Atypical Distribution of Angioma Serpiginosum: A Report of 2 Cases.

Sir,

Angioma serpiginosum, a rare vascular naevoid disorder caused due to ectatic dilatation of capillaries in the papillary dermis, is found almost exclusively in females (female:male 9:1)[1] Lesions usually consist of multiple small asymptomatic non-palpable deep red-to-purple punctata occurring in small clusters and sheets, and extension of lesions may produce serpiginious pattern. Truncal involvement is uncommon.[1]

Case Report 1

A 23-year-old unmarried female presented with complaints of asymptomatic progressive, flat, pinpoint, reddish skin lesions over the body for 4 years. They started from the abdomen, and progressed to involve the different parts of the body including the face. There was no history of photosensitivity, joint pains, fever, or recurrent oral ulcers. There was no history of abdominal pain, tingling sensation of extremities, and blurring of vision.

Case Report 2

A 19-year-old female presented to our dermatology department, with complaints of asymptomatic flat, red skin lesions over right arm, right breast and right upper back in linear and blaschkoid pattern for 10 years, and stable in pattern for 5 years.

General examination was unremarkable in both patients. On cutaneous examination, we found multiple non-blanchable, grouped, pinpoint, red macules distributed over the malar region of the face, bilateral breasts, bilateral arms, abdomen, upper back and bilateral lower limbs in the first patient [Figures 1 and 2], and in a blaschkoid pattern over right arm, right breast and right upper back in the second patient [Figure 3]. Nails, hair, and mucosae were unremarkable in both of the patients.

Figure 1

Generalized, numerous, pinpoint, reddish macules over malar area in first patient

Figure 2

Generalized, numerous, pinpoint, reddish macules over bilateral breasts, bilateral arms, and chest in first patient

Figure 3

Multiple, grouped, pinpoint, reddish-to-pinkish macules over right arm, right breast and, back in and blaschkoid pattern in second patient

The family history, personal, and past history were non-significant in either patient. Laboratory investigations like complete blood count, liver function test, renal function test, bleeding time, and clotting time were within normal limit in both patients, whereas serum antinuclear antibodies and ophthalmic examination of first patient was normal. Histopathological examination of skin biopsy from both patients revealed substantial increase in the number of dilated, slightly thick-walled capillaries within the papillary and upper reticular dermis. A few capillaries in superficial and deep plexus were also dilated. Sparse superficial perivascular lymphohistiocytic infiltrate was present. Overlying epidermis was unaffected [Figure 4]. Dermoscopy of lesions showed multiple red, oval-to-round lagoons [Figure 5].

Figure 4

(Hematoxylin & Eosin, ×40) Histopathology of the first patient revealed presence of substantial increase in number of dilated capillaries within papillary and upper reticular dermis. Overlying epidermis is normal

Figure 5

Dermoscopy revealed multiple red, oval-to-round lagoons (3Gen Dermlite DL4 Polarized Dermatoscope)

On the basis of clinical, histopathological, dermoscopic findings, a diagnosis of angioma serpiginosum was made in both the females.

Discussion

Angioma serpiginosum is a rare, benign, acquired vascular naevoid condition. It has female preponderance and mainly affects lower extremities and buttocks. It rarely undergoes complete spontaneous involution. It is a vascular anomaly with 90% of cases occurring in females, classically beginning in childhood and adolescence with 80% of cases occurring before 20 years of age.[2] Clinically, lesions are copper to bright red, punctate, non-blanchable or partially blanchable, grouped macules that may develop into papules with a background of erythema. Lesions enlarge by developing new lesions at the periphery, with clearing of lesions in the center, and this leads to a serpiginous or gyrate ring–like morphology.[3] Multiple red lagoons and pigment network in the absence of linear vessels are the most frequently observed alterations on dermoscopy.[4] No topical medications have proven effective in its treatment. Excellent therapeutic results have been achieved using pulsed dye laser.[5]

Our case showed generalized as well as linear and blaschkoid distribution of angioma serpiginosum. It also showed the usefulness of dermoscopy as a non-invasive method to diagnose this benign condition. Generalized angioma serpiginosum has not been reported till date, as per our limited knowledge.

Declaration of patient consent

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Conflicts of interest

There are no conflicts of interest.