Targetoid Hemosiderotic Hemangioma Clinical Presentation
- Author: J Andrew Carlson, MD; Chief Editor: Dirk M Elston, MD more...
Targetoid hemosiderotic hemangioma (THH) (hobnail hemangioma) is a benign vascular tumor that typically manifests as a small, single lesion on the extremity or trunk of a young to middle-aged adult. It has a variable clinical appearance, but most can be described as annular lesions with a central violaceous papule surrounded by an eccentric ecchymotic ring that can exhibit a targetoid appearance. The wide variation in clinical appearance and variegation of color explains why these lesions can be mistaken for a hemangioma, melanocytic nevus, or melanoma.
Lesions generally are asymptomatic, but they may be painful, change color, increase in size, or exhibit cyclical or episodic change. These changes typically are described as enlargement with deepening of color hue, followed by decrease in size and diminished color intensity.
Cases with cyclical morphologic changes have been correlated with hormonal events of the menstrual cycle, although it should be noted that some of these cases have not been documented rigorously from a microscopic standpoint and can represent other processes such as endometriosis.
Spontaneous regression without scarring has been described with recurrence in 1 of 2 cases reported.[12, 13]
Classic targetoid hemosiderotic hemangioma (THH) manifests as a solitary pigmented lesion consisting of a central, small (2-3 mm), violaceous papule (the bull's-eye) surrounded by an erythematous-to-ecchymotic ring or halo. Overall dimensions can vary from 1-2 cm. Note the image below.
Lesions typically affect the trunk or the extremities (lower > upper). Head, neck, and oral involvement and the occurrence of 2 simultaneous lesions have been reported.
The typical clinical morphology consists of a macule, papule, or targetlike lesion. Targetoid lesions are more likely to be seen in larger and symptomatic targetoid hemosiderotic hemangiomas, while macular targetoid hemosiderotic hemangioma lesions typically are small.
A central papule is common but is not present universally. The peripheral halo can wax and wane in both size and color as a result of the degree of erythrocyte extravasation and hemosiderin deposition. The predominant color is brown or tan; a few lesions exhibit a black, violaceous, or grayish appearance.
Variability in clinical morphology results in a wide variety of clinical diagnoses, including considerations such as KS, melanocytic nevus, or melanoma.
Dermoscopic examination reveals sharply demarcated red or reddish-blue round and oval structures. Intermixed with these structures are smaller, pale pink, round structures found among a diffuse pink-white pigmentation. These 2 regions are thought to correspond to ecstatic thin-walled vessels of the papillary dermis and slitlike vascular spaces of the reticular dermis, respectively. A few sharply demarcated black macules representing hemorrhagic crusts can also be found.
If serially monitored dermoscopically, a violaceous, expanding ring can be found surrounding the central vascular dilations. This ring becomes an erythematous or hypopigmented halo that eventually fades. Fine delicate pigmentation corresponding to hemosiderin can be found in this halo region.[13, 14, 15]
Pigment networks (ie, brown or black globules or dots) typical of melanocytic proliferations are not found in targetoid hemosiderotic hemangiomas.
Trauma is the only known predisposing factor for targetoid hemosiderotic hemangioma (THH).[16, 17] Reports of targetoid hemosiderotic hemangioma secondary to irritation from a belt and arthropod assaults exist. Histologic findings of foreign body giant cell reaction in a few cases points to a preceding injury. Episodic changes of enlarging and diminishing hemorrhagic/hemosiderotic halo also implicate recurring trauma or vessel fragility.
Hormones can influence clinical morphology, resulting in the cyclic changes of waxing and waning diameter and peripheral color. Estrogen is believed to mediate pregnancy-related vessel changes, such as spider telangiectases, and estrogen is known to promote vascular permeability and fragility, venous distensibility, increased blood flow, and vasomotor instability. In targetoid hemosiderotic hemangioma, fluctuating estrogen levels may result in vessel instability and leakage. Of note, hormonal receptors have not been identified histologically in lesions of targetoid hemosiderotic hemangioma noted to cyclically change with the menstrual cycle.
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