BACKGROUND: Striated palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is a very rare, focal, nonepidermolytic palmoplantar keratoderma with autosomal inheritance. Unlike other palmoplantar keratodermas, no association with visceral or skin cancer has ever been reported. OBJECTIVE: We report a case of malignant melanoma arising in the hyperkeratotic lesions on the right heel of a patient with striated palmoplantar keratoderma. The lesion was completely excised; our patient also underwent sentinel lymph node biopsy and then was treated with high-dose interferon adjuvant therapy. METHODS: Sentinel lymph node biopsy incision was made in elliptical fashion, long enough to harvest a full-thickness skin graft to cover the wide local excision defect. The skin graft was defatted by sharp dissection. Several perforations were made in graft and it was secured in place with sutures and bolster dressing. RESULTS: At follow-up, the grafted skin showed hyperkeratotic changes but no local or systemic signs of the disease was observed. CONCLUSION: The association between striated palmoplantar keratoderma and acral melanoma is discussed.

Rubegni, P., Poggiali, S., Cuccia, A., Biagioli, M., & Fimiani, M. (2004). Acral malignant melanoma and striated palmoplantar keratoderma (Brunauer-Fohs-Siemens syndrome): a fortuitous association?. DERMATOLOGIC SURGERY, 30, 1539-1542.

Acral malignant melanoma and striated palmoplantar keratoderma (Brunauer-Fohs-Siemens syndrome): a fortuitous association?

RUBEGNI, PIETRO;FIMIANI, MICHELE
2004

Abstract

BACKGROUND: Striated palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is a very rare, focal, nonepidermolytic palmoplantar keratoderma with autosomal inheritance. Unlike other palmoplantar keratodermas, no association with visceral or skin cancer has ever been reported. OBJECTIVE: We report a case of malignant melanoma arising in the hyperkeratotic lesions on the right heel of a patient with striated palmoplantar keratoderma. The lesion was completely excised; our patient also underwent sentinel lymph node biopsy and then was treated with high-dose interferon adjuvant therapy. METHODS: Sentinel lymph node biopsy incision was made in elliptical fashion, long enough to harvest a full-thickness skin graft to cover the wide local excision defect. The skin graft was defatted by sharp dissection. Several perforations were made in graft and it was secured in place with sutures and bolster dressing. RESULTS: At follow-up, the grafted skin showed hyperkeratotic changes but no local or systemic signs of the disease was observed. CONCLUSION: The association between striated palmoplantar keratoderma and acral melanoma is discussed.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11365/29424
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