Acroangiodermatitis of Mali (Pseudo-Kaposi Sarcoma) Associated with Chronic Venous Insufficiency and Obesity: A Case Report

Introduction. Acroangiodermatitis, also known as psesudo-Kapsoi sarcoma, is a rare and benign angioproliferative malady. It presents clinically as purple-colored plaques, patches, or nodules resembling Kaposi sarcoma and is often related to chronic venous insufficiency, arteriovenous malformation of the legs, paralyzed limbs, and residual limbs. Obesity is a risk factor for venous insufficiency and could be related to acroangiodermatitis. Case Report. The authors report a case of acroangiodermatitis of the bilateral legs that was misdiagnosed for 3 years. The diagnosis was confirmed using histopathological and immunohistochemical examinations such as hematoxylin and eosin staining and CD34 immunostaining. Multiple modalities were utilized to treat the wounds, including serial debridement, compression dressings, and silver antimicrobial dressings.  Conclusions. It should be stressed that chronic, nonhealing wounds should be biopsied to obtain a proper diagnosis.

Acroangiodermatitis is an uncommon and rarely recognized angiodysplasia of cutaneous blood vessels that is often associated with venous insufficiency or vascular malformation. Clinically, it can be seen in patients with chronic renal failure on hemodialysis, hepatitis C, paralyzed limbs, and residual limbs.¹ These lesions are very painful and itchy, can bleed easily, and are prone to ulcerate. There are variants of acroangiodermatitis, including Stewart-Bluefarb syndrome, Mali type, and gravity purpura (dermite ocre of Favre), and it also can occur after placement of arteriovenous (AV) shunts for hemodialysis patients.^1,2 

Stewart-Bluefarb syndrome is a congenital AV malformation of the lower legs with multiple AV shunts. It occurs early in life unilaterally on the lower extremities.^3,4 Acroangiodermatitis of Mali type is an exaggerated stasis dermatitis seen in older patients with chronic venous insufficiency.¹ Gravity purpura can occur during a first pregnancy, appearing on both lower extremities with varicose veins.^1,2 Due to histopathological and clinical similarities between acroangiodermatitis and other cutaneous vascular pathologies, the diagnosis of this disease is rarely recognized and often misdiagnosed. Therefore, it is important to differentiate between this benign condition and other malignancies, such as Kaposi sarcoma.⁵

The authors report a case of acroangiodermatitis of Mali type of the bilateral legs that was misdiagnosed for 3 years.

Clinical presentation
A 62-year-old Middle Eastern woman, wheelchair bound with multiple comorbidities (diabetes mellitus, morbid obesity and hypothyroidism), presented with a chief compliant of painful bilateral lower extremity ulcers of 3 years’ duration. The ulcers were located on the lower third of the legs between the malleolus and lower calf (gaiter area). The patient previously was treated by several physicians for 3 years with local wound care, topical antibiotics, and hydrocortisone. However, her leg ulcers failed to improve and became necrotic. Due to the recalcitrant nature and exacerbation of her condition, the patient sought a second opinion in hopes of saving her limbs.

On initial presentation to a podiatric physician at the Dr. Edrees Medical Center (Jeddah, Saudi Arabia), thorough evaluation and examination were performed. Her medical history included no history of trauma; no fever or signs and symptoms of systemic infection were identified. 

Physical examination
Full-thickness ulcers with irregular borders were identified on the distal legs,  encompassing the entire circumference of the bilateral legs. Ulcer length on the left leg was 15 cm with depths ranging from partial thickness to 1 cm; on the right leg, ulcer length was 20 cm with a depth ranging from partial thickness to 1.5 cm. Bilaterally, the wound base was mixed, consisting of pale granulation and fibrotic tissues. Chronic venous insufficiency changes (hemosiderin hyperpigmentation, stasis dermatitis, atrophy blanches, nonpitting edema, and lipodermatosclerosis) were noted (Figure 1). Pedal pulses were palpable on both lower extremities.

Diagnosis
A thorough diagnostic examination, including radiographs, venous Doppler of the lower extremities, aerobic and anaerobic wound culture, and biopsy, was performed. Blood testing included complete blood count, basic metabolic panel, erythrocyte sedimentation rate, and C-reactive protein level.

All radiographic and laboratory investigations were overall unremarkable with minimally elevated inflammatory markers. Venous Doppler study confirmed venous insufficiency. Hematoxylin and eosin (H&E) staining revealed proliferation of small dilated vessels in a thickened edematous papillary and upper reticular dermis. These vessels were thick-walled and lined with plump endothelial cells. In addition, H&E noted perivascular fibroblastic proliferation with variable fibroplasia, extravasated hemosiderin, and red blood cells (Figure 2). CD34 immunostaining showed positive staining of endothelial cells of hyperplastic vessels only with the complete absence of perivascular CD34 expression (Figure 3). Based on the history, examination, and histopathological findings, a final diagnosis of pseudo-Kaposi sarcoma (acroangiodermatitis) was concluded.

Treatment
Initial treatment commenced with serial bedside debridement, oral antibiotic (amoxicillin/clavulanic acid 1 g every 12 hours) based on wound culture and sensitivity, compression dressings, leg elevation, and local wound care for about 2 months. Wound size reduced about 50% and achieved 100% granulation tissue by the end of the second month. Then, the patient started to miss her appointments and her wound deteriorated and became infected. Five months after initial presentation, she was admitted to the hospital for surgical debridement and intravenous piperacillin/tazobactam 4.5 g every 8 hours. After 3 weeks, she was discharged from the hospital and was switched to oral ciprofloxacin 500 mg every 12 hours for 14 days combined with compression therapy, leg elevation, local wound care, and application of topical corticosteroid. The wound status was dramatically improved but never closed completely due to noncompliance, morbid obesity, and lack of physical activity.  

Acroangiodermatitis of Mali was first described by Mali in 1965.³ It can simulate other clinical conditions such as Kaposi sarcoma, pigmented purpura, lichen planus, vasculitis, stasis dermatitis, hemangioma, or lymphangioma.⁵

Histopathological characteristics of acroangiodermatitis reveal proliferation of endothelial cells, neovascularization (often in a lobular pattern), and increased pericytes in the dermis. Disease characteristics can resemble Kaposi sarcoma, including hemosiderin pigment deposition, red blood cell extravasation, dermal fibrosis, and infiltration of lymphocytes, histocytes, and plasma cells. Other similarities can be seen, such as a jagged outline of thin-walled vessels with other normal venules and oval endothelial cells in the dermis.^5,6

Immunohistochemical staining with CD34 helps to differentiate between acroangiodermatitis and Kaposi sarcoma. CD34 staining is seen only on endothelial cells in acroangiodermatitis, whereas in Kaposi sarcoma, it presents on endothelial cells as well as perivascular spindle cells.^5,7 A patient can remain undiagnosed for several years due to acroangiodermatitis’s similarity with other pathologies and the rarity of the condition.²

In this reported case, the patient was misdiagnosed for at least 3 years prior despite evaluation by numerous physicians. Due to the rarity of this condition, the first histopathological biopsy was reported as acute and chronic nonspecific inflammation with granular proliferation. This delay in diagnosis led to inadequate treatment and subsequent complications. In this case, the patient presented with venous ulcers that approached complete healing several times with compression, microbial control, and proper wound care. However, during the final stage of epithelialization, the skin developed multiple purple-colored plaques and patches on both lower extremities, which most likely represent acroangiodermatitis of Mali type.⁸ Histopathological and immunohistochemical examination of the second biopsy at a different laboratory confirmed the final diagnosis.

The present patient is morbidly obese and wheelchair bound. Studies^8,9 have indicated a significant correlation between obesity and the severity of venous insufficiency. Therefore, it can be postulated that there could be an indirect relationship between acroangiodermatitis of Mali and obesity due to the exacerbation of venous insufficiency.

Treatment of acroangiodermatitis has not been widely discussed in the literature. However, the mainstay of treatment is correction of the underlying vascular pathology. For example, cases of AV malformation can be treated surgically. In chronic venous insufficiency, compression therapy and leg elevation are the main therapies to relieve symptoms.¹⁰ Other medical modalities have been reported in the literature with good outcomes, such as oral erythromycin 500 mg 4 times daily or dapsone 50 mg twice daily for 3 months in combination with compression therapy.^5,7 Topical corticosteroids with frequent local wound care also were reported.⁵ In the present case, a combination of different modalities was utilized, including topical corticosteroids, surgical debridement, local wound care, antibiotics, compression therapy, and leg elevation, which achieved favorable results (Figure 4). The patient was consistently encouraged to elevate her legs, try to walk, and increase physical activities. Although ulcers of both legs have achieved more than 90% epithelialization at times, the legs have never healed completely and always sloughed off with violaceous patches.

Acroangiodermatitis of Mali is a chronic and benign condition that requires frequent follow-up and continuous compression therapy. In this report, the importance of histopathological and immunohistochemical tests was elaborated and emphasized. The authors conclude that total healing of these lesions is not possible unless there is a reversal of the underlying causes. Patient physical limitations were an obstacle to in the way of this reversal and complete healing and contributed to the chronicity of her condition. 

Authors: Hani M. Badahdah, MD, DPM, MS, CWSP, AACFAS, DABMSP¹; Khalid M. Edrees, DPM, ABPOPPM, FAPWCA, ACFAOM, DABMSP¹; Lujain Alnasr, MD¹; and Enaam Junainah, MD, JBOP²

Affiliations: ¹Dr. Edrees Specialized Medical Center, Jeddah, Saudi Arabia; and ²College of Medicine, Taif University, Taif, Saudi Arabia

Correspondence: Hani Badahdah, MD, DPM, MS, CWSP, AACFAS, DABMSP, Dr. Edrees Specialized Medical Center, 3803 Sari St, Alkhaldiah dist, Jeddah, Saudi Arabia, 23423-9070; BadahdahDPM@gmail.com

Disclosure: The authors disclose no financial or other conflicts of interest.