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Indian Journal of Dermatology, Venereology and Leprology, Vol. 74, No. 2, March-April, 2008, pp. 188

Department of Dermatology and Venereology, Uttarakhand Forest Hospital Trust and Medical College, Haldwani, Nainital, Uttarakhand
Correspondence Address:Department of Dermatology and Venereology, UFHT and Medical College Haldwani (Nainital) Uttarakhand - 263 139, imsag@rediffmail.com

A ten year-old boy presented with diffuse enlargement of the tongue since birth. The swelling was asymptomatic except for occasional, transient bleeding over the last two years. He did not have any other cutaneous/systemic symptoms. Clinical examination revealed diffuse enlargement of the tongue, which was soft and nontender. He also had numerous, 1-5 mm-sized vesicles on the dorsal surface of the tongue, some of which were hemorrhagic [Figure - 1]. The ventral surface had fewer vesicles. A few longitudinal fissures were also seen on the proximal part of dorsal surface of the tongue. Systemic examination did not reveal any abnormality. The histopathological findings are shown in [Figure - 2]A and B.

Diagnosis: Lymphangioma -
Circumscriptum of the Tongue -Circumscriptum of the Tongue

Histopathology showed multiple dilated lymphatics in the papillary dermis. Intraluminal lymph could be seen in these dilated lymphatic channels, which were lined by flat endothelial cells. Red blood cells and lymphocytes were also present in most of the dilated lymphatics.

Lymphangioma circumscriptum is a congenital malformation of the superficial lymphatics, which may present at any age but is usually noted at birth or appears during childhood. Clinically, the condition manifests with fluid-filled vesicles, which may be well defined and discrete or grouped into structures resembling frog’s spawn. The lymphangioma may be translucent when the overlying epidermis is very thin or they may vary in color from red to blue-black when containing blood as seen frequently. The most common sites are the axillary folds, shoulders, flanks, proximal parts of the limbs and perineum. Lymphangioma circumscriptum of the tongue is uncommonly reported.[1,2] Lymphangioma circumscriptum of the tongue may cause speech disturbances, difficulty in eating and swallowing, episodes of choking or dyspnea and severe hemorrhage. Whimster[3] described the pathology of lymphangioma circumscriptum as a collection of subcutaneous lymphatic cisterns with a thick muscular coat communicating through dilated channels with the superficial vesicles. This explains the tendency of the lesions to recur after superficial excision. The etiology of lymphangioma circumscriptum is not clear; however, malformation of the lymphatics has been suggested as a possible cause.[4,5] Histopathologically, it shows dilated lymphatics (either solitary or grouped) particularly in the papillary dermis containing lymph or blood. The epidermis overlying the vesicle appears to be atrophic with elongation of rete ridges due to which the vesicles may appear intraepidermal.

It is an important cause of macroglossia in children and the clinical differential diagnosis includes vascular malformation, hemangioma, neurofibroma, Down’s syndrome, mucopolysaccharidosis and hypothyroidism. It can easily be differentiated by the presence of superficial, tiny vesicles with or without hemorrhage. There is no satisfactory treatment for this condition. Surgical excision, carbon dioxide laser ablation, liquid nitrogen and injectable sclerosant have been used with variable results but lymphangioma has a high recurrence rate with most of these modalities.

Histopathological findings

Discussion

Lymphangioma circumscriptum is a congenital malformation of the superficial lymphatics, which may present at any age but is usually noted at birth or appears during childhood. Clinically, the condition manifests with fluid-filled vesicles, which may be well defined and discrete or grouped into structures resembling frog′s spawn. The lymphangioma may be translucent when the overlying epidermis is very thin or they may vary in color from red to blue-black when containing blood as seen frequently. The most common sites are the axillary folds, shoulders, flanks, proximal parts of the limbs and perineum. Lymphangioma circumscriptum of the tongue is uncommonly reported.^[1],[2] Lymphangioma circumscriptum of the tongue may cause speech disturbances, difficulty in eating and swallowing, episodes of choking or dyspnea and severe hemorrhage. Whimster^[3] described the pathology of lymphangioma circumscriptum as a collection of subcutaneous lymphatic cisterns with a thick muscular coat communicating through dilated channels with the superficial vesicles. This explains the tendency of the lesions to recur after superficial excision. The etiology of lymphangioma circumscriptum is not clear; however, malformation of the lymphatics has been suggested as a possible cause.^[4],[5] Histopathologically, it shows dilated lymphatics (either solitary or grouped) particularly in the papillary dermis containing lymph or blood. The epidermis overlying the vesicle appears to be atrophic with elongation of rete ridges due to which the vesicles may appear intraepidermal.

It is an important cause of macroglossia in children and the clinical differential diagnosis includes vascular malformation, hemangioma, neurofibroma, Down′s syndrome, mucopolysaccharidosis and hypothyroidism. It can easily be differentiated by the presence of superficial, tiny vesicles with or without hemorrhage. There is no satisfactory treatment for this condition. Surgical excision, carbon dioxide laser ablation, liquid nitrogen and injectable sclerosant have been used with variable results but lymphangioma has a high recurrence rate with most of these modalities.

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