Pseudo-Kaposi's sarcoma of the hand associated with an acquired, iatrogenic arteriovenous fistula

Pseudo-Kaposi's sarcoma has been associated, in most cases, with an underlying congenital arteriovenous (AV) fistula. A patient with chronic renal failure and an acquired, iatrogenic AV fistula in his left wrist for hemodialysis developed pseudo-Kaposi's sarcoma on his left hand three years after placement of the AV fistula. Histologic findings included a proliferation of superficial dermal vessels and fibroblasts, extravasated red blood cells, and occasional fibrin thrombi in vessels. To our knowledge, this is the third case of an association between pseudo-Kaposi's sarcoma and an acquired, iatrogenic AV fistula, and the first to involve the hand. Pseudo-Kaposi's sarcoma occurs in association with underlying congenital and acquired AV fistulas.     

Arteriovenous malformation simulating Kaposi's sarcoma (pseudo-Kaposi's sarcoma)

A patient whose condition was initially misdiagnosed as Kaposi's sarcoma involving the left foot received radiation therapy for that disorder. Subsequent examination yielded a correct diagnosis of arteriovenous (AV) malformation. Such cases of AV malformations with skin changes resembling Kaposi's sarcoma have been called pseudo-Kaposi's sarcoma. The clinicopathologic distinctions between Kaposi's sarcoma and pseudo-Kaposi's lesions are discussed. All patients suspected of having Kaposi's sarcoma, especially those younger than 30 years of age, should have careful evaluation for an unsuspected AV malformation.     

Acroangiodermatitis Mali resulting from arteriovenous malformation: report of a case of Stewart-Bluefarb syndrome.

We describe the rare Stewart-Bluefarb syndrome in a 15-year-old boy. This syndrome presents as a congenital arteriovenous malformation of the lower leg with multiple arteriovenous shunts accompanied by the benign acroangiodermatitis of Mali (pseudo-Kaposi's sarcoma). The clinical features of this disorder and the treatment options are reviewed.     

Kaposiforme, HHV-8-negative Akroangiodermatitis bei chronisch-venöser Insuffizienz

A 76-year-old female patient developed severe manifestations of a kaposi-like acroangiodermatitis (so-called Mali's disease) due to chronic venous insufficiency of the lower extremities. The patient presented with large areas of confluent, violaceous or brown-black papules on both lower legs. Histologically, proliferation of thick-walled capillaries was seen in the upper dermis consisting of fully differentiated endothelial cells, as shown by immunohistochemistry. In contrast to true Kaposi's sarcoma, human-herpes-virus-8 DNA could not be detected by polymerase-chain-reaction in this condition. We review the diagnostic criteria used to distinguish between acroangiodermatitis, also called pseudo-Kaposi's sarcoma, and the true Kaposi's sarcoma.     

Stasis dermatitis of the hand associated with an iatrogenic arteriovenous fistula

Stasis dermatitis is a cutaneous finding associated with chronic venous failure resulting in venous stasis. Arteriovenous fistula in the hand may cause a chronic venous stasis. We report a case of stasis dermatitis of the hand associated with an iatrogenic arteriovenous fistula. Stasis dermatitis should be considered as a potential complication of iatrogenic arteriovenous fistula.     

Pseudo-Kaposi's Sarcoma Associated with Acquired Arteriovenous Fistula

Pseudo-Kaposi's sarcoma or acroangiodermatitis usually has underlying conditions of increased venous pressure or circulatory abnormality. We experienced two cases of pseudo-Kaposi's sarcoma in patients with acquired iatrogenic arteriovenous fistula from hemodialysis on their forearms. The patients complained of painful swollen crusted vesicles and purple or erythematous patches or plaques on their hands and fingers. Histologic findings included features of pseudo-Kaposi's sarcoma such as proliferations of small vascular spaces with narrow vascular channels lined by spindle cells, extravasated erythrocytes, and hemosiderin deposits. Percutaneous arteriography performed in Case one excluded the possible coexistence of arteriovenous malformation. In both cases, venous pressure and skin surface temperature were increased around the lesions; these may have played important roles in the development of the lesions. Both cases improved after oral erythromycin treatment, which seemed to be safe and effective for pseudo-Kaposi's sarcoma.     

Pseudo-Kaposi's sarcoma secondary to superficial arteriovenous malformation: Stewart-Bluefarb syndrome

BACKGROUND: Pseudo-Kaposi's sarcoma or Stewart-Bluefarb subtype acroangiodermatitis is uncommon and is caused by arteriovenous fistula and malformation. We report a new case. CASE REPORT: A 33-year-old man presented with painful red-violet plaque on the dorsum of the toes with angiomatous nodules on the sole. Histological and immunohistochemical studies for CD34 were consistent with Kaposi's sarcoma. Doppler ultrasonography and femoral angiography showed multiple distal arteriovenous shunts. Free-flow embolisation with fragments of Ethibloc gelatin sponge was performed and arteriography, performed immediately afterwards, showed delayed venous drainage. The outcome was good with complete drainage of the angiomatous lesions. DISCUSSION: Pseudo-Kaposi's sarcoma Stewart-Bluefarb subtype begins early in life in male subjects, with unilateral skin lesions. It bears clinical and histological resemblance to Kaposi's sarcoma. Doppler ultrasonography and angiography show arteriovenous fistulas that classically develop at shunts, explaining the role of traumatism and high vascular pressure in the genesis of this disease.     

Bilateral pseudo-Kaposi sarcoma in upper limbs

Acroangiodermatitis or pseudo-Kaposi sarcoma is an angioproliferative, self-limited entity that includes a group of diseases, congenital or acquired, with cutaneous lesions similar to Kaposi sarcoma (KS). This term can lead to confusion because it comprises several entities that are completely different, nonetheless, it has an important clinical value as it guides the diagnosis and management of these patients. We report the case of a 67-year-old patient with lesions of acroangiodermatitis in both forearms secondary to arteriovenous shunts from hemodialysis. Doppler ultrasound showed a former arteriovenous fistula in addition to the one already known. Immunohistochemical study showed CD34+ staining in endotelial cells and absence of HHV-8 expression.     

Unilateral limited scleroderma-like changes following formation of an arteriovenous fistula

A 55-year-old man presented with a 1-year history of progressive skin thickening involving the right hand associated with Raynaud's phenomenon, sclerodactyly and painful cutaneous ulcers. An arteriovenous fistula for haemodialysis had been formed on the same arm 2 years previously. There were no symptoms or signs of systemic sclerosis or involvement of the contralateral arm. The limb was clinically ischaemic, attributed to a vascular steal phenomenon from the arteriovenous fistula, superimposed on occlusive arterial disease. A revascularization procedure was performed, which resulted in substantial improvement in the sclerodactyly, Raynaud's phenomenon and hand function. Tissue hypoxia is believed to be a contributing factor in the pathogenesis of scleroderma, and this case demonstrates scleroderma-like changes in the setting of limb ischaemia.     

Expression of the CD34 antigen distinguishes Kaposi's sarcoma from pseudo-Kaposi's sarcoma (acroangiodermatitis)

Summary The differential diagnosis between Kaposi's sarcoma and the so-called 'pseudo-Kaposi's sarcoma' or acroangiodermatitis of the feet is often fraught with difficulty, not only on clinical but also on histological grounds. The aim of this study was to assess whether immunolabelling for the CD34 antigen, a marker of Kaposi's sarcoma cells, could be of value in the distinction between these two angioproliferative disorders. We comparatively examined 16 biopsy specimens from cases of Kaposi's sarcoma and seven biopsies from patients with pseudo-Kaposi's sarcoma, by a streptavidin-biotinperoxidase method, using a monoclonal antibody to the CD34 antigen. All cases of Kaposi's sarcoma showed CD34 labelling both on endothelial cells and on the characteristic spindle-shaped, perivascular cells. Biopsies of pseudo-Kaposi's sarcoma showed a strong labelling of endothelial cells of hyperplastic vessels. However, in sharp contrast with Kaposi's sarcoma, a complete absence of perivascular CD34 expression was noted. It seems therefore that immunolabelling for the CD34 antigen appears to be a valuable tool in the differential diagnosis between Kaposi's sarcoma and pseudo-Kaposi's sarcoma.     

Nail changes in chronic renal failure patients under haemodialysis

Background Chronic renal failure is known to cause various nail pathologies. They may be directly related to the renal condition itself or its complications or to the therapy. Objective To compare nail changes in end‐stage renal failure patients under haemodialysis with healthy persons and to study the potential relationship with various parameters in the patients. Patients and Methods The study comprised 100 patients with chronic renal failure under regular haemodialysis as well as 100 healthy control subjects of matched age and sex. Both groups were subjected to full history taking and thorough general and nail examination. Complete blood picture, liver and kidney function tests and fasting blood glucose level were investigated. Results Nail disorders were more prevalent in patients (76%) than in control group (30%). The half and half nail was the most common finding (20%) followed by – in descending manner – absent lunula, onycholysis, brittle nail, Beau's lines, clubbing, longitudinal ridging, onychomycosis, subungual hyperkeratosis, koilonychias, total leukonychia, splinter hemorrhage, pitting and pincer nail deformity. There was non‐significant correlation between nail changes and age of the patients or duration of haemodialysis. In addition, no evidence of significant relation was found between nail changes and both haemoglobin and albumin levels. Conclusion Frequent nail changes are observed on systematic nail examination of uraemic patients undergoing haemodialysis; however, the cause of them remains obscure and could not be traced to a particular abnormality in the renal condition, medication or the procedure itself and it needs further investigations.     

Hand venous hypertension complicating arterio-venous fistula construction for haemodialysis

Vascular complications of arterio-venous fistula construction for haemodialysis are rare. We report a patient who developed the hand venous hypertension syndrome with ulceration, 2 years after successful construction of a side-to-side radio-cephalic fistula at the wrist. The surgical treatment was ligation of the distended vein immediately distal to the fistula in the hand, and fistula function was preserved. The ulceration healed within 2 weeks of surgery. This complication had not been seen previously in the Dialysis Unit at Cardiff Royal Infirmary where, over the past 20 years, 800 fistulae have been constructed.     

Ultrastructural study of pseudo-Kaposi's sarcoma (Bluefarb-Stewart type)

Summary An ultrastructural study of the skin lesion of a young patient affected by pseudo-Kaposi's sarcoma of the Bluefarb-Stewart type (BSS) is reported. The neoplasm consisted of a proliferation of vascular structures mostly consisting of a solid bud of endothelial cells surrounded by a thinned and polystratified basement membrane and several pericytes. Both endothelial cells and pericytes were of normal ultrastructural appearance. Intervascular stromal cells were few and morphologically identified as macrophages and/or phagocytic fibroblasts. Masses of hemosiderin were detected outside the cells and in the macrophages, endothelial cells, and pericytes. Intracytoplasmatic crystalloid inclusions similar to those found in fetal endothelium and hemangiomas were observed in a few endothelial cells. These findings are different from those of previously reported cases of pseudo-Kaposi's sarcoma and may be helpful in distinguishing Kaposi's sarcoma from BSS. The role of immunodeficiency in the onset of BSS is discussed.     

Stewart-Bluefarb syndrome

Stewart-Bluefarb syndrome is a rare condition involving skin lesions that share clinical features with Kaposi sarcoma and that are secondary to an underlying arteriovenous fistula. We report the case of a 24-year-old man with progressive growth of skin lesions on the lower third of his left leg. Diagnosis of Stewart-Bluefarb syndrome was confirmed histologically and with detection of an arteriovenous fistula.     

Acroangiodermatitis

Kaposi-like acroangiodermatitis or pseudo-Kaposi's sarcoma is a reactive angiodysplasia of the cutaneous blood vessels that resembles true Kaposi's sarcoma, both clinically and histologically. We report a 17-year-old girl with congenital spina bifida and myelomeningocele and cutaneous lesions localized on the lower limbs. Clinical, functional and histological investigation led to a diagnosis of Kaposi-like acroangiodermatitis.     

Innocuous-looking skin scab over an arteriovenous fistula: Case report and literature review

Little is written on the management of an innocuous-looking skin scab over an autogenous arteriovenous fistula (AVF) used for haemodialysis. The seriousness of the underlying pathology can be under-estimated, and this may lead to early loss of the AVF, and major-life-threatening haemorrhage. We describe the management of a 78-year-old patient presenting with an innocuous-looking scab over an AVF and review the pertinent literature on this subject.     

Radionuclide scanning in pseudo-Kaposi's sarcoma.

Sodium pertechnetate Tc 99m radionuclide scanning is considered to be a sensitive indicator in detecting lesions of Kaposi's sarcoma, including occult infiltrations. This report describes a "false-positive" scan in a patient with acroanglodermatitis (pseudo-Kaposi's sarcoma); therefore, this technique may not be useful in the differential diagnosis of these two clinically and histologically similar diseases. We suggest, instead that the technetium scan may be helpful in deciding on the course of therapy in acroangiodermatitis by demonstrating the nature of the underlying vascular anomalies.     

Melanonychia striata: clarifying behind the Black Curtain. A review on clinical evaluation and management of the 21st century

Melanonychia striata is characterized by a tan, brown, or black longitudinal streak within the nail plate that runs from the proximal nail fold to the distal part of the nail plate. Melanonychia striata is due to increased activity of melanocytes or melanocytic hyperplasia in the nail matrix with subsequently increased melanin deposition in the nail plate. The most common cause of melanonychia striata associated with melanocytic activation is ethnic melanonychia which occurs in dark-skinned individuals. Other causes of melanonychia striata related to melanocytic activation include pregnancy, chronic local trauma, infections, medications, dermatological disorders, endocrine disorders, alkaptonuria, hemochromatosis, porphyria, graft-vs-host disease, Peutz-Jeghers syndrome, and Laugier-Hunziker syndrome. Causes of melanonychia striata associated with melanocytic hyperplasia include nail matrix melanocytic nevus, nail lentigo, and nail apparatus/subungual in situ and invasive melanoma. In most cases, melanonychia striata is a benign condition, especially in children. Consequently, most investigators advocate a wait-and-see approach. Nail apparatus/subungual melanoma should be suspected if there is an abrupt onset after middle age, personal or family history of melanoma, rapid growth, darkening of a melanonychia band, pigment variegation, blurry lateral borders, irregular elevation of the surface, a bandwidth >3 mm, proximal widening, associated nail plate dystrophy, single rather than multiple digit involvement, and periungual spread of pigmentation onto the adjacent cuticle and/or proximal and/or lateral nail folds (Hutchinson sign). Prolonged follow-up is mandatory for early detection of possible malignant changes.     

Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is a dermatological condition characterized by purple-colored nodules, plaques or patches, mostly on the extensor surfaces of lower extremities, usually in patients with chronic venous insufficiency and arteriovenous malformations of the legs, but also in hemodialysis patients with iatrogenic arteriovenous shunts, paralyzed limbs and amputation stumps. Acroangiodermatitis in patients with chronic venous insufficiency manifests usually as bilateral skin lesions located on the dorsa of the feet, halux and second toe, or on the medial aspect of lower legs. Acroangiodermatitis may look like Kaposi sarcoma, but in contrast to Kaposi sarcoma, acroangiodermatitis is not characterized by progression of changes, and there is a lack of spindle cells and silt-like vessels on histopathologic analysis. Three cases of acroangiodermatitis encountered in our clinical practice are described. The patients presented with livid-erythematous patches on lower legs and skin changes connected with chronic venous insufficiency, treated at the Department Phlebology Unit. Results of the histopathologic analysis indicated acroangiodermatitis. Thus, in clinical practice it is important to recognize acroangiodermatitis and to exclude Kaposi sarcoma, as sometimes there is similarity with this entity. Topical therapy with neutral and local corticosteroid preparations is often useful, however, the use of compressive bandages and dermatologic follow up are recommended.