4):556-558. doi: 10.1684/ejd.2022.4348. PMID: 36301773
Michelerio A, Grassi S, Elena C, Croci GA, Boveri E, Bossi G, Brazzelli V
Eur J Dermatol 2019 Apr 1;29(2):174-178. doi: 10.1684/ejd.2019.3532. PMID: 30973331
Wilmas K, Wohlmuth-Wieser I, Duvic M
Dermatol Online J 2017 Aug 15;23(8) PMID: 29469737
Heymann WR
Skinmed 2017;15(4):287-288. Epub 2017 Aug 1 PMID: 28859740
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479

Therapy

Mortimore AM, Barsoum R, Lambie D, McMeniman E
Australas J Dermatol 2022 Feb;63(1):102-104. Epub 2021 Jul 27 doi: 10.1111/ajd.13674. PMID: 34314029
Tampouratzi L, Kanni T, Katsantonis J, Douvali T
Acta Dermatovenerol Croat 2021 Apr;291(1):60-61. PMID: 34477069
Rahnama-Moghadam S, Burgin C, Gilbert J, Warren S
Dermatol Online J 2018 May 15;24(5) PMID: 30142740
Siddique L, Ahrns H, Seiverling E
BMJ Case Rep 2017 Nov 30;2017 doi: 10.1136/bcr-2017-221784. PMID: 29196283Free PMC Article
Donahue JG, Lupton JB, Golichowski AM
Obstet Gynecol 1995 May;85(5 Pt 2):813-5. doi: 10.1016/0029-7844(94)00305-w. PMID: 7724121

Prognosis

Lawrence M, Sampson J, Oliver F, Restall P, Mackenzie M
Am J Dermatopathol 2021 Dec 1;43(12):e277-e279. doi: 10.1097/DAD.0000000000002070. PMID: 34797809
Tumer G, Jow T, McElligott S, Schwartz RA, Lambert WC
Skinmed 2017;15(3):171-174. Epub 2017 Jun 1 PMID: 28705275
Severino M, Chandesris MO, Barete S, Tournier E, Sans B, Laurent C, Apoil PA, Lamant L, Mailhol C, Laroche M, Fraitag S, Hanssens K, Dubreuil P, Hermine O, Paul C, Bulai Livideanu C
J Am Acad Dermatol 2016 May;74(5):885-91.e1. Epub 2016 Feb 19 doi: 10.1016/j.jaad.2015.10.050. PMID: 26899198
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479
Gibbs NF, Friedlander SF, Harpster EF
Pediatr Dermatol 2000 May-Jun;17(3):194-7. doi: 10.1046/j.1525-1470.2000.01750.x. PMID: 10886750

Clinical prediction guides

Lawrence M, Sampson J, Oliver F, Restall P, Mackenzie M
Am J Dermatopathol 2021 Dec 1;43(12):e277-e279. doi: 10.1097/DAD.0000000000002070. PMID: 34797809
Williams RF, Nazarian RM, Smith GP
Dermatol Online J 2017 Oct 15;23(10) PMID: 29469787
Moon HR, Kim YJ, Jung JM, Won CH, Lee MW, Choi JH, Chang SE
J Dermatol 2017 Nov;44(11):1276-1280. Epub 2017 Jun 23 doi: 10.1111/1346-8138.13928. PMID: 28646532
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479
Bachmeyer C, Guillemette J, Blum L, Turc Y, Dhôte R, Fermand JP, Aractingi S
J Am Acad Dermatol 2000 Nov;43(5 Pt 2):972-4. doi: 10.1067/mjd.2000.109254. PMID: 11044837

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