Dermoscopic Features of Cutaneous Lymphangioma Circumscriptum of the Scrotum
Lymphangioma circumscriptum (LC) is a rare, benign skin disorder characterized by hamartomatous malformations of deep dermal and subcutaneous lymphatic channels. Cutaneous lymphangioma circumscriptum (CLC) is the most common form of this condition, but it rarely occurs in the scrotum. The ability of dermoscopy to aid clinicians in diagnosing CLC has been explored in previous studies, and this case report highlights the unique dermoscopic features of scrotal LC.
A 19-year-old male patient presented with a 5-year history of asymptomatic, multiple fluid-filled lesions on the scrotal skin. Occasionally, these lesions would rupture, discharging serous fluid. Over the past year, the patient underwent surgical excision of the affected area without a prior biopsy. However, the lesions recurred within months. The patient had no history of sexually transmitted diseases, radiotherapy damage, or infections. Physical examination revealed the entire scrotum covered with multiple white vesicles of varying sizes, ranging from 1 to 3 mm². Some vesicles exuded viscous serous fluid upon puncture, and the vesicle spaces were filled with pale yellow crusts. The underlying scrotal skin was diffusely thickened. No associated lymphadenopathy or lymphedema was observed. Ultrasonography of the scrotum indicated hydrocele of the left testis, with edema and thickening of the scrotal wall.
Dermoscopic examination revealed densely distributed white or buff-colored lacunar areas surrounded by pale yellow or yellow-white structures, ranging from 1 to 3 mm². Reddish to purplish or mauve homogeneous pigmented spots were observed in the lower part of the lacunae. The area exhibited a white or yellowish mulberry-like appearance. Histopathological analysis showed papillated epidermal hyperkeratosis and multiple dilated lymphatic vessels lined by a thin wall of endothelial cells, with an inflammatory infiltrate in the papillary dermis. These lymphatic vessels were filled with lymphatic fluid, and the surrounding collagen fibrils showed significant hyperplasia. Some vessels also contained variable concentrations of red blood cells and eosinophilic proteinaceous substance. Immunohistochemical staining was positive for D2-40 and CD31 but negative for CD34.
CLC is an uncommon benign disorder of the lymphatic channels, typically congenital but can arise at any age. Acquired LC may develop later in life due to injury to deep collecting lymphatics caused by radiotherapy damage or infections such as filariasis, lymphogranuloma venereum, or tuberculosis. While CLC commonly appears on the upper body, it is rare in the genital area. Clinically, it presents as persistent, multiple clusters of translucent or hazy vesicles containing clear lymph fluid, sometimes with hemorrhage. In the genital area, these vesicles may be misdiagnosed as molluscum contagiosum, condyloma acuminata, or idiopathic calcinosis cutis. A definitive diagnosis is usually made through biopsy, which reveals greatly dilated lymphatic channels in the upper dermis filled with lymphatic fluid, erythrocytes, lymphocytes, macrophages, or neutrophils.
Dermoscopy has significantly improved the diagnostic accuracy for CLC. Dermoscopically, CLC typically displays two distinct patterns: yellow lacunae surrounded by pale septa without blood inclusion and yellow to pink lacunae alternating with dark-red or bluish lacunae due to blood inclusion. These findings correlate well with histologic features, where the former pattern is characterized by dilated dermal or subcutaneous lymphatic vessels filled with lymphatic fluid, and the latter includes variable concentrations of red blood cells. Some reports describe a “hypopyon-like feature,” where colored areas vary from yellowish-white to diffuse rose with linear and sharp vessels.
In this case of scrotal LC, dermoscopy revealed densely distributed white and yellowish lacunar areas with red-violet spots in the lower portion, separated by pale yellow or yellowish-white structures. The area resembled a white or yellowish mulberry, differing from the typical transparent or translucent appearance of vesicles in common sites. This difference may be attributed to the hyperplasia of collagen fibers around the lymphatic vessels, consistent with reports that the histopathological correlation of white lines could be the presence of fibroplasia in CLC cases. Linear or sharp vessels were not observed, and the pale yellow and yellow-white structures around the lacunae may correspond to eosinophilic keratin in pathology.
In conclusion, the presence of lacunae, vascular structures, and hypopyon-like features are critical dermoscopic criteria for diagnosing CLC. However, in scrotal LC, the dermoscopic features may differ, highlighting the importance of considering anatomical variations in diagnosis. This case underscores the utility of dermoscopy in identifying unique features of CLC in rare locations, aiding in accurate diagnosis and appropriate management.
doi.org/10.1097/CM9.0000000000000957
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