Nodular fibrosis: a rare complication of non filarial lymph edema
Giti Sadeghian, Mohamad Ali NilfroushZadeh, Amir Hossein Siadat, Hengameh Ziaei
Dermatology Online Journal 14 (3): 20
Lymphedema is a soft-tissue swelling that occurs following lymphatic fluid accumulation. Edema is due to insufficient lymphatic drainage that occurs because of lymphatic vessel obstruction or absence . Chronic lymphedema can cause a clinical picture of tense non-pitting edema with thickened epidermis that is due to accumulation of protein and lipids in the connective tissue. In the early stages pitting edema is seen, but with progression of disease, fibrosis occurs and then edema becomes tense. Hyperplasic changes with warty epidermis and then elephantiasis become evident. The disability due to limb swelling can be severe but the overall prognosis is good . Nodular fibrosis is an uncommon complication of elephantiasis .
|Figure 1||Figure 2|
|Figure 1. Primary lymph edema of the lower extremity complicated by multiple nodular fibrosis lesions
Figure 2. Closer view of Figure 1
|Figure 3. Eosinophilic bundles of thick collagen with slight whorl pattern|
Our patient was a 70-year-old man that was referred to us because of elephantiasis, of both legs. The disease started when he was 30. At that time, it started with mild edema of right leg and ankle and then increased. The left leg was then involved after 1 year and with disease progression, both thighs were also involved. On physical examination, swelling and skin lichenification were evident on both legs. In addition, multiple skin-colored soft masses with diameter of 0.5-5 cm were observed on both legs. These appeared 2 years prior to presentation. The nodules were at first small, but gradually their diameters were increased and some became somewhat pedunculated (Fig. 1 & 2). The histopathology of these lesions showed mild acanthosis and hyperkeratosis with eosinophilic bundles of thick collagen with slight whorl pattern in the dermis (Fig. 3). This presentation was compatible with a diagnosis of nodular fibrosis. Laboratory tests were normal. The patient had no history of renal, hepatic and cardiovascular disease. The patient was not complaining of any disability except extreme swelling of the legs and serous discharge.
Lymph edema occurs because of insufficient lymphatic drainage that causes swelling of soft tissue due to excessive accumulation of fluid . Obstruction of the lymphatic system due to any cause can increase intravascular tissue protein: following osmotic pressure, some fluid is absorbed. Increase of extra vascular fluid protein will cause fibroblasts and mast cell proliferation that produces the clinical symptoms of tense non-pitting edema. The epidermis may become hyperkeratotic and warty  and this predisposes tissues to fissure and allows secondary infection. Recurrent cellulitis, erysipelas and dermato-lymphangio-adenitis are complications of chronic lymphedema . Other complications of lymphedema are papilomatosis , warty xanthomatous lesions , and formation of malignant tumors . Localized neurofibromatosis is reported following lymph edema due to mastectomy . Also, formation of follicular center cell lymphoma is reported secondary to praecox lymphedema . Nodular fibrosis is a rare complication of non-filarial elephantiasis of the legs. This complication is endemic in Ethiopians. The histopathology shows haphazard organization of collagen with a variable number of fibroblasts. There are also a small number of small vessels with coat-sleeves of lymphocytes . In the literature review, we found only 2 reports of this sequella of chronic lymphedema. The first case was reported as "soft fibroma-like lesions" on the legs of a patient with Kaposi sarcoma and lymphedema . The other case was reported as a nodular sub-epidermal fibrosis on the legs of a patient with primary chronic lymphedema . In the current case, multiple soft nodules with a diameter of 0.5-5.0 cm occurred on both legs of a patient with primary lymphedema.
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