Acroangiodermatitis of mali and stewartbluefarb syndrome. It usually begins early in life, unilaterally over lower extremities presenting as. The diagnosis was confirmed using histopathological and. It is a proliferation of preexisting vasculature seen in venous hypertension, arteriovenous malformation, or acquired iatrogenic arteriovenous av fistula.
Two years later, bluefarb and adams 10 described kaposiform skin lesions in one patient with a congenital arteriovenous malformation without chronic venous. Acroangiodermatitis is a benign uncommon vasoproliferative disorder that affects the lower extremities. Histopathological examination of the lesion showed lobular proliferation of small. Acroangiodermatitis of mali acroangiodermatitis of mali george, m carr, r. On histopathology, papules and nodules consist of a proliferation of small dilated vessels in an edematous dermis. Apr 01, 2010 acroangiodermatitis of mali george, m carr, r. The term acroangio dermatitis was introduced in 1965 by mali et al. It has been described in patients with increased venous pressure and stasis caused by venous insufficiency, congenitalacquired arteriovenous mali et al. Acroangiodermatitis secondary to chronic venous insufficiency see erratum 2011. Mali type acroangiodermatitis begins in the form of violaceus macules and patches, and it can transform into painless papulonodules or persistent plaques. Chapter 1 introducton histopathology definition it is a branch of pathology which deals with the study of disease in a tissue section. Acroangiodermatitis mali resulting from arteriovenous malformation.
Dermis acroangiodermatitis mali information on the diagnosis. Acroangiodermatitis or pseudokaposi sarcoma is a rare angioproliferative entity, related to chronic venous insuf. Summary acroangiodermatitis synonym pseudo kaposi sarcoma. The term acroangiodermatitis of mali was introduced for the first time by mali et al. The authors report a case of acroangiodermatitis of mali type of the bilateral legs that was misdiagnosed for 3 years. We report a 17yearold girl with congenital spina bifida and myelomeningocele and cutaneous lesions localized on the lower limbs. Department of histopathology, south warwickshire general hospital, west midlands, uk. Our patient had a case of malitype acroangiodermatitis beginning at a young er age in contrast to the most frequent cases, and it occurred unilaterally. Acroangiodermatitis of mali is a rare cutaneous condition often characterized by purplishblue to brown papules and plaques on the medial and lateral malleolus of both legs 1604 acroangiodermatitis is a rare skin condition characterised by hyperplasia of preexisting vasculature due to venous hypertension from severe chronic venous stasis. Acroangiodermatitis of mali george 2010 clinical and. Acroangiodermatitis is a reactive angiodysplasia of cutaneous blood vessels.
He had deformed joints with hepatomegaly and splenomegaly. Capillary proliferation an overview sciencedirect topics. Histopathology remains one of the major tools of diagnosis in mycology20 table 3. Mar 03, 2014 acroangiodermatitis pseudokaposis sarcoma, coined by mali et al 12 in 1965, is described as a rare skin disease characterized by the presence of hyperplasia of preexisting vasculature. Acroangiodermatitis aad is a rare benign vascular proliferation characterized by violaceous patches or plaques of the extremities resembling kaposi sarcoma. Fixation, tissue processing, histologyand immunohistochemistry procedures for diagnosis of animal tse bse, scrapie, atypical scrapie,cwd pathology department, apha. The clinical and histopathological lesions resembled kaposis sarcoma. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red. Unilateral presentation of pseudokaposis secondary to superficial venous insufficiency clin dermatol j. It would be difficult to propose a system for histopathology and anatomical pathology services applicable to all developing countries. Acroangiodermatitis aad is a rare vasoproliferative disorder, usually of the lower.
There are 0 terms under the parent term acroangiodermatitis in the icd10cm alphabetical index. Acroangiodermatitis, often known as pseudokaposi sarcoma, is an. Acroangiodermatitis of mali pseudokaposi sarcoma associated. A rare vascular phenomenon arpita a mehta, rickson r pereira, chitra s nayak, rachita s dhurat department of dermatology, t. The icd10cm alphabetical index is designed to allow medical coders to look up various medical terms and connect them with the appropriate icd codes. To achieve this it is important that the tissue must be prepared in such a manner. Acroangiodermatitis pseudokaposi sarcoma acroangiodermatitis is a reactive dermal capillary proliferation that occurs on the lower extremities of patients with severe chronic venous insufficiency mali type 58 or congenital arteriovenous malformations stewart.
To provide a standardized method for the appropriate filing andor retrieval of avoid being destroyed or eaten by pets. Acroangiodermatitis of mali in a patient with congenital. The tissue undergoes a series of steps before it reaches the diagnosis. With chapters on organbased approaches with specific protocols for morphologic, molecular examination, and pathological observations governing the therapeutic management of the diseases.
Acroangiodermatitis of mali stewartbluefarb syndrome acroangiodermatitis is a benign vascular process which histologically closely resembles kaposis sarcoma and had thus been named pseudokaposis sarcoma. Nonhealing ulcer is a cause for significant morbidity and mortality. Acroangiodermatitis of mali stewartbluefarb syndrome acroangiodermatitis is a benign vascular process which histologically closely resembles kaposis sarcoma and had thus been named pseudokaposis sarcoma acroangiodermatitis is often associated with different disorders like. In more advanced, tumorous stages of kaposis sarcoma, cellular atypia becomes apparent and allows differentiation between malis acroangiodermatitis and true kaposis sarcoma. Acroangiodermatitis pseudokaposis sarcoma associated with klippeltrenaunay syndrome. A previous biopsy for histopathology was wrongly interpreted. Kaposi sarcoma and requires histopathological examination for its diagnosis. Acroangiodermatitis of mali in a patient with congenital myopathy. Histopathological examination from one of the lesions showed hyperplastic. Although benign, it must be differentiated from malignant conditions like kaposis sarcoma, especially in the present hiv era and therefore, histopathological examination is. The major advantages of histopathology are speed, low cost and the ability to provide a presumptive identification of the infecting fungus as well as demonstrating the tissue reaction. Before presentation, he had also developed similar smaller superficial lesions below this area. Parkes weber syndrome pws is a rare disorder characterised by arteriovenous av fistula, along with capillary, lymphatic, venous malformations and limb hypertrophy.
Case of bilateral acroangiodermatitis due to symmetrical arteriovenous fistulas of the soles. Chitra s nayak 302 arun, 6th road, santacruz east, mumbai400 055 india. To achieve this it is important that the tissue must be prepared in such a manner that it is sufficiently thick or thin to be examined microscopically and all. The term is derived from the greek histos meaning web or tissue, and refers to the science of tissues. Kaposilike acroangiodermatitis or pseudokaposis sarcoma is a reactive angiodysplasia of the cutaneous blood vessels that resembles true kaposis sarcoma, both clinically and histologically. Our patient had a case of mali type acroangiodermatitis beginning at a young er age in contrast to the most frequent cases, and it occurred unilaterally. Thus, the histopathology confirmed acroangiodermatitis of mali. Acroangiodermatitis of mali resulting from arteriovenous malformation. The cases presented here demonstrate 2 potential variants of aad. Results of the histopathologic analysis indicated ac roangiodermatitis. Acroangiodermatitis, often known as pseudokaposi sarcoma, is an uncommon angioprolifera tive entity related to chronic venous insufficiency, arteriovenous fistulae, paralysed limbs, amputation. Claudy, expression of the cd34 antigen distinguishes kaposis sarcoma from pseudokaposis sarcoma acroangiodermatitis, the british journal of. Spontaneous acroangiodermatitis coban i, kokenekunal td.
Most of histopathological techniques simulating to those of. It appears to be a reactive phenomenon related to severe chronic venous insufficiency and stasis of the lower extremities. Unilateral presentation of pseudokaposis secondary to. Histology page 1 histological techniques histology is the study of the cellular organization of body tissues and organs. Nonhea lc arly set nilateral ality acroangiodermatitis. Acroangiodermatitis is a reactive dermal capillary proliferation that occurs on the lower extremities of patients with severe chronic venous insufficiency mali type 58 or congenital arteriovenous malformations stewartbluefarb syndrome. Methods and protocols provides a comprehensive guide to the current issues in histopathology. On examination, the patient had pallor, icterus, and generalized lymphadenopathy with a nonhealing unhealthy ulcer over left medial malleolus. Acroangiodermatitis of mali in protein c deficiency due to a novel proc gene mutation. Department of histopathology postgraduate institute of medical education and research, chandigarh, india.
Acroangiodermatitis pseudokaposis sarcoma, coined by mali et al 12 in 1965, is described as a rare skin disease characterized by the presence of hyperplasia of preexisting vasculature. Stewartbluefarb syndrome is a variant of acroangiodermatitis, which is associated with congenital av malformationfistulas. Acroangiodermatitis synonym pseudokaposi sarcoma is an unusual, benign condition which clinically presents as purplecolored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. With chapters on organbased approaches with specific protocols for morphologic, molecular examination, and pathological observations governing the therapeutic management of. Acroangiodermatitis of mali, chronic venous insufficiency, pseudokaposi. It is a proliferation of preexisting vasculature seen in chronic venous insufficiency, arteriovenous malformation, or acquired iatrogenic arteriovenous av fistula. An 18yearold male presented with a nonhealing wound on left lower limb, pain and swelling over multiple joints, weight loss, and yellowish discoloration of eyes and urine for the past 4 years. Pseudokaposi sarcoma acroangiodermatitis treatment. Histopathological techniques histopathology is the branch of pathology which concerns with the demonstration of minute structural alterations in tissues as a result of disease. Histopathologists provide diagnostic expertise and advice for clinical colleagues, and work in clinical teams that care for patients. Try out the html to pdf api handling of histopathology materials. Mali described the characteristic lesions as peculiar purple macular plaques on the extensor aspects of the digits or foot.
Acroangiodermatitis of mali is a rare cutaneous condition often characterized by purplishblue to brown papules and plaques on the medial and lateral malleolus of both legs. Pathology of acroangiodermatitis pseudokaposis sarcoma. View the article pdf and any associated supplements and figures for a period of 48 hours. Such an ulcer must undergo a biopsy so that an appropriate diagnosis can be reached and treatment instituted rapidly acroangiodermatitis of mali is an uncommon but not rare cause of nonhealing wound in the lower limbs. Malitype acroangiodermatitis begins in the form of violaceus macules and patches, and it can transform into painless papulonodules or persistent plaques. Acroangiodermatitis of stewartbluefarb poses a diagnostic dilemma with ks and klippeltrenaunay syndrome kts. Although benign, it must be differentiated from malignant conditions like kaposis sarcoma, especially in the present hiv era and therefore, histopathological examination is a must for its diagnosis and differentiation. The tissue undergoes a series of steps before it reaches the examiners desk to be thoroughly examined microscopically to arrive at a particular diagnosis. It is a branch of pathology which deals with the study of disease in a tissue section. Dermis acroangiodermatitis mali information on the. Acroangiodermatitis, dermite ocre of favre, gravitational purpura, purpura, stasis, stasis purpura. Histopathology techniques histopathology definition. Acroangiodermatitis of mali also called as pseudokaposi s sarcoma is a very rare, benign condition which clinically presents as purplecolored patches, plaques or nodules, mostly on the extensor.
254 1425 39 450 1375 983 1637 807 1573 688 1359 1382 1301 498 1242 463 826 450 1533 1219 733 1305 78 533 395 3 937 1219 445 644 258 1317 402