Dermatologic Manifestations of Glomus Tumor Clinical Presentation
- Author: Jon H Meyerle, MD; Chief Editor: Dirk M Elston, MD more...
Patients with solitary glomus tumors usually have paroxysmal pain, which can be severe and can be exacerbated by pressure or temperature changes, especially cold. Multiple glomus tumors can also be painful, but this feature is less common, and the pain is usually not severe.
Patients with multiple lesions often seek medical attention because they are worried or have cosmetic concerns. Because multiple glomus tumors are inherited as an autosomal dominant condition, a family history of similar lesions may be helpful for diagnosis.
Extracutaneous sites have been reported, including involvement of the gastrointestinal tract, trachea, nerve, bone, mediastinum, liver, pancreas, kidney and ovary,[14, 15, 16, 17, 18] with a case of a malignant glomus tumor of the kidney having been reported.
Solitary glomus tumors have the following characteristics:
Blue or red blanchable papules or nodules in deep dermis or subcutis
Acral location, most commonly, especially subungual
Size usually smaller than 2 cm
The multiple variant is subdivided into regional or localized, disseminated, and congenital plaquelike forms, as follows:
Regional variant - Consists of blue-to-purple partially compressible papules or nodules that are grouped and limited to a specific area, most commonly to an extremity
Disseminated type - Consists of multiple lesions distributed over the body with no specific grouping; less common than the regional variant
Congenital plaquelike glomus tumors - Consist of either grouped papules that coalesce to form indurated plaques or clusters of discrete nodules; rarest variant of multiple glomus tumors
Two useful findings for diagnosing glomus tumors, particularly solitary painful glomus tumors (especially those under a nail), are the following[19, 20] :
Love test - Eliciting exquisite localized pain by applying pressure to the suspected areas with a pencil tip or pinhead
Hildreth sign - Reduction of pain and tenderness and reduction of tenderness with the Love test by inducing transient ischemia with a tourniquet
For a review of both the Love test, as well as the Hildreth sign, to include a simple clinical diagnostic algorithm, Tang et al's paper is a notable resource. 
Features of malignant glomus tumors, or glomangiosarcomas, may include the following :
Size larger than 2 cm
Deep soft tissue involvement
Glomus tumors are neoplasms caused by a proliferation of glomus cells, which make up a portion of the glomus body. The initiating event for glomus cell proliferation is unknown. Some authors have postulated that trauma induces solitary subungual glomus tumors, although this theory is not well studied.
Most multiple glomus tumors, especially the disseminated form, are inherited in an autosomal dominant pattern with incomplete penetrance. Most hereditary glomangiomas are associated with defects in the glomulin gene, located on chromosome 1.[5, 6, 7]
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