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Wounds 2018 Nov;30(11):E105-E107

College of Medicine, Taif University, Taif, Saudi Arabia.

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. Read More

Open Access Maced J Med Sci 2018 Sep 20;6(9):1688-1693. Epub 2018 Sep 20.

Medical Institute of Ministry of Interior (MVR), Department of Dermatology, Venereology and Dermatologic Surgery, General Skobelev Nr 79, Sofia, Bulgaria.

Background: Kaposi's sarcoma was first described in 1872 by Moritz Kaposi. To date, it is considered a malignant disease is originating from the endothelial cells of the lymphatic vessels believed to be infected with HHV-8. The current classification defines four major epidemiological forms of Kaposi's sarcoma: classical, endemic, AIDS-associated, and iatrogenic. Read More

Biomed J 2018 Jun 11;41(3):209-210. Epub 2018 Jul 11.

Department of Dermatology, Hassan II University Hospital of Fez, Fez, Morocco.

The rainbow pattern is currently a subject of debate, it is considered the specific dermoscopic pattern of Kaposi sarcoma. We present in this research correspondence a review of 700 dermoscopic figures of different biopsy-proven skin diseases in our department of dermatology of the Hospital Hassan II of Fez. All the lesions were localized to the limbs except for one lesion of atrophic scar that was localized on the left shoulder. Read More

J Eur Acad Dermatol Venereol 2018 Nov 28;32(11):e429-e432. Epub 2018 May 28.

Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.

Br J Dermatol 2016 Jun;174(6):e84

Department of Dermatology, University Hospital, Brest, France.

Clin Exp Dermatol 2016 Jul 7;41(5):522-5. Epub 2016 Jun 7.

A1 Sharad Kunj, Thane West, Maharashtra, India.

Stewart-Bluefarb syndrome (SBS) is a subtype of pseudo-Kaposi sarcoma (PKS), which is associated with congenital arteriovenous malformation. It is a rare syndrome, characterized by cutaneous lesions with onset in the second decade of life. We report a case of SBS in a 33-year-old man presenting with a 15-year history of unilateral, reddish-brown, raised lesions on his right lower leg and the dorsum of his foot. Read More

Dermatol Online J 2015 May 18;21(5). Epub 2015 May 18.

Istanbul University.

Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. Read More

Indian J Dermatol 2015 May-Jun;60(3):268-71

Department of Pathology, Ankara Diskapi Yildirim Beyazit Research and Training Hospital, Ankara, Turkey.

Acroangiodermatitis is a rare self-limited angioproliferative lesion which can be associated with congenital vascular malformations or acquired venous insufficiency. Despite of its benign character, differential diagnosis of this lesion is very important because it closely resembles Kaposi sarcoma. Here we present a 26-year-old male patient with unilateral, purplish-red colored papules on his right ankle which diagnosed as acroangiodermatitis and discuss histopathological features, differential diagnosis and treatment of this unusual condition. Read More

Indian Dermatol Online J 2014 Jul;5(3):323-5

Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.

Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Read More

Rev Med Suisse 2014 May;10(430):1081-3

Ann Saudi Med 2014 Mar-Apr;34(2):179-81

Dr. Muzamil Amin Chisti, Department of Dermatology,, King Faisal Specialist Hospital and Research Centre,, Riyadh 11211, Saudi Arabia, T: +966114424608, F: +966114424603,

Acroangiodermatitis (AAD) (synonym, pseudo-Kaposi sarcoma) is a term that encompasses 2 different conditions: (1) AAD of Mali, which refers to skin lesions that mainly develop bilaterally on the lower extremities of patients with chronic venous insufficiency and is an extreme form of stasis dermatitis and (2) Stewart-Bluefarb syndrome, which consists of an arteriovenous malformation that mainly affects the limbs of young patients unilaterally. We present a case of a 68-year-old lady with progressive skin lesions on both lower limbs (right > left) as a result of chronic venous insufficiency that became worse after the leg-vein harvest for coronary artery bypass grafting was taken from the right leg. Up to our knowledge this is the first case of its kind to be reported. Read More

Int Wound J 2015 Apr 5;12(2):169-72. Epub 2013 Apr 5.

Division of Plastic and Reconstructive Surgery, American University of Beirut Medical Center, Beirut, Lebanon.

Stewart-Bluefarb syndrome (SBS), also known as acroangiodermatitis or pseudo-Kaposi, is a condition rarely encountered. It involves skin lesions that are clinically similar to Kaposi sarcoma but are histologically different, and are usually secondary to an underlying arteriovenous fistula. Treatment of this disease usually involves the correction of the underlying vascular abnormality, with the mainstay of therapy ranging from compression devices for venous stasis, limited oral medications (dapsone and erythromycin) and local wound care including topical steroids. Read More

An Bras Dermatol 2011 Jul-Aug;86(4 Suppl 1):S13-6

Laboratório de Vigilância em Leishmanioses, Instituto de Pesquisa Clínica Evandro Chagas, Fundação Oswaldo Cruz, Rio de Janeiro, RJ, Brasil.

Acroangiodermatitis, often known as pseudo-Kaposi sarcoma, is an uncommon angioproliferative entity related to chronic venous insufficiency, arteriovenous fistulae, paralysed limbs, amputation stumps, vascular syndromes and conditions associated with thrombosis. It presents most frequently as purple macules, papules or plaques in the dorsal aspects of the feet, especially the toes, and the malleoli. We report a case of acroangiodermatitis in the plantar aspect of the foot, misdiagnosed for two years, in which haematoxylin-eosin hystopathological stain and immunolabeling with CD34 histochemistry examination were decisive for diagnosis. Read More

Indian J Dermatol Venereol Leprol 2010 Sep-Oct;76(5):553-6

Department of Dermatology, T.N. Medical College and B.Y.L. Nair Ch. Hospital, Mumbai Central, Mumbai, India.

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is an unusual, benign condition which clinically presents as purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. It resembles aggressive conditions like Kaposi's sarcoma and requires histopathological examination for its diagnosis. We report two such cases of acroangiodermatitis. Read More

Dermatol Online J 2010 Jul 15;16(7). Epub 2010 Jul 15.

Department of Dermatology, Venereology & Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Pseudo-Kaposi sarcoma is a disease entity that encompasses acroangiodermatitis as well as Steward-Bluefarb syndrome. It has varied etiologies and clinical presentations. Most important distinction is from Kaposi sarcoma and this is mainly histopathological. Read More

Am J Dermatopathol 2010 Oct;32(7):700-3

Clinica Ponferrada, Spain.

The promontory sign was described 27 years ago and it was claimed to be useful in the diagnosis of early stages of Kaposi sarcoma. However, it is not pathognomonic, because it has also been described in angiosarcoma and in benign vascular tumors. Some authors claim that some reactive vascular lesions known as pseudo-Kaposi do not present the promontory sign. Read More

J Cutan Pathol 2009 Apr;36(4):482-5

Department of Dermatology, Hospital dos Capuchos, Lisbon, Portugal.

Pseudo-Kaposi sarcoma is a benign reactive vascular proliferation mainly involving the lower legs, which can be related to acquired chronic venous insufficiency or congenital arteriovenous malformations. In its most common presentation, acroangiodermatitis is seen in patients with chronic venous insufficiency of the lower limbs as an exaggeration of the stasis dermatitis. However, rare reports of acroangiodermatitis include descriptions in amputees (especially in those with poorly fitting suction-type devices), in patients undergoing hemodialysis (with lesions developing distally to arteriovenous shunts) and in patients with paralyzed legs. Read More

Dermatol Online J 2007 Jul 13;13(3):22. Epub 2007 Jul 13.

Hacettepe University Faculty of Medicine Department of Dermatology, Ankara, Turkey.

Pseudo-Kaposi sarcoma is a rare vascular phenomenon which can be related to congenital vascular malformations or acquired venous insufficiency. The differential diagnosis includes Kaposi sarcoma. In this report, a patient with Pseudo-Kaposi sarcoma Stewart-Bluefarb subtype is presented and the related literature is reviewed. Read More

Acta Dermatovenerol Croat 2007 ;15(3):152-7

University Department of Dermatology and Venereology, Sestre milosrdnice University Hospital, Vinogradska cesta 29, HR-10000 Zagreb, Croatia.

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. Read More

Hautarzt 2008 Mar;59(3):233-6

Abteilung für Dermatologie und Allergologie, Universitätsklinik Ulm, Ulm.

We report on a patient with AIDS stage C3, who received haemodialysis for terminal renal insufficiency and presented with Kaposi sarcoma-like skin lesions on the left hand, distal of his dialysis shunt. Histology, immunohistochemistry and PCR analysis did not support the initially favoured diagnosis of a Kaposi sarcoma, but revealed a pseudo-Kaposi sarcoma related to the Stewart-Bluefarb-syndrome. Read More

Actas Dermosifiliogr 2007 May;98(4):268-70

Servicios de Dermatología, Complejo Hospitalario Universitario Juan Canalejo, La Coruña, España.

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. Read More

Intern Med J 2006 Aug;36(8):535

Department of Dermatology, University School of Medicine, Essen, Germany.

J Cutan Pathol 2005 Jul;32(6):429-32

Department of Clinical Medicine and Immunological Sciences, Section of Dermatology, Universit of Siena, Siena, Italy.

Increasing use of suction-socket lower limb prostheses has been associated with an increased frequency of dermatological manifestations, linked to the fact that the skin of the amputation stump must adapt to an entirely new environment. In particular, verrucous hyperplasia and rare cases of acroangiodermatitis (pseudo-Kaposi sarcoma) have been described. We report a case of amputation stump dermatitis, clinically resembling verrucous hyperplasia, but with predominant histological aspects of acroangiodermatitis in a patient with a suction-socket lower limb prosthesis. Read More

J Am Acad Dermatol 2003 Nov;49(5):887-96

DISEM, University of Genoa.

New and relatively old types of vascular (capillary) proliferations in the skin have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, all of them present with multiple, erythematous-violaceous and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve limbs. Read More

Pediatr Dermatol 2001 Jul-Aug;18(4):325-7

Department of Pediatric Dermatology, Ramos Mejia Hospital, Buenos Aires, Argentina.

Pseudo-Kaposi sarcoma with vascular malformation (Stewart-Bluefarb syndrome) is an uncommon and important entity characterized by congenital arteriovenous malformation and skin lesions that may resemble Kaposi sarcoma. This is usually seen in the lower limb of young people. We report a case of this syndrome in a 17-year-old boy who had skin lesions on the right leg and foot. Read More

Hautarzt 2000 May;51(5):336-9

Hautklinik der Ruprecht-Karls-Universität Heidelberg.

The Stewart-Bluefarb syndrome is defined as an unilateral angiodermatitis due to multiple arterio-venous fistules accompanied by acroangiodermatitis resembling Kaposi sarcoma (pseudo-kaposi sarcoma). The acroangiodermatitis is most common on the lower limb. It leads to ulcerated nodules with a high risk of bleeding and infection, as well as edema, pain and seldom limb hypertrophy. Read More

Hautarzt 1999 Sep;50(9):629-36

Klinik und Poliklinik für Dermatologie, Universitätsklinikum Benjamin Franklin, Freie Universität Berlin.

Acute infection with hepatitis C virus (HCV) is often clinically inapparent, but may affect several organ systems in its chronic course. In dermatology, common diseases such as lichen planus, cryoglobulinemic vasculitis and porphyria cutanea tarda have been described in association with HCV infection. A number of other dermatologic disorders, e. Read More

Dermatology 1999 ;198(4):439-41

Department of Dermatology, CHUV/DHURDV, Lausanne, Switzerland.

Ophthalmology 1999 Jun;106(6):1219-22

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114, USA.

Objective: Capillary hemangioma may appear de novo and involute during the first decade of life, but rarely during pregnancy. This study describes the clinical and histologic findings of an eyelid mixed type cavernous-capillary hemangioma arising in a nevus flammeus and discusses the differential diagnosis of this lesion.

Study Design: Clinicopathologic case report. Read More

Hautarzt 1996 Jul;47(7):493-501

Dermatologische Klinik und Poliklinik, der Universität, Regensburg.

Dermatologic disorders of the stump in patients with a leg amputation may have characteristic pathophysiologic and clinical features. A knowledge of these factors may lead to earlier diagnosis and avoid immobilization. There ist no German language review of this aspect of dermatology. Read More

Clin Exp Dermatol 1993 Mar;18(2):151-3

Department of Dermatology, Hospital Universitario San Carlos, Universidad Complutense, Madrid, Spain.

Arteriovenous malformations (AVM) are vascular anomalies containing a communication between an artery and a vein without an intervening capillary bed. In 1990, Klippel and Trenaunay reported a patient with limb overgrowth, cutaneous angiomata and varicose veins. In 1918, Parkes Weber mentioned the additional feature of AVM terming the condition 'hemangiectatic hypertrophy'. Read More

Hautarzt 1992 Jan;43(1):40-2

Dermatologische Klinik und Poliklinik, Technischen Universität München.

We describe a 17-year-old Turkish girl with painful ulcerative pseudo-Kaposi lesions on the left foot and partial giant growth of the same leg. Stewart-Bluefarb syndrome is discussed with reference to this case. Read More

J Dermatol 1991 Nov;18(11):661-6

Department of Dermatology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.

A 39-year-old man had pseudo-Kaposi sarcoma on his left foot. A biopsy specimen obtained from a cutaneous lesion showed increased numbers of vascular spaces, proliferation of fibroblast-like spindle cells, and deposition of hemosiderin in the dermis. Immunohistochemically, proliferative fibroblast-like spindle cells around the vessels were positive for anti-factor XIIIa antibody. Read More

Hautarzt 1991 Jul;42(7):467-70

Dermatologische Klinik und Poliklinik, Ludwig-Maximilians-Universität München.

A 25-year-old male patient presenting with Prader-Labhart-Willi syndrome is reported. He had sought medical help because of lichenoid skin eruptions on his lower legs. Histologically the lesions turned out to be pseudo-Kaposi's sarcoma. Read More

J Mal Vasc 1991 ;16(2):153-7

Clinique des Maladies Cutanées, Hôpital Saint-Louis, Paris.

Pseudo-Kaposi's Sarcoma with vascular disease concern mainly acroangiodermatitis described by Mali--(with chronic venous insufficiency)--, arteriovenous malformations with angiodermatitis described by Stewart and Bluefarb, and pseudo-Kaposi's Sarcoma occurring after placement of arteriovenous shunt for hemodialysis. Search for relation ships between classical Kaposi's Sarcoma and new AIDS Kaposi's Sarcoma explains new interest devoted to Pseudo-Kaposi's Sarcoma. Read More

G Ital Dermatol Venereol 1990 Jun;125(6):251-4

Divisione Dermatologica, Spedali Civili, Brescia.

The authors described a 39 year old woman affected by epidermal nevus syndrome, with cutaneous (verrucous epidermal nevus), skeletal (thoracolumbar levoscoliosis and frontal bossing) and ocular (papillar coloboma and coroideal nevus) defects. Moreover the patient presented vascular malformations and hamartomas: lymphangioma circumscriptum of the mammary area, left peroneal Gorham's disease, artero-venous acral tumour of the left foot and multiple artero-venous shunts of the lower limbs. Since puberty, hemodynamic modifications have caused pseudo-Kaposi of Bluefarb-Stewart of legs and feet and malleolar painful ulcers. Read More

Hautarzt 1988 May;39(5):304-7

Dermatologische Klinik und Poliklinik, Ludwig-Maximilians-Universität München.

The cases are reported of two patients afflicted with acroangiodermatitis (pseudo-kaposi) in paralysed legs. From the pathogenetic point of view, the lack of a muscle pump and, possibly, disturbed innervation of vessels are of importance. Read More

Ann Dermatol Venereol 1988 ;115(10):1035-9

Clinique Dermatolgique, Hôpital Saint-Louis, Paris.

We report a case with clinical symptoms of a Stewart-Treves syndrome 2 years after homolateral mastectomy for carcinoma. The mammary origin of the syndrome was found at histology and confirmed by assays of hormone receptors in skin biopsies before and during anti-hormone therapy. This treatment rapidly attenuated the clinical signs of both the lymphedema and the pseudo-Kaposi cutaneous lesions. Read More

Z Hautkr 1987 Sep;62(17):1272-9

Universitätsklinik für Dermatologie und Venerologie in Graz.

We present clinical, angiological, and histopathological findings concerning different kinds of so-called "kaposiform" acroangiodermatitis. In particular, we refer to the etiopathogenesis, prognosis, differential diagnosis, and therapy of each entity. The similarities between the so-called "kaposiform" acroangiodermatitis and Kaposi's sarcoma are primarily derived from clinical criteria. Read More

J Dermatol 1986 Dec;13(6):477-9

Lancet 1986 Oct;2(8512):921

Dermatol Monatsschr 1986 ;172(7):415-21

Acta Derm Venereol 1985 ;65(1):75-7

A patient with the Klippel-Trenaunay syndrome and pseudo-Kaposi elements is reported. This combination of symptoms has only been published very occasionally. Selective quantitative measurements of the peripheral tissue blood flow revealed a significantly increased cutaneous blood flow in the hypertrophied leg. Read More

Acta Derm Venereol 1984 ;64(3):246-9

A case of pseudo-Kaposi sarcoma of the feet (acroangiodermatitis) is reported. The patient showed clinical and light-microscopic signs closely resembling Kaposi's sarcoma, but by electron microscopy of skin biopsies definite signs of degeneration of vascular walls and infiltrating cells were demonstrated. Electron microscopy should be applied when conventional histology is inconclusive or fits poorly to the anamnestic information and/or clinical appearance of the lesions. Read More

Z Hautkr 1983 Jun;58(11):834-42

Case report about a 45 year old man with hyperkeratotic papules and plaques on both lower legs now persisting for more than 20 years. After detection of arterio-venous shunts the initial clinical and histological diagnosis of Kaposi-sarcoma had to be replaced by the diagnosis of pseudo-Kaposi's sarcoma, a benign reactive process. In the pathogenesis of this disease arteriovenous shunts are of great importance. Read More

Cutis 1982 Aug;30(2):240-2, 247, 255-6

Phlebologie 1978 Jul-Sep;31(3):249-56

In 1965 Mali and Kuiper described acro-angiodermatitis or pseudo Kaposi. From clinical and histological viewpoints pseudo Kaposi looks exactly like Kaposi angiosarcoma. From a pathogenic viewpoint the authors describe pseudo Kaposi as a long term complication of chronic venous insufficiency. Read More

Schweiz Rundsch Med Prax 1977 Jan;66(1):21-5

Dermatologica 1975 ;151(2):65-79

Acroangiodermatitis is a special form of the 'angiodermite' of Favre and Chaix in patients presenting with chronic venous insufficiency localized on the dorsal areas of the feet and toes. Different stages including exulceration may occur. 60% of our male and 38% of our female patients with postthrombotic syndrome showed signs of acroangiodermatitis. Read More

Arch Dermatol 1974 Dec;110(6):907-10