gms | German Medical Science

Objectives: Reactive angioendotheliomatosis (RAE) is a rare, but self-limited disease characterized by intravascular endothelial cell proliferation that usually occurs in patients with coexistent systemic diseases. Histologic finding and clinical presentation are diverse, and it leads to difficulty in diagnosis. Understanding this disease may help us to prevent misdiagnoses and unnecessary treatments.

Method: A 73-year-old female, diagnosed with rectal cancer with lung metastasis and received operation and chemoradiation 2 years ago, presented 2 x 2 cm sized hyperkeratinized plaque on her right wrist (Fig. 1). She had pulmonary tuberculosis history and was being treated with rheumatoid arthritis. Punch biopsy was done and the result confirmed extremely well-differentiated squamous cell carcinoma. CT showed focal skin ulceration and tendon sheath involvement (Fig. 1).

Results: The authors planned for wide excision but we could not secure the enough margin because mass was too close to the tendon (Fig. 2). After excision, defect was covered by skin graft. Permanent finding confirmed intravascular reactive endothelial proliferation, suggestive of RAE and no malignancy (Fig. 3). Graft was well taken (Fig. 4).

Conclusion: RAE is a rare condition with unspecific features, so that is unfamiliar to most surgeons. If the preoperative biopsy specimen has no enough depth or width to represent the total lesion, we cannot achieve the correct diagnosis. RAE should always be kept in mind when patients have chronic infection, autoimmune disease, renal/hepatic failure and history of kidney/liver transplantation. Also clinical investigation and follow-up should be considered in case of RAE arising in the absence of apparent systemic disease.