Glomangiomas multiple D18.0

Rare (10-20% of all glomus tumors), clinical and histological glomuvenous malformation with multiple, disseminated or segmentally arranged, angiomatous tumors of the skin.

The non-segmental disseminated hereditary form is caused by an autosomal-dominantly inherited mutation in the GLMN gene (glomin gene). This gene is mapped to gene locus 1p21-p22 and encodes a phosphorylated protein (glomulin) that belongs to an Skp1-cullin F-box-like complex. The encoded protein is essential for the normal development of the vascular system.

Sporadic glomangiomas are probably classified as somatic mutations of this gene (punctate mosaicism).

Extremities mainly on the acres (fingers, toes, subungual); trunk; mucous membrane infestation is rare.

0.5- 3.0 cm large, blue to blue-black, isolated or also aggregated into beet-like tumor clusters, soft cutaneous venous lesions (papules and/or nodules), typically without pain symptoms (see glomus tumor below). Rarely occurring on mucous membranes and internal organs.

Excision for functionally or cosmetically disturbing localization. S.a.u. Glomus tumor. Successes have also been described with argon or CO2 laser therapies.

Glomangiomas are not the same as glomus tumors. They are 2 different entities.

Moreira C et al (2017): A 15-year-old girl was referred for angiomatous skin lesions on the right breast that had first been noticed shortly after birth and had slowly increased in size as the patient grew, but significantly faster in recent years. Family history revealed that the patient's father had similar lesions on the left shoulder. Physical examination revealed several bluish, coalescing soft nodules on the right breast. Histologic examination revealed dilated, irregular vascular channels surrounded by endothelial cells and one to several layers of uniform cuboidal cells with pale or faint eosinophilic cytoplasm. The histologic findings were consistent with a glomuvenous malformation (GMM/glomangiomatosis).

1. Ahmed A et al. (2003) Intranasal glomangioma. Rhinology 41: 58-60
2. Allombert-Blaise CJ et al. (2003) Type 2 segmental manifestation of congenital multiple glomangiomas. Dermatology 206:321-325
3. Brouillard P et al. (2002) Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations ("glomangiomas"). Am J Hum Genet 70: 866-874
4. Brauer JA et al. (2011) Glomuvenous Malformations (Familial generalized multiple glomangiomas). Dermatol Online J 17(10):9.
5. Happle R et al. (1999) Type 2 segmental manifestation of multiple glomus tumors: A review and reclassification of 5 case reports. Dermatology 198:270-272.
6. 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
7. Jacobi H et al. (1996) Congenital familial plaque-like glomus tumors. Dermatology 47: 387-390
8. 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

9. Moreira C et al. (2017) Bluish nodules of the breast in an adolescent girl. BMJ Case Rep 2017:bcr2016218398.

10. Schmiedseder M et al (2014) Multiple bluish nodules. Familial glomangiomatosis. Dermatology 65: 556-558
11. Wolff HW et al (1981) Multiple glomus tumors. Dermatologist 32: 354-358