Late-onset Segmental Angioma Serpiginosum

Sir,

Angioma serpiginosum is an uncommon dermatosis, which classically affects the small vessels of the upper dermis, with a characteristic clinical appearance. It is commonly found in females, with a predilection for the lower limbs. Hereby, we report a case of segmental angioma serpiginosum over the chest in a middle-aged man. The unusual features of the late-onset disease, segmental distribution, and truncal involvement prompted us to report the case.

A 35-year-old man presented with asymptomatic, persistent, red-brown rash on the left chest, which was present over the preceding 2 years. There was a definite history of the appearance of new lesions at the periphery, along with fading of the lesions toward the center. He had no other complaints. Cutaneous examination revealed multiple erythematous macules and papules on the left side of the anterior chest [Figure 1]. There was no extension to the back. The lesions were arranged in a band-like pattern over the T2 dermatome. Besides, there were a few discrete lesions, both above and below. The macules were blanchable on diascopy. Rest of the mucocutaneous examination was noncontributory. Medical, surgical, and family history was not significant for any disease. In the absence of dermoscopy, a skin biopsy was taken from a representative lesion. Histopathology findings from both macule and papule were similar and showed dilated capillaries with a thickened wall, underneath an unremarkable epidermis. Inflammatory changes, hemorrhage, and hemosiderin deposits were notably absent [Figure 2]. Considering the clinical and histopathological findings, a diagnosis of angioma serpiginosum was made. The patient has been counseled about the benign nature of the disease and referred for pulse dye laser (PDL).

Figure 1.

Erythematous macules and papules, in a band-like distribution along T2 dermatome. Besides, discrete lesions can be noted

Figure 2.

Photomicrograph showing dilated capillaries with a thickened wall (H and E, ×40)

Angioma serpiginosum was described by Hutchinson in 1889, and the nomenclature was given by Crocker in 1894.[1] It is characterized by multiple grouped punctate telangiectatic macules, having a tendency to become papular over time. New lesions appear at the periphery and the ones at the center fade. Palms, soles, and mucocutaneous junctions are characteristically spared.

Two types of presentations have been documented. One is the diffuse, nonsegmental type, which is inherited in an autosomal dominant pattern. On the other hand, segmental or zosteriform angioma serpiginosum is a sporadic dermatosis.[2] Our case belongs to the latter category.

The disease occurs predominantly in females (90%), and usually starts in childhood. The commonly affected sites are the lower limbs and buttocks. Truncal involvement is uncommon.[3] However, in our case, the lesions were confined to the trunk. The histological picture consists of dilated and tortuous capillaries in the upper dermis; consistent with the findings in this report. Dermoscopy can be helpful in diagnosis by demonstrating “numerous small, relatively well-demarcated, and round to oval red lagoons.”[4] The closest clinical differentials are pigmented purpuric dermatosis and unilateral nevoid telangiectasia. Dermoscopy in pigmented purpuric dermatosis shows multiple purpuric globules over an orange-brown background. Telangiectasias are seen as dilated linear and branched vessels under a dermoscope. However, this diagnostic test could not be performed due to unavailability of resources.

Angioma serpiginosum is thought to be the result of congenital hyperplasia or ectasia of preexisting superficial dermal capillaries. They may represent type I mosaicism. Kumakiri et al. found that the walls of the capillaries were characterized by abundant fine fibrillar material admixed with collagen fibers. It was concluded that angioma serpiginosum is a type of capillary nevus, which manifests as dilatation and proliferation of capillaries.[3]

The clinical differential diagnoses were angiokeratoma, unilateral nevoid telangiectasia syndrome, pigmented purpuric dermatoses, etc., Based on clinical and histological findings, the other possibilities were excluded. Angiokeratoma is characterized by hyperkeratosis and papillomatosis. Pigmented purpuric dermatoses show hemosiderin deposition, extravasation of erythrocytes and features of inflammation. These were absent in our case. Unilateral nevoid telangiectasia is manifested with blanchable telangiectatic macules. Our patient had macules as well as clinically palpable papules. Besides, histology shows thin-walled dilated capillaries in the dermis. However, the histological picture in our case was characterized by dilated capillaries with a thickened wall. Based on these findings, our diagnosis was more in favor of angioma serpiginosum.

Atypical presentations include disseminated lesions,[5] involvement of the sole,[6] association with cherry angioma[7] and angiokeratoma.[8] Our case adds to the armamentarium of diversities of presentation of the disease.

Partial or complete spontaneous regression of the lesions may occur. PDL[9] and 532 nm potassium-titanyl-phosphate lasers[10] have been used in the treatment of angioma serpiginosum.

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Conflicts of interest

There are no conflicts of interest.