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A 60-year-old woman presents to dermatology with a longstanding complaint of a tender, irritated mass hanging from her lower abdomen. The patient says it started as a large fold in her lower abdomen but over the years has grown and become more pendulous. It is now large enough to interfere with normal activity, including walking.
Numerous providers, dermatologists included, have rendered diagnoses—most recently, hidradenitis suppurativa. The antibiotics prescribed for that diagnosis have not helped, however. Similarly, cultures performed on samples from draining sores on the mass’s posterior have failed to illuminate the situation, showing only mixed normal flora.
The patient’s primary care provider referred her to surgery for consideration of removal, or at least reduction, of the mass. The surgeon offered a presumptive diagnosis of elephantiasis nostras verrucosa of the pannus and agreed to perform the surgery. But the patient’s primary care provider requested a second opinion from dermatology.
EXAMINATION
The edematous, pendulous mass is the size of a soccer ball and hangs down from its abdominal base. When the patient stands, the mass stretches almost to the level of her knees. The anterior surface is edematous but otherwise normal in appearance. The intertriginous surfaces of the lesion look entirely different, with multiple small, draining puncta and a few open comedones.
The body of the mass is quite indurated but is neither hot nor tender. There are no comedones or cysts in other intertriginous locations, as might be seen in hidradenitis.
What’s the diagnosis?
DISCUSSION
This case involves a rare entity: vast lymphedema of the pannus leading to the formation of a pendulous mass so large that it filled the space between the patient’s legs, causing pain and discomfort. These findings are analogous to those seen in advanced venous insufficiency. Both manifestations share a name: elephantiasis nostras verrucosa. (Neither has anything to do with the more notorious cases of filarial elephantiasis seen in tropical locations.)
Elephantiasis nostras verrucosa of the lower extremities involves striking skin changes: edema, along with extreme thickening, papularity, and roughness of the skin. These typically manifest downward from just below the knee. The condition represents the effects of late-stage chronic venous insufficiency, often worsened by obesity and a sedentary lifestyle. Other causes of dependent lymphedema, such as congestive heart failure, can also contribute to the problem.
This same pathophysiologic process can affect other areas as well—including the pannus, as seen in this case. Since I had only ever encountered this problem in legs, I did a literature search. I found several references, all of which indicated that surgical removal (panniculectomy) was the best treatment. I could not find any information on the success rate of this surgery, but I did refer the patient back to the surgeon, who had made the correct diagnosis.
TAKE-HOME LEARNING POINTS
- Elephantiasis nostras verrucosa (ENV) is a consequence of uncontrolled venous insufficiency that commonly manifests on lower extremities.
- ENV is a distinctly rare (though not unknown) problem when areas other than legs are affected.
- This patient’s condition is, in my opinion, beyond the reach of medical treatment. But in milder cases, approaches such as weight loss and use of diuretics have been tried with mixed success.
- The best treatment appears to be surgical removal, which is not without potential complications: risk for infection, pain, prolonged recovery time, and wound dehiscence; these issues were discussed thoroughly with the case patient.
A 60-year-old woman presents to dermatology with a longstanding complaint of a tender, irritated mass hanging from her lower abdomen. The patient says it started as a large fold in her lower abdomen but over the years has grown and become more pendulous. It is now large enough to interfere with normal activity, including walking.
Numerous providers, dermatologists included, have rendered diagnoses—most recently, hidradenitis suppurativa. The antibiotics prescribed for that diagnosis have not helped, however. Similarly, cultures performed on samples from draining sores on the mass’s posterior have failed to illuminate the situation, showing only mixed normal flora.
The patient’s primary care provider referred her to surgery for consideration of removal, or at least reduction, of the mass. The surgeon offered a presumptive diagnosis of elephantiasis nostras verrucosa of the pannus and agreed to perform the surgery. But the patient’s primary care provider requested a second opinion from dermatology.
EXAMINATION
The edematous, pendulous mass is the size of a soccer ball and hangs down from its abdominal base. When the patient stands, the mass stretches almost to the level of her knees. The anterior surface is edematous but otherwise normal in appearance. The intertriginous surfaces of the lesion look entirely different, with multiple small, draining puncta and a few open comedones.
The body of the mass is quite indurated but is neither hot nor tender. There are no comedones or cysts in other intertriginous locations, as might be seen in hidradenitis.
What’s the diagnosis?
DISCUSSION
This case involves a rare entity: vast lymphedema of the pannus leading to the formation of a pendulous mass so large that it filled the space between the patient’s legs, causing pain and discomfort. These findings are analogous to those seen in advanced venous insufficiency. Both manifestations share a name: elephantiasis nostras verrucosa. (Neither has anything to do with the more notorious cases of filarial elephantiasis seen in tropical locations.)
Elephantiasis nostras verrucosa of the lower extremities involves striking skin changes: edema, along with extreme thickening, papularity, and roughness of the skin. These typically manifest downward from just below the knee. The condition represents the effects of late-stage chronic venous insufficiency, often worsened by obesity and a sedentary lifestyle. Other causes of dependent lymphedema, such as congestive heart failure, can also contribute to the problem.
This same pathophysiologic process can affect other areas as well—including the pannus, as seen in this case. Since I had only ever encountered this problem in legs, I did a literature search. I found several references, all of which indicated that surgical removal (panniculectomy) was the best treatment. I could not find any information on the success rate of this surgery, but I did refer the patient back to the surgeon, who had made the correct diagnosis.
TAKE-HOME LEARNING POINTS
- Elephantiasis nostras verrucosa (ENV) is a consequence of uncontrolled venous insufficiency that commonly manifests on lower extremities.
- ENV is a distinctly rare (though not unknown) problem when areas other than legs are affected.
- This patient’s condition is, in my opinion, beyond the reach of medical treatment. But in milder cases, approaches such as weight loss and use of diuretics have been tried with mixed success.
- The best treatment appears to be surgical removal, which is not without potential complications: risk for infection, pain, prolonged recovery time, and wound dehiscence; these issues were discussed thoroughly with the case patient.
A 60-year-old woman presents to dermatology with a longstanding complaint of a tender, irritated mass hanging from her lower abdomen. The patient says it started as a large fold in her lower abdomen but over the years has grown and become more pendulous. It is now large enough to interfere with normal activity, including walking.
Numerous providers, dermatologists included, have rendered diagnoses—most recently, hidradenitis suppurativa. The antibiotics prescribed for that diagnosis have not helped, however. Similarly, cultures performed on samples from draining sores on the mass’s posterior have failed to illuminate the situation, showing only mixed normal flora.
The patient’s primary care provider referred her to surgery for consideration of removal, or at least reduction, of the mass. The surgeon offered a presumptive diagnosis of elephantiasis nostras verrucosa of the pannus and agreed to perform the surgery. But the patient’s primary care provider requested a second opinion from dermatology.
EXAMINATION
The edematous, pendulous mass is the size of a soccer ball and hangs down from its abdominal base. When the patient stands, the mass stretches almost to the level of her knees. The anterior surface is edematous but otherwise normal in appearance. The intertriginous surfaces of the lesion look entirely different, with multiple small, draining puncta and a few open comedones.
The body of the mass is quite indurated but is neither hot nor tender. There are no comedones or cysts in other intertriginous locations, as might be seen in hidradenitis.
What’s the diagnosis?
DISCUSSION
This case involves a rare entity: vast lymphedema of the pannus leading to the formation of a pendulous mass so large that it filled the space between the patient’s legs, causing pain and discomfort. These findings are analogous to those seen in advanced venous insufficiency. Both manifestations share a name: elephantiasis nostras verrucosa. (Neither has anything to do with the more notorious cases of filarial elephantiasis seen in tropical locations.)
Elephantiasis nostras verrucosa of the lower extremities involves striking skin changes: edema, along with extreme thickening, papularity, and roughness of the skin. These typically manifest downward from just below the knee. The condition represents the effects of late-stage chronic venous insufficiency, often worsened by obesity and a sedentary lifestyle. Other causes of dependent lymphedema, such as congestive heart failure, can also contribute to the problem.
This same pathophysiologic process can affect other areas as well—including the pannus, as seen in this case. Since I had only ever encountered this problem in legs, I did a literature search. I found several references, all of which indicated that surgical removal (panniculectomy) was the best treatment. I could not find any information on the success rate of this surgery, but I did refer the patient back to the surgeon, who had made the correct diagnosis.
TAKE-HOME LEARNING POINTS
- Elephantiasis nostras verrucosa (ENV) is a consequence of uncontrolled venous insufficiency that commonly manifests on lower extremities.
- ENV is a distinctly rare (though not unknown) problem when areas other than legs are affected.
- This patient’s condition is, in my opinion, beyond the reach of medical treatment. But in milder cases, approaches such as weight loss and use of diuretics have been tried with mixed success.
- The best treatment appears to be surgical removal, which is not without potential complications: risk for infection, pain, prolonged recovery time, and wound dehiscence; these issues were discussed thoroughly with the case patient.
