Background Palmoplantar keratoderma is a rare condition characterized by progressive thickening of the skin on the palms and soles of the feet. In severe cases, it can lead to complications such as sepsis and lifelong disabilities. Traditionally, it has been managed medically with emollients, keratolytics, vitamin supplementation, and surgically with debridement, often leading to recurrence. We present this clinical vignette to highlight the management of severe palmoplantar keratoderma through a nuanced approach involving tissue excision down to the subcutaneous level. To our knowledge, based on a PubMed search using 'Keratoderma, Palmoplantar, Diffuse' Mesh and reviewing 265 articles, this approach has not been reported before with 16-year follow-up data.

Methods The patient presented as a five-year-old girl with severe palmoplantar keratoderma, resistant to medical management. She had been wheelchair-bound with repeated hospital admissions for sepsis. She was initially managed with episodic deep debridement, which offered temporary relief followed by rapid recurrence. After obtaining informed consent, the decision was made to excise the keratoderma from her feet, removing the dermis and subcutaneous tissue. After excision, Integra was placed with negative pressure wound therapy (NPWT), followed by a second layer of Integra with NPWT. Final coverage was achieved with a thin split-thickness graft. For the hands, a similar procedure was performed with excision down to the palmar fascia, extending to the subcutaneous tissue of the fingers, which were pinned straight to reduce the possibility of contracture. After negative pressure wound therapy and granulation, a thin split-thickness skin graft was placed.

After 6 months of walking as a middle school student, there was a breakdown of the heel to the plantar fascia, which was managed using a rectus free flap for heel reconstruction, followed by a conventional split-thickness skin graft. The flap subsequently shrank, with no recurrence.

Results After 16 years, the patient is now a college student who walks with stability in her hands and feet. She can take notes, type, and has almost a full range of motion in her fingers. The patient reports that she is able to lead a normal and productive life.

Conclusion Our case report highlights a novel surgical approach that can potentially be used for the treatment of severe palmoplantar keratoderma. It also underscores the challenges of managing this rare and severe condition with conventional treatments and emphasizes the need for innovative surgical solutions for its management.