Volume 1, Issue 2 , Pages 107-109, June 2006
Angiokeratoma circumscriptum of the dorsal tongue in a child
Article Outline
Summary
Angiokeratoma circumscriptum is a rare cutaneous vascular lesion normally localized to the lower extremities. There are two previous reports of angiokeratoma circumscriptum of the ventral tongue, both in male children, and neither case describes how the patients were treated. We present a 6-year-old boy with what we believe to be the first reported case of angiokeratoma circumscriptum of the dorsal tongue. The recurrent lesion was treated with surgical excision, with no recurrence of symptoms after 1-year of follow-up.
Keywords: Angiokeratoma, Oral cavity, Tongue
1. Introduction
Angiokeratoma is a term used to describe several distinct conditions that share a common histology: superficial dermal vascular ectasia with overlying epidermal hyperkeratosis. Angiokeratoma of Fordyce is the most common presentation, consisting of papules on the scrotum or vulva. The most lethal is angiokeratoma corporis diffusum, or Fabry's disease, an X-linked recessive error of metabolism presenting with lesions on the lower half of the body and the oral cavity. Angiokeratoma circumscriptum, usually present at birth on the lower extremities as multiple papular, plaque-like lesions, is the rarest. There are two reported cases of angiokeratoma circumscriptum of the ventral tongue in the literature [1], [2]. We present a case of angiokeratoma circumscriptum isolated to the dorsal tongue.
2. Case report
A 6-year-old male presented with a 2-year history of a recurrent tongue mass. Prior pathology reports defined the mass as an angiokeratoma. The patient had undergone two laser treatments by another physician using a handheld laser of unknown type, the most recent being 8 months prior to presentation, with temporary resolution of symptoms. The recurrent mass was once again enlarging. It was uncomfortable while eating, and bled and became inflamed when bitten. The patient denied history of local trauma. The past medical history was otherwise unremarkable.
On examination of the oral cavity the patient was found to have a 3
cm×4cm raised lesion anterior to the base of the tongue. The mass extended from the lateral aspect of the left side of the dorsal surface of the tongue onto, but not crossing, the midline (Fig. 1). The mass was non-friable, and moderately tender to palpation. The lesion was pink, raised, and covered with small punctuate black areas. There was some scar tissue surrounding the lesion consistent with prior laser procedures. The tongue moved normally. The remainder of the physical examination was non-contributory.
Fig. 1.
Angiokeratoma circumscriptum on the dorsal tongue.
After discussing all available options, the family elected to have the mass surgically removed. Two months after initial presentation, excision of the tongue mass was performed utilizing electrocautery and sharp dissection (Fig. 2, Fig. 3, Fig. 4). Hemostasis was achieved using electrocautery and purse string sutures around larger vessels with minimal difficulty; total blood loss was 30ccs.
Fig. 2.
Angiokeratoma circumscriptum, surgically excised specimen.
Fig. 3.
Post-excision lingual defect.
Fig. 4.
Dorsal tongue after reapproximation.
Microscopic examination of the specimen revealed submucosal ectatic thin-walled vascular channels filled with blood. Vascular spaces were intimately associated with or encased by squamous mucosa. Reactive epithelial hyperplasia with associated parakeratosis was present, all findings consistent with a histological diagnosis of angiokeratoma. The margins of the specimen were negative for local extension.
One year postoperatively, the patient was doing well. He denied swelling, dysphagia, voice changes, pain or recurrence of the mass. On examination of the tongue it was well-healed with no tenderness or appreciable masses on palpation.
3. Discussion
The etiology and pathogenesis of angiokeratoma circumscriptum is unknown. It is postulated that trauma or venous hypertension cause arteriovenous fistulas in the papillary dermis [3], [4]. The overlying epidermis reacts by undergoing acanthosis and hyperkeratosis.
Cutaneous angiokeratomas are the most frequent clinical manifestation of angiokeratoma corporis diffusum, Fabry's disease. These lesions are most often found in the periumbilical, groin, lumbo-sacral regions, and rarely in the oral cavity, by 10 years of age [5]. Histologically, the angiokeratomas of Fabry's disease demonstrate swollen, lipid-laden endothelial cells in the dilated dermal capillaries, distinguishing this lethal condition from angiokeratoma circumscriptum. The most common form of angiokeratoma is the Fordyce type. Characteristically localized to the scrotum or vulva, angiokeratoma of Fordyce has rarely been reported to involve the oral cavity [6], [7].
There are three reported cases of angiokeratomas solely localized to the oral cavity. Leung and Jordan [3] described the case of an 82-year-old man with a solitary angiokeratoma of the buccal mucosa. Interestingly, the other two cases were reported by the same group in India. Kumar et al. [2] related the case of a 16-year-old boy with histologically defined angiokeratoma circumscriptum on the ventral aspect of the tongue. More recently, Vijaikumar et al. described a 12-year-old boy with angiokeratoma circumscriptum isolated to the ventral tongue [1]. Both boys presented like our patient, with an enlarging lingual mass that occasionally bled without local trauma preceding the lesion's initial appearance or a history of systemic disease. Although including ours there are only three reported cases of lingual angiokeratoma circumscriptum, it is interesting to note that all are male pediatric patients.
Neither of the aforementioned cases of angiokeratoma circumscriptum of the tongue reported how the lesions were treated. Numerous reports in the literature describe successful treatment of cutaneous angiokeratomas with lasers, including copper vapor, potassium tritanyl phosphate, and argon [8], [9], [10]. Our patient's angiokeratoma of the tongue recurred despite two laser treatments. Surgical excision in this case has proven to be a more effective treatment modality. We speculate that angiokeratoma circumscriptum of the oral mucosa is less amenable to laser treatments than angiokeratoma circumscriptum of the skin. Angiokeratoma circumscriptum of the tongue may represent a unique clinical entity distinct from angiokeratoma circumscriptum usually localized to the lower extremities.
References
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- Angiokeratoma corporis diffusum (Anderson-Fabry disease) a case study. Ann. Dent. 1988;47(1):13–15
- Angiokeratoma of the scrotum (Fordyce type) associated with angiokeratoma of the oral cavity. Acta Derm. Venereol. 2002;82(3):208–210
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- . Angiokeratoms in Fabry's disease and Fordyce's disease: successful treatment with copper vapour laser. Acta Derm. Venereol. 1993;73:133–135
- . Angiokeratoma circumscriptum: successful treatment with the argon laser. Ann. Plast. Surg. 1988;20:183–190
PII: S1871-4048(06)00025-6
doi:10.1016/j.pedex.2006.01.001
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 2 , Pages 107-109, June 2006
