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Urticaria pigmentosa

MedGen UID:
22588
•Concept ID:
C0042111
•
Neoplastic Process
Synonym: maculopapular cutaneous mastocytosis
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019316
OMIM®: 154800
Orphanet: ORPHA79457

Definition

Maculopapular cutaneous mastocytosis (MCM) is a form of cutaneous mastocytosis (CM; see this term) characterized by the presence of multiple hyperpigmented macules, papules or nodules associated with abnormal accumulation of mast cells in the skin. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUrticaria pigmentosa
Follow this link to review classifications for Urticaria pigmentosa in Orphanet.

Professional guidelines

PubMed

Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684
Current approaches to the diagnosis and treatment of systemic mastocytosis.
Bains SN, Hsieh FH
Ann Allergy Asthma Immunol 2010 Jan;104(1):1-10; quiz 10-2, 41. doi: 10.1016/j.anai.2009.11.006. PMID: 20143640

Recent clinical studies

Etiology

Clonal mast cell disorders and hereditary α-tryptasemia as risk factors for anaphylaxis.
Kačar M, Rijavec M, Šelb J, Korošec P
Clin Exp Allergy 2023 Apr;53(4):392-404. Epub 2023 Jan 18 doi: 10.1111/cea.14264. PMID: 36654513
Pathology of extramedullary mastocytosis.
Doyle LA, Hornick JL
Immunol Allergy Clin North Am 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010. PMID: 24745677
Urticaria.
Zuberbier T
Allergy 2003 Dec;58(12):1224-34. doi: 10.1046/j.1398-9995.2003.00327.x. PMID: 14616095
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Diagnosis

Cutaneous mastocytosis: A dermatological perspective.
Di Raimondo C, Del Duca E, Silvaggio D, Di Prete M, Lombardo P, Mazzeo M, Spallone G, Campione E, Botti E, Bianchi L
Australas J Dermatol 2021 Feb;62(1):e1-e7. Epub 2020 Oct 11 doi: 10.1111/ajd.13443. PMID: 33040350
Urticaria Pigmentosa.
Panigrahi A, Chakraborty S, Sil A
J Pediatr 2020 Jul;222:249. Epub 2020 May 20 doi: 10.1016/j.jpeds.2020.03.053. PMID: 32444224
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.
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
Urticaria.
Zuberbier T
Allergy 2003 Dec;58(12):1224-34. doi: 10.1046/j.1398-9995.2003.00327.x. PMID: 14616095
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603

Therapy

Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684
Current approaches to the diagnosis and treatment of systemic mastocytosis.
Bains SN, Hsieh FH
Ann Allergy Asthma Immunol 2010 Jan;104(1):1-10; quiz 10-2, 41. doi: 10.1016/j.anai.2009.11.006. PMID: 20143640
Differential diagnosis of chronic urticaria.
Brodell LA, Beck LA
Ann Allergy Asthma Immunol 2008 Mar;100(3):181-8; quiz 188-90, 215. doi: 10.1016/S1081-1206(10)60438-3. PMID: 18426134
Blistering disorders in childhood.
Eichenfield LF, Honig PJ
Pediatr Clin North Am 1991 Aug;38(4):959-76. doi: 10.1016/s0031-3955(16)38162-7. PMID: 1651468

Prognosis

Cutaneous mastocytosis: A dermatological perspective.
Di Raimondo C, Del Duca E, Silvaggio D, Di Prete M, Lombardo P, Mazzeo M, Spallone G, Campione E, Botti E, Bianchi L
Australas J Dermatol 2021 Feb;62(1):e1-e7. Epub 2020 Oct 11 doi: 10.1111/ajd.13443. PMID: 33040350
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.
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
Pathology of extramedullary mastocytosis.
Doyle LA, Hornick JL
Immunol Allergy Clin North Am 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010. PMID: 24745677
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Clinical prediction guides

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.
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
Differential diagnosis of chronic urticaria.
Brodell LA, Beck LA
Ann Allergy Asthma Immunol 2008 Mar;100(3):181-8; quiz 188-90, 215. doi: 10.1016/S1081-1206(10)60438-3. PMID: 18426134
Urticaria pigmentosa.
Brockow K
Immunol Allergy Clin North Am 2004 May;24(2):287-316, vii. doi: 10.1016/j.iac.2004.01.002. PMID: 15120152
Mastocytosis complicating pregnancy.
Worobec AS, Akin C, Scott LM, Metcalfe DD
Obstet Gynecol 2000 Mar;95(3):391-5. doi: 10.1016/s0029-7844(99)00591-8. PMID: 10711550
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Recent systematic reviews

Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Paediatric mastocytosis: a systematic review of 1747 cases.
Méni C, Bruneau J, Georgin-Lavialle S, Le Saché de Peufeilhoux L, Damaj G, Hadj-Rabia S, Fraitag S, Dubreuil P, Hermine O, Bodemer C
Br J Dermatol 2015 Mar;172(3):642-51. Epub 2015 Feb 8 doi: 10.1111/bjd.13567. PMID: 25662299

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