A 23-year-old woman with a history of Kindler syndrome was referred to the department of plastic surgery (Sherbrooke University, Sherbrooke, Quebec) with an ulcerated vegetating tumour in her right hand, which was progressive over a sixmonth period. The patient was from Bosnia and the diagnosis of Kindler syndrome was made at 11 years of age. She had a history of acral blistering in infancy and childhood, progressive photosensitivity and occasional gingivitis. She was treated at 18 years of age for esophageal stenosis and at 22 years of age with labiaplasty for vulva malformation. The patient reported no recent history of asthenia or increased fatigue. On physical examination, she had fragile 'cigarette paper-like' skin with aged appearance, palmoplantar hyperkeratosis and poikiloderma. She also had bilateral syndactylism of the second, third and fourth interdigital spaces. As for the lesion, it covered the radial portion of her right hand palm and it extended to the proximal phalanges of the second and third digits, restricting range of movement of the latter. The lesion measured approximately 8 cm × 6 cm × 3 cm (Figure 1). There was no neurovascular injury. There were verrucous-like lesions on the dorsum of the second and third digits of the same hand.Magnetic resonance imaging showed a polylobulated mass with the following diameters: 8.1 cm craniocaudal, 6.1 cm transverse and 3.1 cm anteroposterior. The epicentre was located at the head of the second and third metacarpals over the palmar aspect of soft tissues. The radial side of the second radius was Kindler syndrome is a rare, autosomal, recessive genodermatosis characterized by trauma-induced acral blisters in infancy and childhood, photosensitivity and progressive poikiloderma. Very few cases in the literature report an association with squamous cell carcinoma, even though it is a very well-known, long-term complication. A case involving a 23-year-old woman with a history of Kindler syndrome who was admitted to the department of plastic surgery (Sherbrooke University, Sherbrooke, Quebec) with an extensive ulcerated squamous cell carcinoma of the right hand is presented. A local excision of the tumour was initially performed, but positive margins and clinically palpable axillary lymphadenopathy over the course of hospitalization necessitated below-elbow amputation and lymph node dissection. To the authors' knowledge, this is the second reported case of aggressive metastatic squamous cell carcinoma of the hand in a patient with Kindler syndrome.
A 23-year-old woman with a history of Kindler syndrome was referred to the department of plastic surgery (Sherbrooke University, Sherbrooke, Quebec) with an ulcerated vegetating tumour in her right hand, which was progressive over a sixmonth period. The patient was from Bosnia and the diagnosis of Kindler syndrome was made at 11 years of age. She had a history of acral blistering in infancy and childhood, progressive photosensitivity and occasional gingivitis. She was treated at 18 years of age for esophageal stenosis and at 22 years of age with labiaplasty for vulva malformation. The patient reported no recent history of asthenia or increased fatigue. On physical examination, she had fragile 'cigarette paper-like' skin with aged appearance, palmoplantar hyperkeratosis and poikiloderma. She also had bilateral syndactylism of the second, third and fourth interdigital spaces. As for the lesion, it covered the radial portion of her right hand palm and it extended to the proximal phalanges of the second and third digits, restricting range of movement of the latter. The lesion measured approximately 8 cm × 6 cm × 3 cm (Figure 1). There was no neurovascular injury. There were verrucous-like lesions on the dorsum of the second and third digits of the same hand.Magnetic resonance imaging showed a polylobulated mass with the following diameters: 8.1 cm craniocaudal, 6.1 cm transverse and 3.1 cm anteroposterior. The epicentre was located at the head of the second and third metacarpals over the palmar aspect of soft tissues. The radial side of the second radius was Kindler syndrome is a rare, autosomal, recessive genodermatosis characterized by trauma-induced acral blisters in infancy and childhood, photosensitivity and progressive poikiloderma. Very few cases in the literature report an association with squamous cell carcinoma, even though it is a very well-known, long-term complication. A case involving a 23-year-old woman with a history of Kindler syndrome who was admitted to the department of plastic surgery (Sherbrooke University, Sherbrooke, Quebec) with an extensive ulcerated squamous cell carcinoma of the right hand is presented. A local excision of the tumour was initially performed, but positive margins and clinically palpable axillary lymphadenopathy over the course of hospitalization necessitated below-elbow amputation and lymph node dissection. To the authors' knowledge, this is the second reported case of aggressive metastatic squamous cell carcinoma of the hand in a patient with Kindler syndrome.
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