Targetoid Hemosiderotic Hemangioma (Hobnail Hemangioma)

Are You Confident of the Diagnosis?

What you should be alert for in the history

Targetoid hemosiderotic hemangioma (THH) is rare ( sixty-two cases reported) and usually asymptomatic. It can occur at any age (median age 32 years).

There is a characteristic halo that can fade and disappear over time. Lesions can undergo cycles of regression and recurrence.

Characteristic findings on physical examination

– Red-blue to brown papules, 2-3mm (Figure 1)
– Solitary, well-circumscribed
– Some are surrounded by a thin pale area and an ecchymotic ring (halo)
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– Can occur anywhere on the body, including tongue and gingiva

Targetoid hemosiderotic hemangioma. (Courtesy of Klaus Helm, MD)

Expected results of diagnostic studies

Histopathology

Biphasic growth pattern:

– Dilated thin-walled dermal vessels, lined by distinct “hobnail” endothelial cells coalescing with smaller, slit-like vessels in the deep dermis

– Extravasation of RBCs with hemosiderin deposition is common

Diagnosis confirmation

Differential Diagnosis

– Melanocytic nevus: distinct growth pattern, distinguishable on histopathological examination

– Other vascular proliferations: absent halo features, distinct biphasic growth pattern on histopathological examination

Who is at Risk for Developing this Disease?

Equal sex distribution

Median age of 32 years (ages of 6-72 have been reported)

What is the Cause of the Disease?

Etiology

The etiology is unknown. The presence of increased VEGFR-3 may implicate lymphatic channel dysregulation. D2-40 staining of lesions also suggests a lymphatic malformation.

Pathophysiology

Trauma has been implicated as an etiologic factor; however, its precise role in the development of lesions is speculative.

Systemic Implications and Complications

Benign, asymptomatic course

Can regress and recur over time

Treatment Options

Treatment is usually unnecessary.

Surgical excision can be performed if desired with little risk of recurrence.

Pulsed dye laser (PDL) can play a theoretical role, but the rarity of this lesion makes the role and/or efficacy of PDL difficult to assess.

Optimal Therapeutic Approach for this Disease

Reassurance
Treatment is usually unnecessary
Surgical excision can be performed if desired

Patient Management

Reassurance of benign course
Treatment is usually unnecessary
Surgical excision can be performed if desired. There is little to no risk of recurrence.

Unusual Clinical Scenarios to Consider in Patient Management

Increasing growth or presence of symptoms should prompt concern for a neoplastic proliferation and prompt a biopsy for histopathologic examination.

What is the Evidence?

North, PE, Kincannon, J, Bolognia, JL, Jorizzo, JL, Rapini, RP. “Vascular neoplasms and neoplastic-like proliferations”. Dermatology. 2008. pp. 1771-94. (An excellent textbook summary with good clinical and pathologic images on a variety of vascular disorders, including THH)

Ghibaudo, N. “Fully regressive targetoid hemosiderotic hemangioma”. J Eur Acad Dermatol Vernereol. vol. 23. 2009. pp. 722-3. (A case report highlighting the role of dermoscopy in vascular lesions as well as the first case of a fully regressive THH)

Al Dhaybi, R, Lam, C, Hatami, A, Powell, J, McCuaig, C, Kokta, V. “Targetoid hemosiderotic hemangiomas (hobnail hemangiomas) are vascular lymphatic malformations: A study of 12 pediatric cases”. J Am Acad Dermatol. vol. 66. 2012. pp. 116-20. (D2-40 was positive in all twelve cases, suggesting a lymphatic origin of the lesions; nine cases were negative for Wilms tumor-1 gene and the proliferative index utilizing Ki-67 was low, allowing the authors to contend that these are actually lymphatic malformations.)

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