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Case Reports
. 2023 Apr 24;15(4):e38074.
doi: 10.7759/cureus.38074. eCollection 2023 Apr.

Buschke-Ollendorff Syndrome: A Rare Cause of Unilateral Genu Valgum

Şafak Aydın Şimşek  1 , Tolgahan Cengiz  1 , Oğuzhan Muslu  2 , Bedirhan Albayrak  1 , İsmail Büyükceran  1 , Hüseyin Sina Coşkun  1 , Nevzat Dabak  1
Affiliations
Case Reports

Buschke-Ollendorff Syndrome: A Rare Cause of Unilateral Genu Valgum

Şafak Aydın Şimşek et al. Cureus. .

Abstract

Buschke-Ollendorff syndrome is a rare, often benign, autosomal dominant skin disorder. This syndrome commonly presents with non-tender connective tissue nevi and sclerotic bony lesions. Characteristic skeletal findings such as melorheostosis and hyperostosis are usually present. Most cases are detected incidentally. Skin lesions appear first and become less noticeable with age. Bone lesions occur in the later decades of life. Another rarely associated symptom, melorheostosis, is manifested by the appearance of wax running through the cortex of the bone. Plain radiographs usually show cortical hyperostosis. This study aims to present a case report of Buschke-Ollendorff syndrome from an orthopedic aspect and emphasize the importance of the disease since it can be easily assessed as a bone tumor. Second, to the best of our knowledge, this is the first case presented with a unilateral genu valgum deformity with a long-term follow-up in the relevant literature.

Keywords: buschke-ollendorff; cortical bone; genetic skin disease; heterotopic ossicication; melorheostosis.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. The orthoroentgenogram of the patient with valgus deformity in the left lower limb.
Figure 2
Figure 2. Reticular skin lesions on the medial surface of the left lower extremity.
Figure 3
Figure 3. The flow of melted wax appearance - melorheostosis - around the left knee on axial and sagittal sections of the CT scan.
Figure 4
Figure 4. Left knee MR sections showing an area of heterotopic ossification containing calcifications extending from the posterior of the femur medial condyle as far as the adjacent joint capsule. Calcified tubular appearances around the left knee are shown on the coronal plane.
Figure 5
Figure 5. Left tibia anterior-posterior and lateral plain radiograph after the surgical intervention. As seen in the plain radiographs, deformity correction is obtained via the circular frame of Ilizarov.
Figure 6
Figure 6. Plain radiographs of the left knee at 3-year follow-up. Although the deformity is corrected, the melorheostosis is still present.
Figure 7
Figure 7. Comparative orthoroentgenograms of the patient. Preoperative and postoperative imaging.
See this image and copyright information in PMC

References

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