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.

Localized acquired lymphangiectasias after breast surgery: Enhanced non‐invasive diagnosis using dermoscopy and reflectance confocal microscopy

“…They clinically appear as clusters of asymptomatic, translucent, and flesh‐colored vesicular lesions; some of them may turn purple due to the presence of red blood cells . Lymphangioma circumscriptum is the most common congenital form whereas acquired lesions, known as lymphangiectasias, resulting from damage and/or obstruction of previously normal lymphatics, usually develop secondary to surgery, radiotherapy, and/or infections . In this case, clinical presentation may range from few, isolated vesicles measuring 2‐10 mm in diameter on normal‐appearing skin, to several, clustered, sometimes verrucous, lesions associated with diffuse lymphedema .…”

“…In 1956, acquired lymphangiectasias (ALs) were first described as a complication of radical mastectomy and several reports have been published so far . Diagnosis of ALs is usually clinical, although histopathologic confirmation is often required as they may mimic different disorders including epidermal nevi, angiokeratoma, basal cell carcinoma, cutaneous metastases, molluscum contagiosum, herpes virus infection, warts, and immunobullous diseases …”

“…1 Lymphangioma circumscriptum is the most common congenital form whereas acquired lesions, known as lymphangiectasias, resulting from damage and/or obstruction of previously normal lymphatics, usually develop secondary to surgery, radiotherapy, and/or infections. [2][3][4][5][6][7][8] In this case, clinical presentation may range from few, isolated vesicles measuring 2-10 mm in diameter on normal-appearing skin, to several, clustered, sometimes verrucous, lesions associated with diffuse lymphedema. 4 In 1956, acquired lymphangiectasias (ALs) were first described as a complication of radical mastectomy 9 and several reports have been published so far.…”

“…8,10 Diagnosis of ALs is usually clinical, although histopathologic confirmation is often required as they may mimic different disorders including epidermal nevi, angiokeratoma, basal cell carcinoma, cutaneous metastases, molluscum contagiosum, herpes virus infection, warts, and immunobullous diseases. 5,11 The introduction of non-invasive diagnostic tools such as dermoscopy and reflectance confocal microscopy (RCM) in the clinical practice has allowed us to enhance the diagnostic accuracy of several benign and malignant skin lesions along with several inflammatory and infectious cutaneous disorders. [12][13][14] Although different reports described the dermoscopic 15,16 and RCM 17,18 aspect of lymphangioma circumscriptum, the literature on AL is very scant.…”

Non‐invasive diagnosis of acquired lymphangiectases using optical coherence tomography

“…7 Acquired lymphangiectases can mimic various dermatological disorders, namely angiokeratoma, basal cell carcinoma (BCC), cutaneous metastases, warts and immunobullous diseases, so histopathological examination is sometimes needed. 8,9 Optical coherence tomography (OCT) is a non-invasive diagnostic method with a penetration depth around 1.0-1.5mm, producing real-time, in vivo, cross-sectional images of skin lesions. 10 OCT is used for various indications, such as diagnosis of BCC, psoriasis, immunobullous diseases and haemangiomas.…”