Linear verrucous hemangioma.

Abstract

A 7-year-old boy presented with multiple warty lesions on an erythematous background over the right upper extremity. These lesions had been present since birth as erythematous rash. These lesions increased in size and number as well as became warty as the boy grew up. The course of the disease was complicated by recurrent ulcerations, minor bleeding and infection in the lesions. Other history was noncontributory. Cutaneous examination revealed several well-circumscribed, hyperkeratotic erythematous plaques present in a linear fashion over the medial aspect of the right upper extremity (extending from tip of fingers to axilla) [Figure 1]. Similar lesions were present over medial part of dorsum of right hand and to some extent, the forearm. Some of these plaques had central ulceration (crateriform plaques). Of note, palmar lesions were devoid of any verrucous lesions on their surface. Both upper limbs were of equal length and girth. Clinically, angiokeratoma circumscriptum neviforme (ACN) and linear verrucous hemangioma (LVH) were considered as differential diagnoses. Routine blood investigations did not reveal any abnormality. Histopathological examination revealed epidermal hyperkeratosis, parakeratosis, and irregular acanthosis. Dermis showed numerous dilated blood vessels of various sizes extending down into the subcutaneous tissues [Figure 2]. A diagnosis of linear verrucous hemangioma was made and boy was referred to department of plastic surgery for further management.Figure 1: Hyperkeratotic erythematous plaques in a linear pattern. Note: Some of the verrucous plaques are crateriformFigure 2: Hyperkeratosis, parakeratosis, acanthosis, and increased number of vascular spaces throughout the dermis and hypodermis without any inflammation. (H and E, ×100) Higher magnification of epidermis and upper dermis (inset A, H and E, ×100) and hypodermis showing increased blood vessels (inset B, H and E, ×100)Verrucous hemangioma is a rare vascular malformation involving whole of the dermis and hypodermis, with a predilection for the lower extremities. It is usually present at birth, but it may present later in adulthood also. The lesions initially appear as bluish red, well-demarcated, soft, and compressible swellings. Later on, they become verrucous and tend to increase in size and number. Secondary bleeding and infection are frequent complications.[1] The linear or serpiginous form of verrucous hemangioma (LVH) is extremely rare, and only a few cases have been reported. It is not known whether linear lesions actually follow Blaschko's lines or the linear arrangement represents genetic mosaicism.[2] The most important differential diagnosis for linear form is ACN. Clinically, both conditions are indistinguishable from each other. The histologic features of both conditions are similar, as both lesions show vascular spaces just beneath a papillomatous and hyperkeratotic epidermis. However, in contrast to ACN, the vascular spaces in LVH also involve the lower dermis and subcutaneous tissues. Deep excision is the most preferred modality of treatment for verrucous hemangiomas, as a superficial therapeutic approach would be unsuccessful because of the deep angiomatous proliferation.[13] Recently, treatment with a combination of CO2 and dual pulsed dye laser (PDL)-Nd YAG laser has provided a satisfactory response in some cases.[3]