Successful treatment by negative-pressure wound therapy for ulcer located on diffuse plexiform neurofibroma.

Dear Editor,

Hematoma is common within diffuse plexiform neurofibroma (dNF) and can lead to skin ulceration. We encountered two neurofibromatosis type 1 (NF1) patients whose ulcers due to hematoma in dNF were successfully treated by negative‐pressure wound therapy (NPWT).

Case 1 was a 48‐year‐old Japanese male NF1 patient who was referred to us with a few‐day history of an ulcer on a dNF on the right buttock (Fig. 1a). The patient had noticed swelling of the dNF on the buttock without any apparent cause and was referred to another hospital where the nodule was incised, and the hematoma was removed. Physical examination on the first visit to us revealed an ulcer with an opening size of 4.0 cm × 1.5 cm and a depth of 7.0 cm located on the dNF (Fig. 1a). Computed tomography revealed a tissue defect in the area of heterogeneous density (Fig. S1a). We performed NPWT to treat the ulcer using V.A.C.® (Acelity L.P., San Antonio, TX, USA). After therapy with negative pressure at 75 mmHg for 14 days, the ulcer was markedly improved (opening size, 2.0 cm × 0.8 cm; depth, 2.5 cm) (Fig. 1b).

Figure 1

(a) Clinical findings for case 1 at the first visit. A deep ulcer is evident on the diffuse plexiform neurofibroma in the right buttock. (b) Clinical findings for case 1 after negative‐pressure wound therapy (NPWT) for 14 days. The ulcer has improved markedly. (c) Clinical findings for case 2 at the first visit. A purplish, swollen, nodular lesion is evident in the right chest wall. Severe scoliosis and a nasal cannula for home oxygen therapy to treat restrictive thoracic disease due to severe scoliosis are also seen. (d) Clinical findings for case 2 at 13 days after the first visit. Part of the skin covering the nodular lesion has become necrotic and ulcerated, and the nodular lesion has started to shrink. (e) Clinical findings after 7 days of NPWT. The ulcer is markedly improved.

Case 2 was a 43‐year‐old Japanese female NF1 patient who was referred to us with a 2‐day history of swelling of a dNF on the right chest wall without any apparent cause (Fig. 1c). Physical examination revealed a tender, purplish, nodular lesion 22 cm in diameter on the right chest wall and severe scoliosis. Thirteen days after the first visit, part of the skin covering the nodular lesion became necrotic, resulting in an ulceration with an opening size of 6.0 cm × 1.5 cm and 8.0 cm in depth, from which bloody exudate was discharged. Consequently, the nodular lesion consisting of dNF began to shrink (Fig. 1d). Computed tomography revealed a tissue defect in the area of heterogeneous density (Fig. S1b). We performed NPWT to treat the ulcer using V.A.C. with negative pressure at 125 mmHg for 7 days. The ulcer subsequently improved (opening size, 3.0 cm × 0.8 cm; depth, 4.5 cm) (Fig. 1e).

Negative pressure has been utilized for the treatment of wounds since the 1940s.1 Kinetic Concepts, which is now affiliated with Acelity L.P., has patented a method under the brand name of V.A.C., and the generic term NPWT is widely used for various types of ulcers.1 Some reports have described the application of NPWT to the wound after the resection of neurofibroma.2, 3, 4 In addition, Cavallaro et al. reported using NPWT for an ulcer arising due to embolization for the treatment of plexiform neurofibroma.5

Previously, it has remained unclear whether NPWT is likely to cause bleeding from diffuse plexiform neurofibromas for which embolization has not been performed. Because we were at first concerned regarding the risk of bleeding due to negative pressure, we performed NPWT in case 1 at the relatively low negative pressure of 75 mmHg. This report suggests that NPWT is potentially useful and safe as a therapy for ulcers located on dNF, even if the tumor has abundant fragile blood vessels causing hematoma.

Conflict of Interest

None declared.

Fig. S1. Computed tomography findings in (a) case 1 and (b) case 2 before negative‐pressure wound therapy. Both examinations show tissue defects in the area of heterogeneous density.

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