Acquired lymphangioma circumscriptum on the abdomen during pregnancy: A case report

Abstract

To our knowledge, acquired lymphangioma circumscriptum during pregnancy has only been reported on the vulva, and herein, we report a novel case of acquired lymphangioma circumscriptum during pregnancy on the abdomen. A 34-year-old woman presented with numerous papules draining clear fluid on her lower abdomen during the third trimester of her third pregnancy. She is healthy with no past medical history. Physical examination showed numerous clustered groups of hypopigmented flat-topped papules on the lower abdomen below the umbilicus. A punch biopsy showed epidermal acanthosis and hyperkeratosis and dilated lymphatic vessels in the superficial and upper dermis. Further imaging revealed no deeper lymphatic involvement and no underlying abdominal pathology. The favoured diagnosis was an acquired variant of lymphangioma circumscriptum on the abdomen, likely secondary to pregnancy. As the lesions were asymptomatic, no treatment was required. Dermatologists should recognize and differentiate this rare manifestation of acquired lymphangioma circumscriptum in pregnancy from other dermatoses.

Introduction

Lymphangiomas are rare benign disorders of lymphatic vessel malformation and proliferation. Lymphangioma circumscriptum (LC), the most common subtype of lymphangioma, involves lymphatic dilation of the upper dermis with possible extension to the subcutaneous tissue. ¹ LC is typically congenital but can be acquired, and its clinical presentation can be heterogeneous, with important diagnostic mimickers to consider. To our knowledge, acquired LC during pregnancy has been previously reported only on the vulva. ² Herein, we report a novel case of acquired LC on the abdomen during pregnancy.

Case report

A 34-year-old woman presented with numerous papules on her lower abdomen during the third trimester of her third pregnancy (Figure 1(a) and (b)). She described first noticing fluid drainage from the lower abdomen, and her partner subsequently noted dilated pores with associated drainage of clear fluid. The lesions were otherwise asymptomatic. She denies a history of similar lesions during her previous two pregnancies. An ultrasound performed at the time showed a 5 mm subcutaneous cyst. She is otherwise healthy with no past medical history. She has not had any previous radiation treatment or abdominal surgeries. Physical examination showed numerous clustered groups of hypopigmented flat-topped papules with some papules in a linear distribution on the lower abdomen below the umbilicus (Figure 1). The differential diagnosis included LC, lichen nitidis, and verruca.

Figure 1.

Clinical images of numerous clustered groups of hypopigmented flat-topped papules with some papules in a linear distribution (a) on the mid-abdomen at the linea nigra of a pregnant woman (b) under dermatoscopic lighting.

A punch biopsy was performed. Pathology showed dilated lymphatic vessels in the superficial and upper dermis as well as epidermal acanthosis and hyperkeratosis.

Based on the clinical and histopathological findings, the favoured diagnosis was an acquired variant of LC. Further imaging was done to rule out deeper lymphatic involvement and underlying pathology. An abdominal ultrasound showed a benign appearing 8 mm subcutaneous cyst in the abdominal wall, and a pelvic and transvaginal sonogram was unremarkable. As the lesions were asymptomatic, no treatment was required.

Discussion

To our knowledge, although previous case studies have reported LC on the vulva during pregnancy, ² this is the first reported case of acquired LC during pregnancy on the abdomen.

The pathogenesis of acquired LC is not well understood but is hypothesized to be secondary to lymphatic drainage obstruction that results in increased pressure and dermal backflow and subsequent dilatation of the upper dermal lymphatics. ³ Acquired LC is most commonly reported in the vulvar region secondary to pelvic surgery, radiation therapy, inflammatory bowel disease and infection, such as filariasis or tuberculosis.^1,4 Periumbilical lesions should raise suspicion for underlying intra-abdominal disease. ⁵ In this case, given that the clinical history and imaging were unremarkable for abdominal pathology, acquired LC on the abdomen is likely secondary to the increased physiological demands and hormonal changes during pregnancy that impair lymphovenous drainage. ⁶

Although LC typically presents with clusters of firm, translucent 2–4 mm vesicles resembling frogspawn ⁷ with a translucent pink hue due to fluid blood content, ¹ LC may also have heterogeneous clinical presentations with lesions appearing more nodular, papillomatous or verrucous. ⁴ As such, important diagnostic mimickers include condyloma acuminata, herpes zoster, molluscum contagiosum and leiomyomas, and these can be delineated via biopsy. ⁴ While LC is generally asymptomatic, some patients may experience pruritis, pain, burning, drainage and psychological distress. ⁷ The most common complications of LC include infection, as these lesions may act as portals of entry, and excessive fluid drainage leading to chronic lymphedema.^1,7 More rare complications include the development of squamous cell carcinoma and hidradenitis suppurativa ⁸ within LC lesions and lymphangiosarcoma following exposure to radiation therapy. ⁹

While the goal of treatment for LC is to remove all diseased lymphatics to prevent recurrence, existing treatments have limited efficacy with high recurrence rates and potential adverse events, and therapeutic options for pregnant patients are even more limited. Although wide local excision of the lymphatic channel has the lowest recurrence rate ~17%, ¹⁰ this should not be done in the expectant population. ² Minimally invasive therapies, such as sclerotherapy with sodium tetradecyl sulphate, have been reported as effective in non-pregnant women. ¹⁰ Cryotherapy is a therapeutic modality that can be used during pregnancy to treat LC. ² Overall, clinicians should consider the depth, size, location and extent of LC and the associated symptoms and potential for secondary complications when implementing therapeutic regimens.

To date, this is the first report of acquired LC on the abdomen in pregnancy. Dermatologists should recognize and differentiate this rare manifestation of acquired lymphangioma from other dermatoses that may have similar clinical presentations but require different management approaches. For pregnant patients who present with clustered verrucous vesicles or papules, a detailed clinical and pregnancy history and appropriate work-up should be performed.