Dermatology Online Journal

Spontaneous acroangiodermatitis in a young woman
Noelyn Anne Hung ¹, Mathew Strack ², Andre Van Rij ³, Colin John North ⁴, and John Bartlett Blennerhassett ¹
Dermatology Online Journal 10 (2): 8

1. Healthlab Otago, Dunedin Hospital, Dunedin, New Zealand 2.Department of Medicine, Dunedin School of Medicine University of Otago, New Zealand 3. Department of Radiotherapy, HealthCare Otago, Dunedin Hospital, Dunedin, New Zealand 4. Department of Surgery, Dunedin School of Medicine, University of Otago, New Zealand. noelynh@healthotago.co.nz

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Abstract

We present a case of acroangiodermatitis that for nearly 15 years was unrecognized and treated with skin grafting and radiotherapy. This case is also unusual in that neither venous insufficiency nor an underlying arteriovenous malformation could be demonstrated. Acroangiodermatitis is infrequently reported; a review of the literature emphasizes the similarities to Kaposi sarcoma both clinically and histologically. Because treatment for Kapsoi's sarcoma and hemangioendothelioma involves skin grafting and radiotherapy, awareness of this entity by dermatologists, surgeons, and pathologists is important.

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Introduction

Acroangiodermatitis was first referred to as angiodermite de Favre et Chaix in the French literature [1, 2, 3], and later as congenital dysplastic angiopathy, pseudo-Kaposi sarcoma [4] or acral capillary angiomatosis [5]. Reports by Blufarb and Adams [6], and Waterson et al. [7] also noted angiomatous dermatosis of the leg simulating Kaposi sarcoma with an underlying arteriovenous fistula. Mali et al. [3] coined the term acroangiodermatitis that is favored in recent literature. This lesion is still poorly known despite the reports dating back to 1921 [1].

Acroangiodermatitis usually affects the toes and feet of young adults, presenting as purplish-red nodules and plaques forming an irregular lesion that may measure several centimeters in size. Pain and recurrent ulceration can be troublesome. The lesion may first appear in childhood. An arteriovenous malformation or other form of venous insufficiency is usually demonstrated clinically or radiographically in the involved limb.

We present the clinicopathologic features of a case of acroangiodermatitis in a young white female; this lesion was clinically and pathologically confused with an aggressive vascular tumor and treated with skin grafting and radiotherapy. This case is also unusual in that neither venous insufficiency nor an arteriovenous malformation could be clinically demonstrated in the involved limb.

Clinical synopsis

At age 21, a woman sought medical advice for an intermittently tender lesion of the skin of the left foot, which had changed little since appearing 2 years earlier. The patient's medical history is complicated by epilepsy. Since age 15, she has been dependent on anticonvulsant therapy (phenytoin and carbamazepine). An angiotensin-converting enzyme inhibitor was also prescribed at age 15 when a temporal association was noted between her menses and seizures (catamenial epilepsy). Mild hypertension was noted during medical examinations and the therapy continued. An episode of severe iron-deficiency anemia attributed to menorrhagia was reversed with iron therapy and oral contraceptive use.

The lesion at presentation was described as an irregularly pigmented purple rash 30 mm by 20 mm. The attending surgeon diagnosed a venous flare and prescribed compression bandaging and hydrocortisone cream. A year later at surgical followup the lesion had significantly improved. At age 23 (2 years later), the patient complained of chilblains, and a diagnosis of Raynaud phenomenon was entertained. The rash was not noted at that time.

At age 31, the dusky red irregular rash recurred in the same site, and the patient was seen by a dermatologist who suspected an atypical hemangioma and performed a punch biopsy. The pathological diagnosis rendered was capillary hemangioma. Two years later (at age 33), when the lesion had ulcerated, another dermatologist examined the lesion and described it as a "congenital vascular lesion similar to a pyogenic granuloma". A cortisone cream was prescribed, and 3 weeks later the lesion was noted to be "healing very nicely." However, a month later, measuring 30 mm by 22 mm, the lesion ulcerated again, and the patient was unable to continue her work as a kitchen hand. The patient complained that the lesion was a nuisance and requested surgical removal and skin grafting, which were performed shortly after. This time the pathological diagnosis was aggressive hemangioendothelioma, although unusual pathological features were noted.

Three months following surgery and grafting the patient experienced increasing pain at the site, and the scar had darkened. The darkening consisted of increased pigmentation at the margin of the skin graft, with a few similar spots within the grafted area (Fig. 1). Colleagues consulted locally and internationally recommended radiotherapy.

Figure 1
Graft 3 months following surgery, with darkening edges and patches within

Following radiotherapy the pain lessened, but by the time the patient was age 35 the area of pigmentation recurred measuring approximately 20 mm by 22 mm. Because of continuing pain and difficulty wearing shoes, the patient could still not work. Further grafting was performed and radiotherapy given.

A year later the grafted area was described as "extensively erythematous with a small ulcer". The lesion was red, itchy, and painful. The ulceration persisted despite topical antibiotics. A year later, at the time of writing this report, the ulcer is healing but a fine purple ribbon surrounds the graft. Review of the re-excised graft and previous biopsies resulted in the diagnosis of acroangiodermatitis.

Pathologic findings.—Sections of formalin fixed tissue were routinely processed and stained with H&E. Estrogen and progesterone receptors were analyzed using a routine streptavidin-biotin-peroxidase method, in addition to a monoclonal antibody to the CD34 antigen (all DakoCytomation antibodies).

Figure 2
Lamellar myxoid fibrosis of collagen (right) surrounding the proliferation of small vessels. Magnification x200.

The initial punch biopsy demonstrated a proliferation of small capillaries with minimal intervening stroma. The stroma contained hemosiderin deposition and ulceration, interpreted as evidence of mechanical trauma. The first excisional biopsy, prior to radiotherapy or grafting, demonstrated widely distributed lobules of mitotically active endothelium-lined small vessels in the superficial dermis. The dermis contained hemosiderin granules and showed a lamellar myxoid fibrosis. The epidermis appeared normal. A diagnosis of aggressive angioendothelioma was rendered, and pathologist commented on the high possibility of local recurrence but probable negligible metastatic potential.

Figure 3
Re-excised graft with similar proliferation of small vessels. Magnification x400.

Re-excision of the initial graft showed similar features (Fig. 3), and excision of the lesion appeared complete. Microscopic examination of the second graft demonstrated similar features with scarring, and endothelial atypia attributable to radiation effect. Abnormally thickened arterial vessels were noted in the deep dermis, and the vascular lesion appeared to extend into the deep dermis and vertical resection margins. Estrogen and progesterone receptors were negative, and CD34 antibody highlighted the luminal endothelial cells.

Discussion

The clinical and pathological difficulty of establishing a diagnosis of acroangiodermatitis has been discussed for over 35 years, principally in the dermatological literature. The similarity of this lesion to Kaposi sarcoma has been well documented [8], and in 1987 a further paper by Strutton and Weedon [9] emphasized the importance of this diagnosis in the light of the association of Kaposi sarcoma with the AIDS epidemic.

Venous insufficiency appears to be the common pathophysiologic mechanism [10]. Most cases occur unilaterally and in either childhood or early adulthood, and there is an underlying congenital arteriovenous fistula. In the cases reported by Kim et al. [11] and Goldblum et al. [12], an iatrogenic arteriovenous fistula was described. Thus acroangiodermatitis is a recognized complication of the Klippel-Trenaunay-Weber syndromes (angio-osteohypertrophy syndromes). Bilateral acroangiodermatitis may appear in late adulthood, associated with longstanding venous insufficiency. Patients with post-thrombotic syndrome [12], lower-limb paralysis [4], and Prader-Labhart-Willi syndrome [13] are also reported to develop acroangiodermatitis. This case is unusual in that no underlying malformation or venous stasis could be demonstrated, hence our designation of it as spontaneous. The history of chilblains is interesting and raises the possibility of an abnormal vasculature. To the best of our knowledge, there is no association with anticonvulsant or captopril therapy.

In addition to a clinical and pathologic resemblance to Kaposi sarcoma, acroangiodermatitis may clinically resemble other lesions, such as pigmented purpura, vasculitis, and lichen planus, which often require biopsy to distinguish.

The similarity of Kaposi sarcoma histologically lies in the tumorous proliferation of small vessels with admixed lymphocytes and hemosiderin-laden macrophages. However, these vessels are not usually the slitlike vessels of typical Kaposi sarcoma; they have a rounded lumen with prominent endothelial nuclei more reminiscent of epithelioid or lymphangiomatoid variants of Kaposi sarcoma [14]. Histologically, the differential diagnosis also includes vascular forms of fibrous histiocytoma, other hemangiomata, and lymphangioendothelioma. The absence of CD34 positivity in the more peripheral cells may also help to differentiate these lesions [12].

Treatment should include topical hydrocortisone and compression bandaging. Surgical intervention involves attention to the underlying vascular insufficiency. The initial diagnosis and treatment of venous flare in this case was the most appropriate, and re-emphasizes the importance of a detailed history and close clinicopathologic correlation. Increased awareness of this lesion may preclude inappropriate therapy in future cases.

Acknowledgments: The authors wish to sincerely thank Dr David Weedon, Brisbane, Australia, for his help in establishing the diagnosis, and Dr Ghazala Kafeel, Brunei, for her critical review of our paper.

References

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