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Glomangiomatosis generalizedD18.0
Synonym(s)
Familial glomangiomatosis; familial glomuvenous malformation; Glomangiomas; glomuvenous malformations; Multiple familial glomangiomas
DefinitionThis section has been translated automatically.
Rare (10-20% of all glomus tumors), clinical and histological glomus tumor variant with multiple, disseminated or segmentally arranged, angiomatous tumors of the skin
EtiopathogenesisThis section has been translated automatically.
Autosomal-dominantly inherited genetic defect of the VMGLOM gene (glomin gene), which maps on the gene locus 1p21-p22 Sporadic glomangiomas can probably be classified as somatic mutations of this gene (punctual mosaic).
ManifestationThis section has been translated automatically.
With children and young adults.
LocalizationThis section has been translated automatically.
Extremities mainly at the acra (fingers, toes, subungual); rarely is an infestation of the mucous membrane.
Clinical featuresThis section has been translated automatically.
0.5 - 3.0 cm in size, blue, to blue-black, isolated or aggregated to beet-like tumor volumes, soft papules and/or nodules, typically without pain symptoms (see below glomus tumor). Rarely found on mucous membranes and internal organs.
HistologyThis section has been translated automatically.
In the dermis and subcutis, detectable convolutes of large, dilated, thin-walled vessels, which are reminiscent of venous malformations but are distinguished from these by a surrounding single- or multi-row cell row of cuboid cells with very uniform chromatin-tight nuclei. Thrombi or phlebolites can be found in the vascular spaces. No mitoses detectable.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Excision for functionally or cosmetically disturbing localization. S.a.u. Glomus tumor. Successes have also been described with argon or CO2 laser therapies.
LiteratureThis section has been translated automatically.
- Ahmed A et al (2003) Intranasal glomangioma. Rhinology 41: 58-60
- Brouillard P et al (2002) Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations ("glomangiomas"). At J Hum Genet 70: 866-874
- Brauer JA et al (2011) Glomuvenous malformations (Familial generalized multiple glomangiomas).
Dermatol Online J 17(10):9. - Irrthum, H et al (2001) Linkage disequilibrium narrows locus for venous malformation with glomus cells (VMGLOM) to a single 1.48 Mbp YAC. Hum Genet 9: 34-38
- Jacobi H et al. (1996) Congenital familial plaque-type glomus tumors. Dermatologist 47: 387-390
- Kapur N et al (2002) Local and systemic expression of basic fibroblast growth factor in a patient with familial glomangioma. Br J Dermatol 146: 518-522
- Schmiedseder M et al (2014) Multiple blue knots. Familial glomangiomatosis. Dermatologist 65: 556-558
- Wolff HW et al (1981) Multiple glomus tumors. dermatologist 32: 354-358