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INTRODUCTION

Angiokeratomas are telangiectasias with keratotic elements. They present in different clinical scenarios including (a) solitary or multiple angiokeratomas occurring predominantly on lower extremities; (b) angiokeratoma of Fordyce affecting the scrotum and the vulva; (c) angiokeratoma of Mibelli, an autosomal dominant disorder affecting dorsum of hands and feet, elbows, and knees; (d) angiokeratoma corporis diffusum associated with Fabry’s disease, an X-linked recessive disorder characterized by α-galactosidase-A deficiency and affecting the lower abdomen, buttocks, and genitalia; and (e) angiokeratoma circumscriptum usually grouped on one extremity.

EPIDEMIOLOGY

Age: solitary or multiple angiokeratomas usually affect young adults, angiokeratomas of Fordyce affect middle-aged and elderly individuals. Angiokeratoma of Mibelli and angiokeratoma circumscriptum are usually diagnosed in childhood.

Sex: angiokeratoma of Mibelli and angiokeratoma circumscriptum exhibit female predominance. Otherwise, there is no sex predisposition.

PHYSICAL EXAMINATION

Red to violaceous, well-circumscribed hyperkeratotic papules and plaques.

DIFFERENTIAL DIAGNOSES

Solitary lesions can be mistaken for melanoma, acquired hemangioma, lymphangioma, seborrheic keratosis, and warts.

LABORATORY DATA

Dermatopathology

Marked dilated, thin-walled blood vessels in the papillary dermis, associated with an overlying acanthotic hyperkeratotic epidermis.

COURSE MANAGEMENT

Management of angiokeratomas remains a challenge. Many modalities have been reported in the literature with variable success. Treatment modalities include

Figure 29.1

(A) Angiokeratomas on the abdomen of a young patient. (B) Angiokeratoma imaged through an epiluminescence microscope (DermLite)

  • Lasers: angiokeratomas have occasionally been treated successfully with lasers.

    • – The pulsed dye laser (PDL) is an effective device for the improvement of the vascular component of angiokeratomas, but frequently some keratosis remains. The target chromophore is hemoglobin. PDL has proven successful at 595 nm, 5-to-7-mm spot, 9 to 11 J/cm2, DCD 30/20. Covering the angiokeratoma with a glass slide, that is, diascopy, is helpful. The endpoint is lesional purpura. Healing occurs in more than 10 to 14 days. Multiple treatments may be required (Fig. 29.3).

Figure 29.2

Angiokeratoma on the left thigh resistant to multiple treatments with pulsed dye laser

Figure 29.3

(A) Biopsy-proven angiokeratoma on the thigh of a young child. (B) Some resolution after one treatment with pulsed dye laser at a wavelength of 595 nm with a 10-mm spot, pulse duration of 1.5 ms, a fluence of 7.5 J/cm2, and DCD 30/20

    • – Resurfacing lasers such as CO2 and Er:YAG lasers can be utilized for lesional vaporization. Patients generally require local infiltration with 1% lidocaine with or without epinephrine prior to treatment. ...

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