A Bruise-Like Patch to the Forehead and Scalp

Ravneet Ruby Kaur, Hilary Mandel

Disclosures

Dermatology Nursing. 2009;21(2):98-99. 

The “Clinical Snapshot” series provides a concise examination of a clinical presentation including history, treatment, patient education, and nursing measures. Using the format here, you are invited to submit your “Clinical Snapshot” to Dermatology Nursing.

History: An 82-year-old man with a history of nonmelanoma skin cancer presented for a skin check.

Description: He had an enlarging purpuric ovoid patch of the right hairline for 2.5 months. On initial examination, the lesion was 4 cm x 1.5 cm. The patient denied pain, pruritus, bleeding, trauma, or previous radiation to the area. The patient was not on any anti-coagulants, corticosteroids, or aspirin, but was taking non-steroidal antiinflammatory drugs daily for joint pain.

One month later, the examination showed a 9.5 x 1.5 cm smooth, linear purpuric patch at the right hairline and frontal scalp. Figure 1 shows the lesion at the time of initial presentation. The lesion did not appear to be consistent with a melanocytic process, such as a nevus or melanoma, and had a vascular appearance. Laboratory analysis including a complete blood count with platelets, chemistry panel, and liver function tests were unremarkable. The chest x-ray was negative. A shave biopsy was performed. Figure 2 shows the pathology specimen at a magnification of 4X.

Figure 1.

Enlarging purpuric ovoid patch of the right hairline.

Figure 2.

Vascular neoplasm favoring low-grade angiosarcoma.

Differential Diagnoses: Post-traumatic bruise, senile purpura, hobnail hemangioendothelioma, Kaposi’s sarcoma (arguably a form of angiosarcoma), radiationinduced vascular proliferation, bacillary angioimatosis.

Diagnosis: Low-grade cutaneous angiosarcoma.

Microscopic Findings and Clinical Course: Histopathologic examination of the biopsy specimen revealed a vascular neoplasm favoring low-grade angiosarcoma, although hobnail hemangioendothelioma was also considered in the differential. The diagnosis was confirmed by the Armed Forces Institute of Pathology.

Excision with split thickness skin graft was performed. Microscopic examination from the excision specimen showed focal atypical vessels and vascular proliferation with rare atypical endothelial cells at the deep margin. The lateral margin was clear. Figure 3 shows the pathology slides specimen at a magnification of 20X. Focal atypical vessels and vascular proliferation with rare atypical endothelial cells can be seen. Of note, actinic keratosis was seen in the non-neoplastic skin. Staging computed tomographic studies of the head, neck, chest, and abdomen were negative for masses, lymphadenopathy, metastases, or hemorrhage. The Tumor, Nodes, Metastasis (TNM) classification of tumor staging was: T2, N0, M0. Six weeks postoperatively the STSG and left thigh donor site had healed well.

Figure 3.

Focal atypical vessels and vascular proliferation.

Discussion: Angiosarcoma is a rare, aggressive vascular neoplasm (Eiling et al., 2002). Fewer than 5% of soft tumor sarcomas occur in the head and neck, with only approximately 10% classified as angiosarcomas (Pawlik et al., 2003). Most occur in the scalp and face with a strong predilection for elderly White men. Pawlik and colleagues (2003) found that angiosarcoma can occur in the settings of chronic lymphedema, chronic radiation dermatitis, or as a result of severe trauma. Lesions tend to be multifocal, violaceous patches; nodules or plaques with extensive local growth are common. Asgari, Cockerell, and Weitzul (2007) described a useful diagnostic tool in cutaneous angiosarcoma: the Head Tilt Maneuver which highlights the vascular nature of these lesions. The maneuver involves lowering the patient’s head below the level of the heart, which serves to intensify the erythema of the vascular lesions. This easy noninvasive maneuver can help facilitate early diagnosis and gain a better appreciation of the clinical extent of the tumor.

Elder, Elenitsas, Johnson, and Murphy (2005) found that metastasis to regional lymph nodes and lungs may occur. Five-year survival is between 12% and 33%. Zanetta and colleagues (2005) suggested that a decrease in cell adhesion molecules such as cadherins, which are calcium-ion dependent adhesive proteins located at intercellular adherens junctions, may contribute to the local invasiveness and potential metastasis of angiosarcoma. Histopathology demonstrates irregular anastomosing vascular channels lined by atypical endothelial cells with frequent mitotic figures. Nuclear atypia is present with large atypical hyperchromatic cells (Weedon, 2002).

Elder and colleagues (2005) found that hobnail hemangioendothelioma, in contrast, shows ectatic or dilated vascular channels lined by plump endothelial cells in the upper dermis with intraluminary papillary projections. Distinguishing features of Kaposi sarcoma include benign-appearing endothelial cells in the vascular channels with absence of mitoses, slit-like vascular spaces, eosinophilic globules, and a plasma cell infiltrate.

Elder and colleagues (2005) agreed with Ohsawa and colleagues (1995) that CD31 is a specific immunohistochemical marker for endothelial cell differentiation and CD34 is also sensitive. Other useful endothelial cell markers include Factor VIII-Related Antigen and Ulex europaeus lectin type 1. Also, the absence of von-Willebrand Factor labeling and a decrease in Weibel-Pallade bodies favor a lymphatic endothelial origin of angiosarcoma (Elder et al., 2005).

Pawlik and colleagues (2003) contended that the gold standard of treatment is excision although complete removal is difficult because of extension beyond the apparent clinical lesion. It is difficult to obtain negative surgical margins, only seen in less than 21.4% in one study. Eiling and colleagues (2002) found that recurrence is frequent, requiring close clinical followup. Other treatment options include radiation therapy and chemotherapy with liposomal doxorubicin. Pestoni, Paredes-Saurez, Peteiro, and Toibio (2005) found paclitaxel to be effective as well. In a recent study based on 69 cases of sporadic cutaneous angiosarcoma, estimated 5-year disease-specific survival was 48% (Deyrup, McKenney, & Tighiouart, 2008).

Conclusion: It is important not to overlook ecchymotic lesions of recent onset, especially without a history of trauma. Skin biopsies should be performed on these lesions with a high index of suspicion for angiosarcoma. Since the disease is rare, standard protocols have not been developed. We recommend that patients diagnosed with cutaneous angiosarcoma be followed closely every 3 to 6 months for the first 2 years and then every year for life after. Special attention should be paid to lymph nodes and other sites for metastasis.

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