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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

Mastocytosis by minor criteria.
McGinnis E, Moshref Razavi H
Int J Hematol 2020 Jan;111(1):1-2. Epub 2019 Nov 8 doi: 10.1007/s12185-019-02774-z. PMID: 31705295
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
Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment.
Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106

Recent clinical studies

Etiology

Urticaria Pigmentosa Without Pruritus.
Nahm WJ, Corriveau DJ, Mathe CA, Wyant WA, Vega C, Arthur A, Min KC
J Drugs Dermatol 2024 Mar 1;23(3):e81-e82. doi: 10.36849/jdd.7558. PMID: 38443117
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
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
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
Skeletal mastocytosis.
Andrew SM, Freemont AJ
J Clin Pathol 1993 Nov;46(11):1033-5. doi: 10.1136/jcp.46.11.1033. PMID: 8254091Free PMC Article

Diagnosis

Urticaria Pigmentosa Without Pruritus.
Nahm WJ, Corriveau DJ, Mathe CA, Wyant WA, Vega C, Arthur A, Min KC
J Drugs Dermatol 2024 Mar 1;23(3):e81-e82. doi: 10.36849/jdd.7558. PMID: 38443117
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
Mast Cell Disorders.
Yu JE, Akin C
J Allergy Clin Immunol Pract 2016 May-Jun;4(3):557-8. doi: 10.1016/j.jaip.2015.10.001. PMID: 27157940
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 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

Therapy

Mastocytosis: Moving the Field to Precision and Personalized Medicine.
Castells MC
Immunol Allergy Clin North Am 2018 Aug;38(3):xv-xvii. Epub 2018 Jun 9 doi: 10.1016/j.iac.2018.05.001. PMID: 30007470
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
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
Urticaria and angioedema.
Burrall BA, Huntley AC
Clin Rev Allergy 1985 Feb;3(1):95-116. doi: 10.1007/BF02993044. PMID: 2579715
The treatment of urticaria pigmentosa.
James MP
Clin Exp Dermatol 1982 May;7(3):311-20. doi: 10.1111/j.1365-2230.1982.tb02433.x. PMID: 6809372

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
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
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
Bullous urticaria pigmentosa.
Leaf FA, Jaecks EP, Rodriguez DR
Cutis 1996 Nov;58(5):358-60. PMID: 8934078
Skeletal mastocytosis.
Andrew SM, Freemont AJ
J Clin Pathol 1993 Nov;46(11):1033-5. doi: 10.1136/jcp.46.11.1033. PMID: 8254091Free PMC Article

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
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
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
Adult osteosclerosis.
Evans RA, Hughes WG, Dunstan CR, Lennon WP, Kohan L, Hills E, Wong SY
Metab Bone Dis Relat Res 1983-1984;5(3):111-7. doi: 10.1016/0221-8747(83)90011-5. PMID: 6676625

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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