Late onset bilateral angioma serpiginosum involving upper limbs in a male: a rare presentation

Angioma serpiginosum (AS) is a rare vascular disorder characterized by punctate red macules in a nevoid distribution arranged in groups to form a serpiginous pattern [1]. The disease is usually seen in late childhood in females and mostly occurs unilaterally over the lower extremity.
We report a case of bilateral AS on the upper extremities and chest in a 22-year-old male patient. Late onset (over 20 years of age), bilaterality, male sex and involvement of upper extremity add rarity to our case presentation and warrant its reporting.
A 22-year-old male presented with asymptomatic, red coloured lesions over the right side of chest and both arms. The lesions initially began on the chest one year back and gradually spread to involve the right and left arms. The patient was otherwise apparently healthy, and no family history of a similar disease was present. On physical examination, he was found to have multiple, punctate, grouped, red macules over the anterolateral chest on the right side, extending from just above the nipple to the anterior axillary fold and both upper arms up to the mid-forearms in a serpiginous pattern (Figs. 1 A–C). The lesions were not blanchable on diascopy. However, Darier’s sign was negative. The rest of the cutaneous and systemic examination was unremarkable. Laboratory tests including blood counts and serum biochemistry were within the normal range. Serum testosterone and oestrogen levels were normal. Skin biopsy from the chest showed normal epidermis and dilated tortuous vessels in the upper dermis and dermal papillae, without inflammation or pigment deposition (Fig. 2). Toluidine blue stain for mast cells did not reveal any infiltrating mast cells. Based on the clinical features and histopathology, the diagnosis of AS was made. The patient refused treatment after learning about the benign nature of the disease.
Angioma serpiginosum was first described in 1889 by Hutchinson as an infective nevoid disease and was named by Crocker a year later [2]. Around 90% cases are seen in females, with 80% of all cases occurring under 20 years of age [2, 3]. Familial cases have been documented. Angioma serpiginosum involves the lower extremities in most cases, but any site can be involved with usual sparing of the mucocutaneous junctions, palms and soles. Involvement of the eye and nervous system has been reported, too [4]. Involvement in a Blaschkoid pattern may be seen [5]. Bilateral involvement is usually seen in those...


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