Targetoid Hemosiderotic Hemangioma: Two Rare Cases

Sir,

“Hobnail hemangioma” is an umbrella term used to describe all benign and malignant vascular tumors having hobnail-like endothelial cells on histopathology. Targetoid hemosiderotic hemangioma (THH) is a benign vascular tumor with targetoid morphology, often described synonymously as hobnail hemangioma.[1] It is rarely reported in Indian literature. We hereby report two cases of THH. Our first case was, a 20-year male with a solitary, asymptomatic erythematous annular patch with a central evolving papule for 8 months. On examination, we observed a targetoid lesion of 1 cm diameter on the lateral aspect of the left elbow, consisting of three distinct zones, a central black papule with a pale intermediate zone surrounded by a peripheral purple ring of ecchymoses [Figure 1a]. There was no history of trauma preceding the lesion. Dermoscopy revealed a homogenous central area, red and dark lacunae, peripheral red-violaceous ring, pale intermediate areas, and a peripheral pigment network [Figure b].

Figure 1.

(a) A targetoid lesion, 1 cm diameter showing a central black papule, pale intermediate zone surrounded by a peripheral purple ring of ecchymoses (b) Dermoscopy showing homogenous central area, red, dark lacunae, peripheral red-violaceous ring, pale intermediate areas, peripheral pigment network. DermLite III DL3N Polarized and Fluid Dermoscope/Pigment boost

Our second case was an older 35-year male with a single well-defined lesion since last year. It was gradually progressing in size, soft to firm, and non-tender. On examination, we saw a 4 cm diameter black plaque on the right thigh, surrounded by a distinct purple ring with a few interspersed pale areas [Figure 2a]. The patient denied any history of trauma, drug intake, or acute illness and there were no other cutaneous or systemic abnormalities. A 4 mm punch biopsy was performed with THH, tufted angioma, Kaposi sarcoma, and melanoma as differentials. Histopathology revealed a clear biphasic pattern. There were irregularly dilated vessels in the superficial dermis which were lined by a single layer of endothelial cells. These cells were epithelioid-like with hobnail appearance having scanty cytoplasm and round nuclei protruding into the lumen. Within the deep dermis, the blood vessels were similar to lymphatics having a more slit-like, angulated morphology and dissecting the collagen bundles. Additionally, there was scanty lymphohistiocytic infiltrate, extravasation of red blood cells, and hemosiderin deposition [Figure 2b and c]. Hence the diagnosis was established as THH. We successfully excised the lesions in both cases with no recurrence on regular follow up.

Figure 2.

(a) A 4 cm diameter black plaque on the right thigh, surrounded by a distinct purple ring with few interspersed pale areas (b) Haematoxylin Eosin stain at low power 10× showing, blood vessels in papillary dermis lined by a single layer of hobnail-like endothelial cells, reticular dermis showing angulated vessels, dissecting collagen bundles (c) Haematoxylin Eosin stain at high power 40× showing the similar biphasic pattern in papillary and reticular dermis along with sparse lymphohistiocytic infiltrate, extravasation of erythrocytes, hemosiderin deposits

THH was first described as hobnail hemangioma in the year 1988 by Santa Cruz and Aronberg.[1] Its morphology and histopathology vary according to the stage of evolution. It is commonly located in the trunk or extremities.[2] It has no gender predilection and the age of presentation varies from 5 to 67 years, though more common in younger age group.[3] The pathogenesis of THH is explained by trauma to a pre-existing hemangioma and hormonal influence as explained by changes in the lesions in the female during menstruation.[4]

THH is a benign vascular tumor of probable lymphatic origin. However, hobnail hemangioma is an umbrella term to include all vascular tumors that have hobnail-like endothelial cells on histopathology which may be both benign and malignant. Retiform hemangioendothelioma, Dabska tumor (malignant endovascular papillary angioendothelioma), progressive lymphangioma, skin or soft tissue angiosarcoma, polymorphic hemangioendothelioma, atypical vascular lesions after radiotherapy, all are a part of this group.[5]

Panigrahi[6] reported a case of THH in the pediatric age group and Gutte RM[7] reported one in a 30-year male, but no other case has been reported from India till date. To conclude, THH is a rare entity and should be kept as a differential for all acquired vascular tumors.

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Conflicts of interest

There are no conflicts of interest.