Unilateral Chronic Ulcerated Nodules on the Leg of a Young Male

INTRODUCTION

Acroangiodermatitis is a benign disorder associated with various vascular pathologies ranging from congenital vascular malformation to acquired chronic venous insufficiency. Acroangiodermatitis of Mali (AAM) or pseudo-Kaposi sarcoma is a rare complication of venous insufficiency and usually presents in the older age group.^[1] It can mimic several benign conditions like tuberculosis verrucosa cutis, chromoblastomycosis, sporotrichosis, and malignant conditions like Kaposi Sarcoma, emphasising the role of histopathology in accurate diagnosis.^[2]

CASE REPORT

A 28-year-old male, an ironer by occupation, presented to the outpatient department of Dermatology with the complaint of swelling over the right leg and recurrent episodes of ulcerated nodules over a period of four years. The nodules developed spontaneously and gradually turned into verrucous, measuring about 2*3 to 4*5 cm in size with well-defined margins, overlying ulceration, and brown to black adherent crusts [Figures 1a-b].

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Figure 1a:

Lateral aspect of distal right leg showing multiple nodules with ulceration, erythematous granulation tissue, with hyperpigmentation and scaling of the surrounding skin.

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Figure 1b:

Medial aspect of distal right leg showing multiple nodules with ulceration, erythematous granulation tissue, and yellow to brown crusts with hyperpigmentation and scaling of the surrounding skin.

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Some lesions exhibited foul-smelling, scanty, purulent discharge. The surrounding skin showed hyperpigmentation. Pitting oedema was present over the distal aspect of both legs. All peripheral pulses were palpable. Dermoscopy of the lesion revealed a shiny white structureless area, four dot clods or white rosettes, white intersecting lines in a sieve-like pattern with red lacunae in between, and a yellowish crust indicating dermal fibrosis, narrowed adnexal orifices, and proliferation of blood vessels, respectively [Figure 2a].^[3]

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Figure 2a:

Dermoscopy of lesion showing shiny white structureless areas (black arrows), four dot clods or white rosettes (yellow circle), white intersecting lines in a sieve-like pattern with red lacunae in between (black circle), and a yellowish crust (red star). (Dermlite DL5, polarized, 10x).

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Histopathology of the ulcerated nodule revealed marked acanthosis with pseudoepitheliomatous hyperplasia and lobular proliferation of capillaries lined by plump cells with extravasation of red blood cells [Figure 2b].

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Figure 2b:

Histopathology of the lesion shows marked acanthosis with pseudoepitheliomatous hyperplasia (yellow star), lobular proliferation of capillaries lined by plump cells with extravasation of red blood cells (black arrow). ( Haematoxylin and eosin., 10x. )

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Lower limb arteriovenous doppler suggested bilateral varicose veins. Complete blood counts, liver and renal function tests, urinalysis, bleeding time, clotting time, enzyme-linked immunosorbent assay (ELISA) test for human immunodeficiency virus (HIV), and chest radiograph were within normal limits. Pus culture from exudate revealed growth of Acinetobacter.

On the basis of history, examination, dermoscopy, histopathology, and Doppler studies, the patient was diagnosed with acroangiodermatitis of Mali. The patient was advised to wear compression stockings, and ulcers were debrided. Calcium dobesilate 500 mg twice daily was started for venous stasis. Meropenem injection was given for Acinetobacter infection in accordance with the pus culture and sensitivity reports. Remarkable improvement was achieved at 4 weeks of follow-up without any adverse effects [Figures 3a-b].

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Figure 3:

(a) Lateral aspect of distal right leg showing resolution of nodules with post-inflammatory hypopigmentation and whitish scales. (b) Medial aspect of distal right leg showing clinical resolution of nodules with residual brownish crusts and post inflammatory hypopigmentation.

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DISCUSSION

Acroangiodermatitis of Mali (AAM), also known as pseudo-Kaposi sarcoma, was first coined by Mali in 1965.^[1] It is an uncommon complication of venous insufficiency and typically occurs in the elderly population.^[2] Venous hypertension, impaired calf muscle pump function, and tissue hypoxia can lead to venous stasis and endothelial injury, causing increased capillary permeability and leakage of blood components into surrounding tissues. These events promote inflammatory cell infiltration and neovascularisation, contributing to disease progression.^[4] It presents as macules and patches which progress into nodules or indurated plaques, located on the bilateral lower extremities with underlying oedema.^[2]

Four variants of acroangiodermatitis have been described: Stewart-Bluefarb syndrome associated with congenital arteriovenous malformation and arteriovenous shunts; Mali type associated with stasis dermatitis in older adults; acroangiodermatitis of pregnancy, and acroangiodermatitis due to arteriovenous shunts in patients of chronic renal failure and on haemodialysis.^[1]

Treatment consists of the correction of underlying pathology, which involves general measures like compression stockings, elevation of the lower limbs, and local wound care.^[5] Calcium dobesilate helps to prevent the progression by reducing the size and thickness of lesions by decreasing capillary permeability and strengthening the basement membrane of vessels.^[6] Other alternate therapies that have been tried include local corticosteroid preparations, oral erythromycin, or dapsone in combination with compression therapy.^[2]

Although acroangiodermatitis of Mali is classically reported as a bilateral condition occurring in older individuals, only a limited number of cases with unilateral presentation in the younger population, similar to our case, have been reported by Mehta et al.^[5] Chhabra G et al.^[7] , Ozkaya et al.^[8] and Heller et al.^[9] [Table 1]. In our patient, prolonged standing may have contributed to the development of varicose veins, thereby predisposing him to the condition.

CONCLUSION

While early-onset cases of acroangiodermatitis are typically associated with vascular malformations, our case stands out due to its unilateral presentation with underlying chronic venous insufficiency. Acroangiodermatitis of Mali, though benign, mimics Kaposi sarcoma. As the former is treatable with measures that manage chronic venous insufficiency, prompt reporting of the condition becomes important. Early and accurate recognition of acroangiodermatitis of Mali is crucial to prevent chronic symptoms and scarring, significantly improving the patient’s quality of life.