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Diffuse cutaneous mastocytosis

MedGen UID:
44303
•Concept ID:
C0024901
•
Neoplastic Process
Synonyms: Cutaneous Mastocytoses, Diffuse; Cutaneous Mastocytosis, Diffuse; Mastocytosis, Diffuse Cutaneous
SNOMED CT: Diffuse cutaneous mastocytosis (703826004)
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:0019315
OMIM®: 154800
Orphanet: ORPHA79456

Definition

Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM; see this term) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM; see this term) and one with infiltrations (Pseudoxanthomatous DCM; see this term). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse cutaneous mastocytosis
Follow this link to review classifications for Diffuse cutaneous mastocytosis 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
Assessment of clinical findings, tryptase levels, and bone marrow histopathology in the management of pediatric mastocytosis.
Carter MC, Clayton ST, Komarow HD, Brittain EH, Scott LM, Cantave D, Gaskins DM, Maric I, Metcalfe DD
J Allergy Clin Immunol 2015 Dec;136(6):1673-1679.e3. Epub 2015 Jun 1 doi: 10.1016/j.jaci.2015.04.024. PMID: 26044856Free PMC Article
Diagnostic criteria and classification of mastocytosis: a consensus proposal.
Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nuñez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM
Leuk Res 2001 Jul;25(7):603-25. doi: 10.1016/s0145-2126(01)00038-8. PMID: 11377686

Recent clinical studies

Etiology

Diffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.
Rydz A, Lange M, Ługowska-Umer H, Sikorska M, Nowicki RJ, Morales-Cabeza C, Alvarez-Twose I
Int J Mol Sci 2024 Jan 23;25(3) doi: 10.3390/ijms25031401. PMID: 38338679Free PMC Article
Pediatric and Hereditary Mastocytosis.
Renke J, Irga-Jaworska N, Lange M
Immunol Allergy Clin North Am 2023 Nov;43(4):665-679. Epub 2023 May 19 doi: 10.1016/j.iac.2023.04.001. PMID: 37758405
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 mastocytosis: report of six cases.
Inamadar AC, Palit A
Indian J Dermatol Venereol Leprol 2006 Jan-Feb;72(1):50-3. doi: 10.4103/0378-6323.19720. PMID: 16481712
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

Diagnosis

Diffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.
Rydz A, Lange M, Ługowska-Umer H, Sikorska M, Nowicki RJ, Morales-Cabeza C, Alvarez-Twose I
Int J Mol Sci 2024 Jan 23;25(3) doi: 10.3390/ijms25031401. PMID: 38338679Free PMC Article
Pediatric and Hereditary Mastocytosis.
Renke J, Irga-Jaworska N, Lange M
Immunol Allergy Clin North Am 2023 Nov;43(4):665-679. Epub 2023 May 19 doi: 10.1016/j.iac.2023.04.001. PMID: 37758405
Diffuse Cutaneous Mastocytosis.
Zaouak A, Helel I, Hammami H, Fenniche S
Skinmed 2022;20(5):355-356. Epub 2022 Oct 31 PMID: 36314699
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
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

Diffuse cutaneous mastocytosis: Case report and literature review.
Hosking AM, Makdisi J, Ortenzio F, de Feraudy S, Smith J, Linden K
Pediatr Dermatol 2018 Nov;35(6):e348-e352. Epub 2018 Sep 6 doi: 10.1111/pde.13651. PMID: 30187958
Effective management of severe cutaneous mastocytosis in young children with omalizumab (Xolair(®) ).
Hughes JDM, Olynyc T, Chapdelaine H, Segal L, Miedzybrodzki B, Ben-Shoshan M
Clin Exp Dermatol 2018 Jul;43(5):573-576. Epub 2018 Feb 16 doi: 10.1111/ced.13408. PMID: 29451318
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 mastocytosis: a case of bullous urticaria pigmentosa.
Vasili E, Savo I, Doci X, Shkodrani E, Ntemoy A
Cutis 2013 Feb;91(2):70-2. PMID: 23513553
Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature.
Koga H, Kokubo T, Akaishi M, Iida K, Korematsu S
Pediatr Dermatol 2011 Sep-Oct;28(5):542-6. Epub 2010 Jul 29 doi: 10.1111/j.1525-1470.2010.01212.x. PMID: 20678092

Prognosis

Diffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.
Rydz A, Lange M, Ługowska-Umer H, Sikorska M, Nowicki RJ, Morales-Cabeza C, Alvarez-Twose I
Int J Mol Sci 2024 Jan 23;25(3) doi: 10.3390/ijms25031401. PMID: 38338679Free PMC Article
Pediatric and Hereditary Mastocytosis.
Renke J, Irga-Jaworska N, Lange M
Immunol Allergy Clin North Am 2023 Nov;43(4):665-679. Epub 2023 May 19 doi: 10.1016/j.iac.2023.04.001. PMID: 37758405
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
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
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

Clinical prediction guides

When Mast Cells Run Amok: A Comprehensive Review and Case Study on Severe Neonatal Diffuse Cutaneous Mastocytosis.
Olteanu EG, Bataneant M, Puiu M, Chirita-Emandi A
Genes (Basel) 2023 Oct 29;14(11) doi: 10.3390/genes14112021. PMID: 38002964Free PMC Article
Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children.
Hermans MAW, Pasmans SGMA, Arends NJT, van den Bosch TPP, van Daele PLA, van Doorn MBA, Huisman EJ, Mooyaart AL, Damman J
Clin Exp Dermatol 2022 Sep;47(9):1694-1702. Epub 2022 Jul 11 doi: 10.1111/ced.15262. PMID: 35596520Free PMC Article
Natural evolution in pediatric cutaneous mastocytosis: 10-year follow-up.
Czarny J, Renke J, Żawrocki A, Nowicki RJ, Lange M
Int J Dermatol 2021 Oct;60(10):1253-1257. Epub 2021 Apr 27 doi: 10.1111/ijd.15542. PMID: 33904158
Paediatric mastocytosis: long-term follow-up of 53 patients with whole sequencing of KIT. A prospective study.
Meni C, Georgin-Lavialle S, Le Saché de Peufeilhoux L, Jais JP, Hadj-Rabia S, Bruneau J, Fraitag S, Hanssens K, Dubreuil P, Hermine O, Bodemer C
Br J Dermatol 2018 Oct;179(4):925-932. Epub 2018 Sep 9 doi: 10.1111/bjd.16795. PMID: 29787623
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

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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