Pseudolymphomatous Cutaneous Angiosarcoma Presenting With Persistent Firm Facial Edema in a Patient With Systemic...

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Pseudolymphomatous cutaneous angiosarcoma (cAS) is a rare vascular neoplasm.
• It presents diagnostic challenges due to its lymphocytic infiltrate, mimicking lymphoid neoplasms.
• Systemic sclerosis (SSc) is an autoimmune disorder associated with increased cancer risk.

Observation:

• A patient with a 21-year history of SSc developed persistent, firm facial edema.
• Imaging revealed inflammatory changes, lymphadenopathies, and lytic lesions.
• Skin biopsy showed a diffuse neoplasm with vascular channels and dense lymphocytic proliferation.

Findings:

• The case represents the first documented instance of cAS presenting with facial edema in a patient with SSc.
• Histopathology confirmed cAS with features of vascular channels and endothelial cell proliferation.
• Immunohistochemistry, including ERG, was crucial for differentiating cAS from lymphoid processes.

Implications:

• This case underscores the diagnostic difficulties of cAS, particularly its presentation with facial edema in SSc patients.
• It highlights a potential association between SSc and cAS, warranting further investigation into cancer risk.
• Early detection and accurate diagnosis are critical for improving patient prognosis in cAS.