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Bullous diffuse cutaneous mastocytosis

MedGen UID:
1781582
•Concept ID:
C5546764
•
Neoplastic Process
Synonyms: Bullous DCM; bullous DCM; bullous diffuse cutaneous mastocytosis
SNOMED CT: Bullous diffuse cutaneous mastocytosis (1148891004)
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:0017243
Orphanet: ORPHA280785

Definition

Bullous diffuse cutaneous mastocytosis (BDCM) is a form of diffuse cutaneous mastocytosis (DCM; see this term) characterized by generalized erythroderma and severe blistering associated with the accumulation of mast cells in the skin. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBullous diffuse cutaneous mastocytosis

Professional guidelines

PubMed

Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis.
Marzano AV, Maronese CA, Genovese G, Ferrucci S, Moltrasio C, Asero R, Cugno M
J Allergy Clin Immunol 2022 Apr;149(4):1137-1149. doi: 10.1016/j.jaci.2022.02.007. PMID: 35396080

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: 38338679
A widespread blistering eruption: diffuse cutaneous mastocytosis.
Ewulu A, Cardis MA, Carter MC, Habeshian KA
Dermatol Online J 2023 Dec 15;29(6) doi: 10.5070/D329663004. PMID: 38478675
Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination: A Case Report.
Hudson A, Finlayson L
J Cutan Med Surg 2016 Nov;20(6):596-599. Epub 2016 Jul 19 doi: 10.1177/1203475416661312. PMID: 27436822
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
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3):15S-18S. PMID: 1705949

Diagnosis

A widespread blistering eruption: diffuse cutaneous mastocytosis.
Ewulu A, Cardis MA, Carter MC, Habeshian KA
Dermatol Online J 2023 Dec 15;29(6) doi: 10.5070/D329663004. PMID: 38478675
Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis.
Marzano AV, Maronese CA, Genovese G, Ferrucci S, Moltrasio C, Asero R, Cugno M
J Allergy Clin Immunol 2022 Apr;149(4):1137-1149. doi: 10.1016/j.jaci.2022.02.007. PMID: 35396080
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
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
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3):15S-18S. PMID: 1705949

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
Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination: A Case Report.
Hudson A, Finlayson L
J Cutan Med Surg 2016 Nov;20(6):596-599. Epub 2016 Jul 19 doi: 10.1177/1203475416661312. PMID: 27436822
Diffuse cutaneous bullous mastocytosis in a newborn.
Wawrzycki B, Pietrzak A, Chodorowska G, Kanitakis J
Dermatol Ther 2013 Mar-Apr;26(2):176-9. doi: 10.1111/j.1529-8019.2013.01544.x. PMID: 23551376
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
Bullous mastocytosis.
Orkin M, Good RA, Clawson CC, Fisher I, Windhorst DB
Arch Dermatol 1970 May;101(5):547-64. PMID: 4192086

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: 38338679
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
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
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3):15S-18S. PMID: 1705949
Bullous mastocytosis.
Orkin M, Good RA, Clawson CC, Fisher I, Windhorst DB
Arch Dermatol 1970 May;101(5):547-64. PMID: 4192086

Clinical prediction guides

Diffuse cutaneous bullous mastocytosis in a newborn.
Wawrzycki B, Pietrzak A, Chodorowska G, Kanitakis J
Dermatol Ther 2013 Mar-Apr;26(2):176-9. doi: 10.1111/j.1529-8019.2013.01544.x. PMID: 23551376

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