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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVBullous diffuse cutaneous mastocytosis

Hematopoietic and Lymphoid System Disorder
- Hematologic disorder
  - Hematopoietic and lymphoid cell neoplasm
    - Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
      - Cutaneous mastocytosis
        Diffuse cutaneous mastocytosis
        Bullous 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

See all (1)

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 Free PMC Article

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 mastocytosis: analysis of 10 cases and a brief review of the literature.

Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W
J Eur Acad Dermatol Venereol 2012 Dec;26(12):1565-71. Epub 2011 Nov 18 doi: 10.1111/j.1468-3083.2011.04350.x. PMID: 22092511

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

See all (15)

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: 38338679 Free PMC Article

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

See all (43)

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

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

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

Bullous mastocytosis.

Orkin M, Good RA, Clawson CC, Fisher I, Windhorst DB
Arch Dermatol 1970 May;101(5):547-64. PMID: 4192086

See all (25)

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 Free PMC Article

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

See all (22)