Lymphangioma circumscriptum of the male genitalia.

A 52-year-old male presented with clear fluid-filled lesions with discharge over the penis and scrotum since last 8 years [Figure 1]. There was no history of similar lesions in the family, any sexually transmitted disease, or surgical procedure performed on scrotum in the past. Routine hemogram was normal with normal eosinophil count. Ultrasonography of skin showed multiple tiny cysts in the upper papillary dermis without any subcutaneous component. Histopathology showed atropic epidermis with elongated rete ridges and multiple dilated lymphatic channels lined by single layer lining with lymphocytic infiltrate [Figure 2].

Figure 1

Multiple tiny skin-colored to translucent vesicles and papules over scrotum and penis

Figure 2

Grouped cystic lymphatic spaces present in the upper dermis lined with single layer lining with lymphocytic infiltrate (H and E, ×40)

Hence, on the basis of clinicohistopathological correlation, diagnosis of lymphangioma circumscriptum (LC) was made.

LC is congenital or acquired hamartomatous lymphatic malformation of the skin. It is the most common type of cutaneous lymphangioma; the other types are cystic hygroma, lymphangio-endothelioma, and cavernous lymphangioma. It is characterized by multiple translucent vesicles of different sizes, which contain clear lymph fluid, but it may become hemorrhagic. It may become grouped with verrucous changes which sometimes give warty appearance of the lesion. It can occur anywhere over the body, but commonly occurs over proximal extremity, shoulder, and neck. Involvement of genital is very rare. LC has superficial dermal and the deeper subcutaneous cisternal component.[1]

The pathogenesis of LC was first described by Whimster[2] in 1976 which states that LC arises from the collection of subcutaneous lymphatic cisterns which receive lymph from the surrounding tissue but fails to drain into the normal lymphatic system, as they are not connected to them; however, they are connected with the superficial lymph vessels. LC usually remains asymptomatic, but may be complicated by secondary bacterial infections and frequent discharge.

Definitive treatment is necessary for the improvement of cosmetic appearance and to prevent the complication of LC. Various treatment modalities are available such as radiofrequency ablation, sclerotherapy, ablative lasers, and surgical excision of both the superficial as well as deep components to prevent recurrence of lesion which is the most common postoperative complication with recurrence rate up to 25–50% in the 1st 3 months.[13]

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