Angiokeratomas on male genital lichen sclerosus: Are they related?

Sir,

Lichen sclerosus (LS) is a common inflammatory dermatosis with a predilection for anogenital skin. Adult anogenital LS is 10 times more common in women than men. Male genital LS presents with symptoms such as pruritus, dyspareunia and urinary dribbling, and signs such as lichenoid and zoonoid balanoposthitis, sclerosis, structural effacement, adhesions and anatomic naviculo-meatal abnormality. Predominant purpura, angiokeratomas, bullae, erosions, and ulceration have been reported.[1] Angiokeratomas are vascular lesions which are defined histologically as one or more dilated blood vessels lying directly subepidermal and showing an epidermal proliferative reaction. We report a very rare association of LS with angiokeratomas.

A 27-year-old male presented with white lesions on genitalia and secondary phimosis of 2 years duration. The lesions were asymptomatic except for occasional tearing and splitting of the foreskin after sexual intercourse or masturbation. Examination revealed an ivory-white atrophic plaque circumferentially over the foreskin causing phimosis [Figure 1a] and a similar atrophic white plaque over the scrotum [Figure 1b]. There were multiple small 1–2 mm discrete reddish-purple papules at the margins of the scrotal plaque as well as on the surrounding normal scrotal skin [Figure 1b]. Dermoscopy of the white plaques showed white structureless areas, white chrysalis-like areas, and branching vessels. Dermoscopy of reddish-purple papules showed red lacunae separated by whitish veils [Figure 2]. Based on clinical findings and dermoscopy, the diagnosis of genital LS with angiokeratoma-like papules was established.

Figure 1.

Ivory-white atrophic plaques of lichen sclerosus around the foreskin causing phimosis (a) and on the scrotum (b) with multiple small 1–2 mm discrete reddish-purple angiokeratoma-like papules present over the plaque as well as on the surrounding normal skin (yellow circles)

Figure 2.

Dermoscopy of the white plaque of lichen sclerosus showing white structureless areas (red arrow), white chrysalis-like areas (green circle), and branching vessels (blue arrow). Dermoscopy of reddish-purple angiokeratoma-like papules showing red lacunae separated by whitish veils (yellow circles) (DermLite DL3N; wet, contact, polarized, ×10)

LS can be associated with organ-specific autoimmune diseases, atopy, obesity, trauma, and extracellular matrix protein-1 (ECM-1) autoreactivity. It has also been found to be associated with infections such as human papillomavirus, borreliosis, and hepatitis-C. Chronic exposure of occluded susceptible epithelium to urine is the main pathological trigger for male genital LS. Histopathology shows atrophic epidermis, hyalinization of superficial dermis, and band-like lymphohistiocytic infiltrate in the deeper dermis.

Bullous, hemorrhagic and telangiectatic variants of LS have been described to occur over the limbs and trunk, but the association between genital LS and angiokeratomas is underreported. They have been incorrectly mentioned as two separate conditions requiring different treatment approaches in recent literature.[2]

Dermoscopy of LS shows whitish background and white-yellowish to milky-pinkish patchy structureless areas, corresponding to sclerosis and hyalinization on histopathology. Other dermoscopic features include reduced vessel density, called as vascular desertification, polymorphic linear and dotted vessels, red purpuric dots, globules or blotches, gray-blue dots in a peppering pattern, scales, and yellow comedo-like openings.[3] Autoantibodies against ECM-1 affect the vessel walls, leading to vascular changes during the early stage of the inflammation, whereas at a later stage, fibrosis outweighs the vascular changes. However, dermoscopic and histopathological vascular ectasia in male genital LS is independent of the disease duration, due to the high vascular supply of the genital skin.[4] Dermoscopy of angiokeratoma shows well-demarcated red lacunae, corresponding to wide dilated vascular spaces in the dermis and whitish veil, corresponding to hyperkeratosis and acanthosis.[3,5]

Autoantibodies to ECM-1 are responsible for the histopathological evidence of vasculitis and thickening of the blood vessel walls. This results in hypoxia and ischemia with subsequent cellular and vascular damage. Both, the vessel wall changes, and the hyalinization and weakening of the dermis, ultimately give rise to ectatic thin-walled vascular spaces in the papillary dermis intimately associated with the epidermis. These are clinically seen as angiokeratoma-like papules in association with LS.[6]

Therefore, angiokeratoma-like papules in LS are a secondary feature. Various factors such as local trauma, increased venous pressure, degenerative changes in the elastic tissue of the vessel wall, and the surrounding supportive tissue, as well as abnormalities in the extracellular protein network, are implicated in the pathogenesis of angiokeratoma-like papules in male genital LS.[6]

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.