Acquired widespread lymphangiectasia mimicking immunobullous disease: A case report

Abstract

A 76-year-old Caucasian woman presented with a 3-year history of a recurrent pruritic eruption on the hips, thighs, and under the breasts associated with intermittent lesions resembling vesicles and bullae that failed to respond to topical corticosteroids. She had a history of severe lichen sclerosis et atrophicus, leading to invasive squamous cell carcinoma of the vulva for which she underwent radical vulvectomy and bilateral inguino-femoral lymph node dissection. On physical examination, involving the inframammary breasts, abdomen, hips, and proximal thighs there were multiple erosions with hemorrhagic crust and multiple clustered translucent papules. 4+ pitting and nonpitting edema were present on both legs. Biopsies were consistent with acquired lymphangiectasia. Acquired lymphangiectasia can be difficult to identify clinically. In our case, the unusually widespread distribution was morphologically reminiscent of immunobullous disease. The extensive surgical disruption to the patient's lymphatic system was likely responsible for this unique presentation.

Keywords: General dermatology; acquired lymphangiectasia; squamous cell carcinoma.

Figure 1.

Abdomen: multiple clustered translucent and hemorrhagic papules morphologically reminiscent of vesicles.

Figure 2.

Thigh: multiple erosions with hemorrhagic crust and multiple clustered translucent papules.

Figure 3.

4×. Superficial dermal thin-walled dilated vascular channels surrounded by hyalinized collagen.

Figure 4.

10×. Thin-walled, dilated vascular channels surrounded by hyalinized collagen and scant perivascular lymphohistiocytic inflammation.