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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.2 Rio de Janeiro Mar./Apr. 2004
Acroangiodermatitis (pseudo-Kaposi's sarcoma)*
Rubem David AzulayI; Bernard Kawa KacII; Gerson Cotta-PereiraIII; Ana Flávia Lemos da CunhaIV
Professor at UFRJ and UFF. Titular Professor, Fundacao Tecnico Educacional Souza
Marques and Universidade Gama Filho. Head of the Dermatology Institute, Santa
Casa de Misericordia Hospital, Rio de Janeiro
IICoordinator of the Histopathology Service at the Dermatology Institute, Santa Casa de Misericordia Hospital, Rio de Janeiro
IIIHead of the Immunochemistry and Histochemistry Service, Santa Casa de Misericordia Hospital, Rio de Janeiro
IVPost-Graduate Student, Fundacao Tecnico Educacional Souza Marques
Acroangiodermatitis is a very rare disease. It is characterized by erythematous violaceous cutaneous lesions, localized mainly in the legs and feet, resembling Kaposi's Sarcoma. A case is presented of a female, 57 years old presenting erythematous-violaceous lesions in both legs. She also presented varices, reticularis livedo and venous stasis. The clinical and histopathological lesions resembled Kaposi's Sarcoma. The immunohistochemistry (CD-34) confirmed the diagnosis. This is the first case observed in Brazil.
Keywords: Skin neoplasms.
The profile known today as acroangiodermatitis was first described by Kopf & Gonzale,1 who designated it congenital dysplastic angiopathy.
In 1965, Mali and cols.2 studied 18 cases of angiodermatitis associated with chronic venous insufficiency and preferred the denomination acroangiodermatitis, which is currently the most often used term. In this same line of investigation, Bluefarb & Adams3 described this condition as "arteriovenous malformation with angiodermatitis". In 1974, Earth and cols.,4 studying a case in a 24-year-old youth, noted the similarity, as much from the clinical as from the histopathological point of view; between this syndrome and Kaposi's sarcoma. Thus, they proposed the designation of pseudo-Kaposi's sarcoma.
In 1982, Brenner and cols.5 published a study entitled "Arteriovenous Kaposi-like Malformation with Angiodermatitis".
Strutton & Weedon6 consecrated the designation of acroangiodermatitis.
According to Partsch,7 acroangiodermatitis is only a variant of Favre and Chain angiodermatitis in patients suffering from chronic venous insufficiency.
Rüdlinger8 performed a meticulous study on the subject, demonstrating the similarities of the syndromes of Mali and of Stewart-Bluefarb with Kaposi's sarcoma, however they concluded that they were different above all in relation to the prognosis and proposed the expression Kaposi-like acroangiodermatitis.
A female patient, 57 years old, white, secretary, born and resident in Rio de Janeiro, presented erythematous-violaceous lesions on her legs (Figures 1 and 2) that were painless and non-pruriginous. They had appeared three weeks previously. In the exam of the inferior members, livedo reticularis and the presence of varicose veins of varying caliber were noted. The patient had venous stasis in the inferior members.
Routine laboratory exams did not reveal alterations, and the anti-HIV test was negative. Histopathology revealed hyperplastic epidermis, at times psoriasiform (Figures 3 and 4), and, in the papillary dermis and the middle reticular dermis, a proliferation of blood vessels covered by a tumescent endothelium with wide and irregular lumen, distributed singly or in groups, amid a moderate interstitial lymphohistiocytic infiltrate. Leakage of red blood cells was also noticed together with a thickening of the collagen fibers. The immunohistochemical test, using the CD-34 marker, was done with the intention of reaching a differential diagnosis between acroangiodermatitis, whose lesion does not present positive fusiform cells in CD-34, and Kaposi's sarcoma, the cells of which are 100% positive to CD-34, even in the most incipient lesions. The exam revealed an endothelium in the blood vessels that reacted positively to the primary antibodies against the CD-34 antigen. However in the preparations no oval endothelial or fusiform cells were observed in the interstices of the superior part of the reticular dermis nor were there oval cells among the collagen bundles that were positive to CD-34.
The treatment proposed was erythromycin 2g/day along with measures seeking to decrease the stasis. An improvement in the condition was observed, but without a complete regression of the lesions.
Partsch7 verified that in patients with postthrombotic syndrome acroangiodermatitis appeared in 60% of the male cases and 38% of female patients. Landthaler9 reported two cases of acroangiodermatitis following paralysis of the legs, which lead to a lack of circulation in the muscles. Pascher and Askin10 followed up the course of a patient with varicose veins for ten years; trying to clarify the case, they decided to perform a biopsy. The histopathological exam revealed aspects similar to those of Kaposi's sarcoma. Rozen11 studied the case of a 25-year-old woman that had erythematous-purpuric lesions with discreet ulceration and intense pain, which lead to the amputation of two fingers. The histopathology revealed alterations characteristic of acroangiodermatitis.
Jung and cols.12 studied the case of a male patient, 58 years old, with bilateral erythematous-purpuric lesions on the dorsal surface of the feet and toes. Histopathological exam suggested Kaposi's sarcoma; however based on certain histopathological details, they discarded that diagnosis. They concluded that acroangiodermatitis is a variety of "dermatitis due to stasis."
Badell and cols.13 described two cases of acroangiodermatitis owing to the use of a suction-socket prosthesis. Replacement of the prosthesis improved the picture.
Kim and cols.14 reported the appearance of two cases after hemodialysis as a consequence of iatrogenic arteriovenous fistula in the forearms. The lesions were painful and presented edema and erythematous vesicles and plaques in the hands and fingers.
In a synthesis of the etiopathology of this disease, Rashkovsky and cols.15 suggested the following possibilities: chronic venous insufficiency, arteriovenous malformations in the legs, iatrogenic arteriovenous shunt in patients under hemodialysis, paralysis of the legs and amputation of members.
In regard to the histopathology of this disease the broadest work was done by Era and cols.16 They studied ten cases thoroughly and their findings were: in the superficial and middle dermis, a proliferation of small vessels; a perivascular inflammatory process consisting of lymphocytes, histiocytes, eosinophiles and eventually, plasmocytes; leakage of red blood cells; deposits of hemosiderin and fibrosis. Although the epidermis is generally normal, in one case they observed spongiosis and discreet acanthosis. In all of the cases the antibody to Factor VIII was present, which demonstrates the proliferation of new vessels. In their study they suggested that the histological alterations resemble dermatitis due to stasis, however, they call attention to the fact that, in the latter, the histological involvement reaches the deep dermis, and the epidermal alterations are more intense. The purpura do not generally present fibrosis and eosinophiles. In vasculitis, the differential histological diagnosis is simplified by the presence of vascular fibrinoid necrosis.
The histopathological differential diagnosis of Kaposi's sarcoma is not always easy, however in acroangiodermatitis the vessels are quite regular without the fissures that are very common in Kaposi's sarcoma. In the latter, the leakage of red blood cells is not so intense and there is a lesser amount of hemosiderin. There are cases, however, in which the differential diagnosis becomes difficult. In such cases one should use the CD-34 antigen which is expressed in 100% of the cases of Kaposi's sarcoma and absent in acroangiodermatitis, according to the work of Kanitakis and cols.17 q
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17. Kanitakis J, Narvaez D, Claudy A. Expression of the CD34 antigen distinguishes Kaposi's sarcoma from pseudo-Kaposi's sarcoma (acroangiodermatitis). Brit J Dermatol 1996; 134: 44-46. [ Links ]
in January, 25th of 2001
Approved by the Consultive Council and accepted for publication in April, 22nd of 2003
* Work done at the Dermatology Institute, Santa Casa de Misericordia Hospital, Rio de Janeiro (Head: Prof. Dr. Rubem David Azulay).