Acroangiodermatitis Research Articles

Overlap of histological features of pigmented purpuric dermatosis and kaposi sarcoma – pseudo kaposi sarcoma

Pseudokaposi sarcoma can be congenital or acquired. Due to prolonged venous stasis, leads to peripheral tissue hypoxemia, which results in neovascularisation and fibroblast proliferation. Treatment includes rectification of the underlying pathology such as graded compression stockings or compression pumps for venous stasis and ulcer management with regular wound care. Medical management includes oral antibiotics, along with compression therapy with good results. Our patient presented mild lower limb pitting edema unilaterally with erythematous barely elevated plaque over the anterior aspect of the lower limb.

"Pseudotumors" in Dermatology.

In dermatology, “pseudo” is often used as a prefix for entities resembling another standard condition, either morphologically or histopathologically. Correspondingly, “pseudotumor” is a term encompassing dermatological conditions which are not true proliferations, but either have a clinical resemblance to a known tumor (e.g., Pseudokaposi's sarcoma is actually a non-neoplastic condition) or a histopathological resemblance to one (e.g., pseudo-myogenic hemangioendothelioma named due to a histopathological resemblance between myocytes and tumor cells). Often such a nomenclature can create confusion and unnecessary alarm for both the physicians and the patients. Through this article we attempt to summarise “pseudotumors” in dermatology and classify them into clinical and histopathological “pseudotumors”, so as to produce a ready reckoner for this confusing nomenclature.

Mastocytosis in the skin accompanied by pseudo-Kaposi's sarcoma.

Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of neoplastic mast cells in the skin and/or other extracutaneous tissues. Most patients with skin involvement can be subclassified into one of the three subtypes of cutaneous mastocytosis currently recognized by the World Health Organization (i.e., mastocytoma, maculopapular cutaneous mastocytosis and diffuse cutaneous mastocytosis); however, some patients may occasionally present with atypical skin lesions that cannot be ascribed to any of these disease subtypes. Here, we report three patients diagnosed with mastocytosis and an unusual cutaneous involvement mimicking Kaposi's sarcoma. Skin biopsies showed neoplastic mast cell infiltrates together with features commonly seen in acroangiodermatitis, and immunohistochemistry for human herpesvirus 8 was negative. One patient fulfilled the criteria for aggressive systemic mastocytosis, showed no response to cytoreductive therapy, and died because of disease progression. The remaining two patients had indolent and smoldering systemic mastocytosis, respectively, but they showed several features associated with an unfavorable prognosis such as extensive involvement of the hematopoiesis by the KIT D816V mutation, increased serum β2-microglobulin, and decreased serum lactate dehydrogenase. The presence of pseudo-Kaposi's sarcoma skin lesions is an uncommon finding in mastocytosis which may alert physicians to the possible existence of underlying features indicative of a poor prognosis.

Diffuse dermal angiomatosis of the breast: an emerging entity in the setting of cutaneous reactive angiomatoses

New and emerging types of cutaneous vascular (capillary) proliferations have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, they are characterized by multiple, red violaceous, and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve the extremities. Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically have a pseudoangiosarcomatous pattern, they are reactive in that they originate from the (sub)occlusion of vascular lumina by different localized or systemic disorders. The vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn. Among them, diffuse dermal angiomatosis of the breast is a variant of diffuse dermal angiomatosis involving middle-aged women with macromastia, obesity, smoking, and vasculopathic disorders, considered a distinct disorder in the spectrum of cutaneous reactive angiomatoses. It presents with reticulated erythematous to purple patches with sometimes a tendency to ulcerate and bleeding, appearing on large, pendulous breasts. The pathogenesis is related to tissue hypoxemia resulting from subclinical torsion, compression, and increased venous hydrostatic pressure due to the macromastia, aggravated by the associated ischemic conditions such as hypertension and diabetes. There is no evidence-based therapy, but reduction mammoplasty is a viable treatment option. This should be evaluated in all patients who fail conservative therapy.

Novel Manifestations of Acroangiodermatitis: A Report of Two Cases.

Novel Manifestations of Acroangiodermatitis: A Report of Two Cases.

An Observational Study of Dermatological Manifestations in Patients of Chronic Renal Failure Undergoing Hemodialysis

Background: Chronic renal failure (CRF) presents with an array of cutaneous manifestations. Newer changes are being described since the advent of hemodialysis, which prolongs the life expectancy, giving time for these changes to manifest. Aim: The aim of this study was to evaluate the prevalence of dermatologic problems among patients with chronic renal failure (CRF) undergoing hemodialysis. Methods: ninty three .. patients with CRF on hemodialysis were examined for cutaneous changes. Results: all the patients complained of some skin problem. The most prevalent finding was xerosis (54.8%), followed by pallor (21.5%), pruritus (19.35%) and cutaneous pigmentation (18.3%). Other cutaneous manifestations included Kyrle's disease (5.4%); fungal (5.4%), bacterial (6.5%) and viral (3.2%) infections; purpura (9.7%). The nail changes included half and half nail (21%), koilonychia (39.78%), onychomycosis (1.08%), onychorrhexis (6.45%), splinter hemorrhages (1.08%), and Beau's lines (2%). Hair changes included sparse hair (18.28%), and brittle and lusterless hair (11.83%). Oral changes included macroglossia with teeth markings (23.65%), . Some rare manifestations of CRF like uremic frost, gynecomastia and pseudo-Kaposi's sarcoma were not observed. Conclusions: CRF is associated with a complex array of cutaneous manifestations caused either by the disease or by treatment. The commonest are xerosis and pruritus and the early recognition of cutaneous signs can relieve suffering and decrease morbidity.

English

Acroangiodermatitis (Synonym: pseudo-Kaposi sarcoma) is an unusual, benign, self‑limited angioproliferative lesion. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. This condition presents clinically as purple-colored patches, plaques or nodules on the lower extremities. Despite being benign, this lesion assumes importance because of its close resemblance to the aggressive Kaposi sarcoma and requires histopathological examination for its diagnosis. We describe here a case of a 55-year old female who presented with raised, hyperpigmented lesions over both ankles and histopathological examination from one of the lesions confirmed the diagnosis of acroangiodermatitis.

Beating the Pink

Introduction: Kaposi sarcoma (KS) is an HIV associated skin lesion. Kaposi sarcoma is rare in HIV negative patients and is associated with HHV-8 infection. Clinically it presents as a pink nodular mass, single or multiple which can mimic pyogenic granuloma and bacillary angiomatosis. The etiology associated with HHV-8. Treatment options modalities can vary between from electrocuatery to surgical excision. Case report: A man in his 50s came to the dermatology department with a pink purplish nodular mass on his leg that had developed over two years. He had no subjective complaint. Histopatholgical examination confirmed diagnosis of KS. The patient’s blood screening and investigations were all normal including viral markers. Conclusion: Kaposi sarcoma is an associated skin lesion. It should be distinguished from some vascular tumors such as pyogenic granuloma (PG), pseudo-Kaposi sarcoma, and bacillary angiomatosis (BA).

Clinicopathologic analysis of acroangiodermatitis: 12 case reports

Objective To investigate the clinicopathologic features and differential diagnosis of acroangiodermatitis (AM). Methods Clinical and pathological data on 12 patients with AM were retrospectively reviewed. Results Clinical manifestations of AM consisted of circumscribed brown to violaceous macules, plaques, nodules and ulceration. Lesions were located in bilateral legs in 6 patients, and in unilateral legs in the other 6 patients. Histopathological examination revealed an increased number of lobular or clump - shaped capillaries and small veins whose lumens were round and regular, swelling of vascular endothelial cells, and different degrees of erythrocyte extravasation, hemosiderin deposition, dermal fibrosis and sparse infiltrates of inflammatory cells. The lesions were histologically located in the superficial dermis in 3 cases, in the upper and middle dermis in 8 cases, and in the entire dermis in 1 case. Immunohistochemical studies showed that vascular endothelial cells stained positive for CD31 and CD34, while perivascular cells stained negative for CD34. Conclusions AM has specific clinical and pathological manifestations, and pathological examination is essential for the diagnosis of AM. Key words: Sarcoma, Kaposi; Disease attributes; Pathology; Diagnosis; Acroangiodermatitis

Pseudo-kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas

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. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

A case of Parkes-Weber syndrome accompanied by pseudo-Kaposi's sarcoma

A girl who aged eight years and seven months presented with prunosus patches on the right buttock for 8 years,gradual unilateral enlargement of the right lower limb for more than 7 years,and multiple vegetations for 1 year.Dermatological examination showed nevus flammeus and multiple malodorous vegetations over the right lower limb with high skin temperature.The right lower limb was thicker and longer than the left lower limb.X-ray examination,magnetic resonance imaging and Doppler ultrasound examination revealed high-flow vascular malformations.Pathological examination of the vegetations showed vascular proliferation,fibroblast proliferation and erythrocyte extravasation.She was diagnosed as Parkes-Weber syndrome accompanied by pseudo-Kaposi's sarcoma. Key words: Parkes-Weber syndrome; Pseudo-Kaposi's sarcoma; Arteriovenous fistula

Case report Acroangiodermatitis (Pseudo-Kaposi's sarcoma); A challenging vascular condition

Acroangiodermatitis (synonym pseudo-Kaposi's sarcoma) is an unusual, benign condition which clinically presents as erythematous, violaceous, brown, or purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities. Acroangiodermatitis is often associated with different disorders including chronic venous insufficiency, arteriovenous malformations like Klippel-Trenauny syndrome, iatrogenic arteriovenous shunts like fistula in patients under hemolysis (lesions are developing distally to arteriovenous shunts), and in paralysed limbs. It has also been reported above amputation stump of the limbs (especially in those with poorly fitting suction-type devices). It resembles aggressive conditions like Kaposi's sarcoma and requires histopathological examination for its diagnosis. We report a case of a 47-years old man with lesions of acroangiodermatitis in both lower legs and feet, secondary to chronic venous insufficiency. Histopathology showed dilated capillaries in the dermis with extravasated red blood cells, hemosiderin deposits and granulation tissue formation. In conclusion we would like to emphasize the importance of histopathology and immunohistochemistry in distinguishing this benign condition from similar looking malignant conditions especially Kaposi’s sarcoma.

Acroangiodermatite (pseudossarcoma de Kaposi): uma condição raramente reconhecida. Um caso na planta do pé associado a insuficiência venosa crônica

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. Patient had chronic venous insufficiency. The lesion responded well to the treatment with a combination of leg elevation and compression.

Recurrent Foot Ulcers in Young Woman: Stewart-Bluefarb Syndrome: Report of One Case and Literature Reviews

Stewart-Bluefarb syndrome (SBS) is one kind of the acroangiodermatitis, which is associated with an acquired or congenital arteriovenous malformation. This is a rare syndrome characterized by cutaneous lesions that usually onset at the second decade. We report the Stewart-Bluefarb syndrome in a 20-year-old woman presenting as recurrent foot ulcers of the right foot with multiple arteriovenous malformations. Diagnosis of SBS was confirmed histologically and radiologically. The clinical features of this disorder and the treatment options are reviewed.

Acroangiodermatitis of Mali in a patient with congenital myopathy

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. Here we report a case of acroangiodermatitis in a patient with congenital myopathy and have also discussed its pathogenesis.

Acroangiodermatitis of mali: A rare vascular phenomenon

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. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red blood corpuscles (RBCs), hemosiderin deposits, and hyperplastic granulation tissue. Both were treated with oral antibiotics and topical steroids. The ulcers showed a good response within 2 months of treatment.

Pseudo‐Kaposi’s sarcoma because of suction‐socket lower limb prosthesis

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. We report on a 28 year-old-male who presented pseudo-Kaposi's sarcoma in an amputation stump because of suction-socket lower limb prosthesis.

Pseudomaladie de Kaposi secondaire à une malformation artérioveineuse superficielle : syndrome de Stewart-Bluefarb

Pseudo-Kaposi's sarcoma or Stewart-Bluefarb subtype acroangiodermatitis is uncommon and is caused by arteriovenous fistula and malformation. We report a new case. 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. 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.

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.

Pseudosarcoma de Kaposi bilateral en miembros superiores

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.