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Acroangiodermatitis
Marissa Heller MD, Julie K Karen MD, William Fangman MD
Dermatology Online Journal 13 (1): 2

New York University Department of Dermatology

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Abstract
A 26-year-old man with a history of chronic primary lymphedema of the left lower extremity presented with elephantiasis, confluent, violaceous, mascerated plaques, and ulcers on the dorsal aspects of the toes of the left foot. Histopathologic examination showed a proliferation of small blood vessels associated with extravasated erythrocytes and hemosiderin deposits consistent with the diagnosis of acroangiodermatitis. Treatment of the focal ulcers includes compression therapy, local wound care, and surgical elimination of the shunt if there is an associated arteriovenous malformation.


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Clinical synopsis
A 26-year-old man was referred to the Charles C. Harris Skin and Cancer Pavilion for the evaluation and treatment of ulcers of the toes of the left foot. There was a remote history of a fractured left leg. Chronic primary lymphedema of the left leg developed three years ago. He subsequently developed a deep venous thrombus in his left leg with a pulmonary embolism that required pulmonary endarterectomy. A filter was placed in the interior vena cava, and he was treated with coumadin. Over the past three years, he noted purple skin lesions and development of ulcers on the toes.

Physical examination revealed confluent, violaceous, mascerated plaques with ulcers were present on the dorsal aspects of the toes of the left foot. The dorsal aspect of the foot exhibited verrucous changes. There was 1+ edema of the left foot, and a 1+ dorsalis pedis pulse was noted. The right foot was uninvolved.

 
Figure 3
Histopathology reveals a thick papillary dermis with proliferation of capillaries and venules with plump endothelial cells. There are extravasated erythrocytes and hemosiderin deposition. There is an infiltrate of plasma cells and some lymphocytes.



Comment
Acroangiodermatitis, a rare condition that is also known as pseudo-Kaposi sarcoma, is a proliferation of the preexisting vasculature. Clinically, it appears as violaceous or brown macules, patches, papules, or plaques on the distal aspects of the lower extremities. It may be unilateral or bilateral and is commonly located over the extensor surfaces, the lateral malleoli, and the dorsal aspects of the feet. Ulcers may develop over the affected areas [1].

Acroangiodermatitis can be associated with venous hypertension, with an arteriovenous malformation, or with an acquired iatrogenic arteriovenous fistula [1, 2]. A variety of other vascular conditions has been reported in association with acroangiodermatitis. These include limb paralysis, vascular damage from amputation or intravenous drug abuse, the thrombophilic 20210A mutation in the prothrombin gene, and Klippel-Trenaunay syndrome [1, 3, 4, 5].

Histopathologic examination shows proliferation of the capillary bed throughout the dermis. Extravasated erythrocytes, fibrosis with spindle cells, and hemosiderin pigment deposition are noted. There may be a superficial, perivascular infiltrate of lymphocytes, macrophages, and eosinophils. Endothelial cells lining the vessels stain with CD34. A differentiating factor between acroangiodermatitis and Kaposi sarcoma is that in the former, the vascular hyperplasia is of preexisting vasculature and in the latter, the vascular hyperplasia is independent of preexisting vasculature [6].

Treatment of acroangiodermatitis involves correction of the underlying vascular pathology. Mainstays of therapy include compression stockings or a compression pump for venous stasis and local wound care for ulcers. In the case of arteriovenous malformations, surgical correction of the shunt can be employed [7]. Medical therapy options are limited. There is one case report of regression of lesions with treatment with oral dapsone 50 mg twice daily for three months in combination with leg elevation and compression [8]. There are case reports of improvement with oral erythromycin in patients with arteriovenous fistulas for hemodialysis [9].

References
1. Rongioletti F, Rebora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol 2003;49:887

2. Samad A, Dodds S. Acroangiodermatitis: review of the literature and report of a case associated with symmetrical foot ulcers. Eur J Vasc Endovasc Surg 2002;24:558

3. Gucluer H, et al. Kaposi-like acroangiodermatitis in an amputee. Br J Dermatol 1999;141:380

4. Martin L, et al. Acroangiodermatitis in a carrier of the thrombophilic 20210A mutation in the prothrombin gene. Br J Dermatol 1999;141:752

5. Lyle WG, Given KS. Acroangiodermatitis (pseudo-Kaposi sarcoma) associated with Klippel-Trenaunay syndrome. Ann Plast Surg 1996;37:654

6. Calonje E, Wilson-Jones E. Vascular tumors: tumors and tumor-like conditions of blood vessels and lymphatics. In: Elder D, et al, eds. Lever's Histopathology of the Skin. 8th ed. Philadelphia: Lippincott-Raven, 1997:914

7. Pires A, et al. Effect of compression therapy on a pseudo-Kaposi sarcoma. Dermatology 1999;198:439

8. Rashkovsky I, et al. Acro-angiodermatitis: review of the literature and report of a case. Acta Derm Venereol (Stockh) 1995;75:475

9. Kim TH, et al. Pseudo-Kaposi's sarcoma associated with acquired arteriovenous fistula. J Dermatol 1997;24:28

Survivor seizes chance to make people aware
Mark Goldstein, 74, developed, overcame bout of breast cancer
BY DAVE BENJAMIN Staff Writer



Mark Goldstein 

JACKSON - He is a survivor and his aim is to heighten the awareness that men can develop breast cancer.

"Statistically, this is a woman's disease and there is no doubt about that" said Mark Goldstein, 74, a 19-year survivor male breast cancer.

Goldstein, who lives in northern New Jersey, spoke to the Men's Club at the Westlake Golf and Country Club adult community on Sept. 20.

"There are about 185,000 women diagnosed with breast cancer each year, compared to 2,030 men who are diagnosed yearly, but it's just as tragic for a man lose his life to this form of the disease as it is for a woman to lose her life," he said.

Goldstein said many men cannot believe they can develop what is thought as a woman's disease,

"If you don't think that is shocking then all you would have to do is to have been in my shoes at that time when everybody around me expressed, some seriously and some with a wink, that 'Mark got what women get,' " he said.

Before that, Goldstein said, he did not have a clue that men could develop breast cancer. That all changed in 1988.

Goldstein said one day in February 1988 he was shaving when he looked in the mirror and noticed that his left nipple was receding and beneath it he could feel a lump. In typical male fashion he did nothing.

"Had that been my wife, we would have been in the doctor's office the next day, but for me there was not only no reaction, there was inaction," he said. "I did nothing for three months."

He said about 2,000 men are diagnosed with breast cancer every year and about 450 succumb to the disease. That number is increasing, he said.

"But you can't find (male) breast cancer listed under the 15 conventional cancers," he said. "There's prostate, colon, bladder, melanoma, lymphoma and on and on. We're off the radar in terms of awareness."

He told his audience that if breast cancer was a sport, men would know all about it.

"We would have all the statistics on it," he said. "We would chart it. We would socialize about it and it would probably have a uniform, but it's not a sport."

Goldstein said embarrassment, fear and denial all play a role in the delay of seeking help. He said the public and insurance companies must be educated on the subject.

"I submitted a claim to my wife's company and it came back with a note that said the service that has been billed cannot be performed on a person of this sex," Goldstein said. "I called them up and I didn't have to say much, just the word 'sue.' "

Goldstein underwent treatment for the disease - chemotherapy, radiation and a modified radical mastectomy - and from 1988 and 1992, "I lived a relatively normal post-breast cancer life," he said. "I did my job. I engaged in activities that I had done before and about halfway through [that period of time] I felt I transitioned from being a survivor to being a conqueror. Then one day in September 1992 I felt I reached a transition [again] from a survivor and conqueror to becoming an advocate."

Goldstein said he heard about a race in New York City and knew he had to participate in the "For Women Only Race for the Cure." He filled out the race application using only his first initial and paid the entry fee with a money order. On race day he appeared at the starting line.

"This was the start of my advocacy," he told the audience. "When I presented myself they said I couldn't run because I was a man," but that did not stop Goldstein and off he went.

Goldstein said there are different kinds of tragedies and said they typically happen to somebody else.

"When these tragedies occur there is a response," he said. "People who see these tragedies say, 'Thank God it wasn't me.' "

Goldstein said there are also personal tragedies where people ask "Why me?" Those individuals focus on questions like "What did I do to bring this to myself?" "What could I have done to stop it from happening?" and "What should I do about what's happened to me?"

It is the "see and seize" group that converges with the "what should I do?" people from the "why me?" group who act.

"From that comes the 'why not me?' group who says they can do something and the most perfect symbol of this is Nancy Brinker, who in 1982 made a pledge to her dying sister Susan (Komen) that she would do everything she could to eradicate breast cancer," Goldstein said. "That was the start of the Susan G. Komen Breast Cancer Foundation, the foundation we support here at Westlake."

Goldstein said that in their response to breast cancer, women have started foundations, initiated crusades, designated awareness months, formed networks, lobbied public officials, walked, run and marched.

"Twenty years ago breast cancer was a closeted subject and no one spoke about it," he said, "but the Komen Race for the Cure has turned that around."

Goldstein said he has run in every Susan G. Komen Race for the Cure, and others, for a total of 167 races throughout the United States and internationally. In 2005 he was inducted into the National Distance Running Hall of Fame

"I am fortunate to have the endorsement by way of sponsorship of the New Balance Athletic Shoe Company. I am a member of the honorary Team New Balance which is a team of survivors and I'm the only male survivor on that team."

Goldstein, said he participates in the Race for the Cure to dispel the misconception that breast cancer is only a women's disease.

Goldstein said he appreciates what the Westlake Men's Club has done in its support of the Race for the Cure,

Goldstein said he lives with lymphedema, which is the byproduct of excessive lymph removal that occurred during the modified radical mastectomy he underwent 19 years ago. Lymphedema is incurable.

Men's Club Vice President Larry Hartman said the organization is 6 years old. Between 80 and 100 men attend monthly meetings. The club sponsors a Little League baseball team, provides scholarships to Jackson high school students, raises funds to send phone cards to New Jersey soldiers who are serving in Iraq and Afghanistan, helps fund the Race for the Cure and supports research into prostate cancer and autism.

Living Columnist Kathy Kemp

Miss Ezell: A treasure and friend

One spring day in 1990, I decided to help a little old lady fix her roof.

It was ancient and leaking, Nora Ezell told me. She had been up there, hammering shingles, the day I stopped by to interview her for a story about her quilts.

I'd already seen pictures of her colorful bed covers. Some of her designs- such as the "wedding ring" and the "friendship chain" - had been passed through generations. It was her "story" quilts, whose panels often celebrated the accomplishments of black Americans, that were attracting folklorists and art dealers. (See them in the Alabama Folk Art exhibit in the Young & Vann building, 1731 First Ave. North, through December).


"Miss Ezell," as I came to call her, quit hammering long enough to tell me her life's story: One of 10 children, she was born in Mississippi and grew up west of Birmingham. Her father farmed and her mother quilted. Young Nora dropped out of school, married, divorced, married again, worked in a garment factory, underwent a double mastectomy and beat cancer, only to lose her only child, Annie Ruth, to the same disease. Miss Ezell took up quilting in the 1980s to entertain her sick daughter.

She told me this, and much more, during my visit to her modest frame house, in the tiny Greene County town of Mantua. She was then 72 and suffering from lymphedema, which caused her arm to swell. I was decades younger and physically fit. So when Miss Ezell told me of her roof problems, I offered to help.

Never mind my fear of heights, or the fact that my construction skills mostly involved nailing picture hangers to the wall.

The following Saturday, a friend and I drove to Mantua and found Miss Ezell in her backyard, a ladder propped against the house. A roll of tar paper lay on the grass. "Let us do that," I told her as I grabbed one end of the roll and my friend the other. It must have weighed 75 pounds. We couldn't budge it.

Miss Ezell eyed us with disdain. Something she had told me in our interview kept ringing in my ears: "I cannot stand stupid people."

She stepped past us, lifted that roll to her shoulder and climbed the ladder to the roof. "Y'all coming?" she asked. By then we were all giggling. "Watch what your mama does," Miss Ezell said as she tarred and nailed down the roofing paper.

Needless to say, she did all the work. My friend didn't have much of a clue, and I sat on the ridge, too terrified to move. Even so, I got covered in tar. Miss Ezell loaned me a muumuu to wear on the ride home.

We'd had such fun that I returned to "help" her with other home repairs. Or I'd see her at art shows, where she regularly introduced me as her daughter.

Miss Ezell died of a stroke earlier this month at age 88. Around the world, various obituaries noted that her quilts have hung in the Smithsonian Institution and in New York's American Folk Art Museum, and that she won a National Heritage Fellowship in 1992.

There are plenty of folks like me, in Alabama and beyond, who also admired her spirit and benefited from her kindness and wisdom. She was a national treasure, in more ways than one. E-mail
kkemp@bhamnews.com.