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Urticaria pigmentosa

MedGen UID:
22588
•Concept ID:
C0042111
•
Neoplastic Process
Synonym: maculopapular cutaneous mastocytosis
SNOMED CT: Urticaria pigmentosa (78745000); Localized cutaneous mastocytosis (78745000); Urticaria pigmentosa (703828003)
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
Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations.
Castells M, Metcalfe DD, Escribano L
Am J Clin Dermatol 2011 Aug 1;12(4):259-70. doi: 10.2165/11588890-000000000-00000. PMID: 21668033Free PMC Article
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

Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
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
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
Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
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
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

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
Ultraviolet phototherapy for pruritus.
Rivard J, Lim HW
Dermatol Ther 2005 Jul-Aug;18(4):344-54. doi: 10.1111/j.1529-8019.2005.00032.x. PMID: 16297008

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 Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
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
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
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 Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
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 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
Urticaria pigmentosa: a clinical, hematopathologic, and serologic study of 30 adults.
Topar G, Staudacher C, Geisen F, Gabl C, Fend F, Herold M, Greil R, Fritsch P, Sepp N
Am J Clin Pathol 1998 Mar;109(3):279-85. doi: 10.1093/ajcp/109.3.279. PMID: 9495199
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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