Cutaneous lesions from lymphangioma circumscriptum

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A 32‐year‐old diabetic woman under evaluation for hypersplenism was evaluated extensively at other centres but the cause of massive splenomegaly was not diagnosed. The differentials considered were a myeloproliferative or a storage disorder. Her subsequent investigations showed a normal bone marrow examination with no evidence of abnormal cells. Liver function tests and blood results were normal. Magnetic resonance imaging (MRI) of the abdomen revealed multiple cystic lesions. She was offered diagnostic/therapeutic splenectomy but the patient was keen to ascertain the cause of splenomegaly preoperatively for which she sought further medical care at our hospital.
On clinical review, history of surgical management of a vascular malformation of the right chest wall as a child was obtained. There was a persistent swelling over the right chest wall and axilla (Fig. 1). She also had multiple clusters of tiny, translucent vesicles on her limbs and trunk which would resolve spontaneously with minimal hyperpigmentation (Fig. 2).
Although the MRI abdomen revealed multiple cysts, it was inadequate to diagnose the exact nature of the cysts – the differentials considered were lymphatic, venous or parasitic cysts. We proceeded with further imaging to secure a definitive preoperative diagnosis. An ultrasound duplex along with limited computed tomography (CT) of the abdomen was performed. They revealed no colour flow (ruling out a low/medium flow venous malformation) and no calcification/layering or daughter cysts (ruling out parasitic cysts).
A diagnosis of lymphangioma circumscriptum was made. The patient underwent splenectomy; histopathology confirmed the diagnosis. The cutaneous vascular malformation was managed conservatively.
Lymphatic cysts of the spleen are a rare differential diagnosis of massive splenomegaly.1 They are benign and present as isolated lesions or as a part of a systemic lymphangiomatosis involving the skin, liver, muscles and bone. Lymphangioma circumscriptum – the most common form of cutaneous lymphangioma – can involve visceral organs. These cysts are visible on CT or MRI abdomen – complete characterization requires administration of contrast to differentiate between the venous and lymphatic components. Ultrasound has a role in ruling out parasitic cysts and in combination with duplex studies rules out low flow venous/venolymphatic malformations. The splenic cysts can be micro‐ or macrocystic; many are diagnosed only post‐operatively on histopathology. The aetiopathogenesis is attributed to abnormal sequestration of lymphatic tissue during development. The differential diagnosis includes lymphoma, storage disorders, parasitic cysts, epithelial cysts and, very rarely, malignancy.3 The cutaneous malformation can be treated by multiple modalities, for example, CO2 laser, intralesional bleomycin or thermoablation by radiofrequency/laser.4
This patient had splenomegaly secondary to a multicystic lymphatic malformation of the spleen; the pointer to this diagnosis was the presence of a co‐existent cutaneous involvement.
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