DOI: 10.36347/sjmcr.2024.v12i04.032

Pages: 520-523

Introduction: Klippel-Trénaunay syndrome is a rare congenital vascular disorder characterized by a triad of cutaneous capillary malformations, varicosities and hypertrophy of soft tissues and overgrowth bones. Among the cutaneous malformations are angiokeratomas, which appear as a papule from 2 to 10 mm in diameter, usually dark red in color, although they may be blue or black, and their surface is keratotic on palpation. Angiokeratomas pose a therapeutic challenge, especially in cases of multiple or extensive lesions. Laser modalities have demonstrated efficacy and may be successful treatment options. Clinical Case: A 13-year-old male patient with a clinical history of Klippel-Trénaunay syndrome diagnosed at birth. He came to the outpatient services because he presented dermal nodular lesions at the inguinal region, painful on palpation, with a duration of one week. In addition, he had an increase in diameter of the left lower limb with multiple varicose veins, ochre dermatitis on the inner side of his left leg. On the right lower limb there are violaceous patches with the presence of euchromic papules grouped together, of hard consistency at the left knee region. He also presents multiple violaceous keratotic papules on the scrotum, thigh and left knee bleeding on manipulation and trauma. Skin excision was performed in the right inguinal region for biopsy, which reported macroscopically fibrous, multinodular element measuring 0.7x0.5x0.3cm, whitish surface of firm consistency and whitish heterogeneous surface upon sectioning. Microscopic inspection was carried out with hematoxylin and eosin technique, which reported skin with epidermis showing acanthosis, hyperkeratosis, papillary dermis with congestive dilated vessels, negative for malignancy and compatible with angiokeratoma. Among the treatment options, it was decided to initiate CO2 laser therapy with satisfactory results. Conclusions: Klippel-Trénaunay syndrome is an extremely rare vascular pathology ........