252 Akademie DOI: 10.1111/j.1610-0387.2010.07579.x

JDDG | 3˙2011 (Band 9) © The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin • JDDG • 1610-0379/2011/0903

RedaktionProf. Dr. Rainer Rompel,

Kassel

IntroductionKeratosis palmoplantaris papulosa(Buschke-Fischer-Brauer disease) [1–4]is a form of keratosis that is inherited in anautosomal dominant pattern and mani-fests only on the palms of the hands andsoles of the feet. Onset occurs betweenages 20 and 40 years. The gene locus ison chromosome 15q22.2–15q22.31 [2].The disease has a progressive course, ini-tially presenting with solitary papulesand later with thickening of the skin thatcan impair or even prevent walking.Therapy options include chemical ormechanical keratolysis as well as systemicacitretin [5–6]. The effects of these therapyforms are only transient, however. Thereare reports in the literature of surgicalapproaches consisting of excision andskin grafting [7–10]. In September 2005we performed skin transplantation onthe plantar aspects of both feet of onepatient in combination with vacuum-assisted closure. The results of the proce-dure and 3-year follow-up observationare presented in the following.

Patient and methodsA 44-year-old man came to our clinic inearly September 2005 with progressivehyperkeratotic lesions that had beenpresent for 15 years on the soles of bothfeet (Figures 1 and 2). The lesions wereso severe that there was restricted mobil-ity. There was also considerable pain dueto a bacterial superinfection with oozingof pus from infected areas. There was noknown family history of disorders ofkeratinization.

Surgical treatment of severe palmoplantarkeratodermaStefan Rapprich, Manfred HagedornDepartment of Dermatology, Darmstadt Hospital, Germany

Dermatosurgery – Tricks of the Trade

Figure 1: 44-years-old patient with severe plantar keratoderma and minor findings palmar.

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long vacuum therapy, there was completeacceptance of the graft (Figure 8).The patient was gradually mobilized andwas provided with suitable footwear toaccommodate the dressing.

ResultsThree months after the first operation,the soles of the feet had completelyhealed and the patient was able to walkfreely with no pain. Sensation was nearlycompletely restored in the soles of bothfeet. At the split-skin graft harvest sitesthere was complete re-growth of the hairwith all but invisible scarring (Figure 9).At follow-up, nearly 4 years after surgery,there were no new keratotic lesions, thepatient continued to be able to walk nor-mally, and there was normal sensationon the soles of the feet (Figure 10 a–d).

DiscussionSurgical treatment of plantar keratosiswas first described in 1953 by Wynn-Williams. Taking a split-skin graft fromthe scalp is a tried and tested method. In the patient presented here, one innova-tion to the procedure is the successful useof a vacuum dressing following surgery.Given that the patient has been freefrom recurrence for 4 years, it may bepresumed that the gene defect does not

The patient had undergone prior treat-ment with curettage and laser ablation in2003 and 2004, but these attempts wereultimately unsuccessful. There was evendiscussion of amputation of the feet as alast resort.At admission, the patient had a highlypainful, severe superinfected cleat-likeverrucous elevations on the plantar surfaces of both feet with involvement ofthe toes and disfigurement of the contour of the foot. Walking was impos-sible. Solitary areas of hyperkeratosiswere noted on the palms of both hands,but these did not disturb the patient(Figure 1).There were no signs of cancer on labo -ratory tests, abdominal ultrasound, orchest x-ray.On the basis of the clinical picture anddisease course, we diagnosed keratosis

palmoplantaris papulosa (Buschke-Fischer-Brauer disease).We performed a complete radical excisionof the lesions down to the subcutis withcirculatory arrest under general anesthesia(Figures 3 and 4). The procedure was per-formed first on the plantar surface of theleft foot and one week later on the rightside. Wound base conditioning was witha vacuum dressing (Figure 5). Two weekslater a split-skin transplant from the scalpwas performed (Figures 6, 7 and 8).Vacuum therapy for wound base condi-tioning was performed with a blacksponge at 125 mmHg negative pressureand after split-skin transplant with a whitesponge at 75 mmHg negative pressure.Three weeks after regeneration of thescalp, the split-skin transplant was per-formed on the right foot with adminis-tration of general anesthesia. After 5-day-

Figure 2: Detail of severe plantar keratoderma.

Figure 3: Intraoperative situation after radical excision of keratoses.

Figure 4: Wound granulation within 5 days byvacuum assisted therapy.

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quick and safe wound conditioning.Harvesting a split-skin graft from scalpallows for cosmetically acceptable resultsby avoiding visible scarring. <<<

Correspondence toDr. med. Stefan RapprichKlinikum DarmstadtHeidelberger Landstr. 379D-64297 DarmstadtTel.: 06151-1079554133Fax: 06151-1074150E-mail: stefan.rapprich@klinikum-darmstadt.de

taken into account. If several split-skingrafts are to be harvested from the scalp,the patient should plan for a regenerationphase of at least 3 weeks.

Conclusions for clinical practiceRadical excision of severe plantar kerato-sis with split-skin coverage is a goodtreatment option for what is often a verydistressing disorder. The use of a vacuumdressing to cover the wound can achieve

manifest in skin grafted from the scalp,but is only expressed in punctate fashionon palmoplantar regions.There are no reports as yet in the literatureof differing responses of various forms ofpalmoplantar keratosis to surgical therapy.Whether to operate on one foot or bothat the same time should be decided on anindividual basis. Factors such as immobi-lization, underlying disease, patient compliance, care at home, should also be

Figure 5: Vacuum therapy after excision at the right feet. Left foot was treated by split-skin graft 2 weeks before.

Figure 6: 3 weeks post split-skin-graft from thescalp another one is taken.

Figure 7: Intraoperative situation after mesh-graft-transplantation.

Figure 8: 5 days after transplantation completeengraftment of the skin by vacuum therapy.

Figure 9: Normal hair growth at donor site 4months postoperative.

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in 14 families with the rare genoderma-tosis keratosis punctata palmoplantarisBuschke-Fischer-Brauer. Eur J Derma-tol 2003; 13: 16–20.

5 Happle R, van de Kerkhof PC, TraupeH. Retinoids in disorders of keratiniza-tion: their use in adults. Dermatologica1987; 175: 107–24.

6 Hesse S, Berbis P, Privat Y. Keratodermiapalmo-plantaris papulosa (Buschke-Fischer’s disease): efficacy of acitretin.Br J Dermatol. 1993; 128: 104–5.

7 Farina R. Plastic surgery for the manage-ment of palmoplantar keratodermia(palmoplantoneoplasty). Aesthetic PlastSurg 1987; 11: 249–53.

8 Rußwurm R, Hagedorn M. Lichen ruber ulcerosus. Hautarzt 1989; 40:233–5.

9 Salamon T, Bogdanov B, Sucur D. Thesurgical treatment of palmo-plantar keratoderma. Br J Dermatol 1969; 81:363–6.

10 Wynn-Williams D. Plantar keratodermiatreated by split-skin grafts. Br J PlastSurg 1953; 6: 123–9.

3 Brauer A. Über eine besondere Formdes hereditären Keratoms (keratodermadisseminatum hereditarium palmare etplantare). Arch Dermatol Syph 1913;114: 211–36.

4 Emmert S, Kuster W, Hennies HC,Zutt M, Haenssle H, Kretschmer L,Hallermann C, Neumann C. 47 patients

References 1 Buschke A, Fischer W. Keratodermia

maculosa disseminata symmetrica palmaris and plantaris. IkonographiaDermatologica 1910; 5: 183–92.

2 Braun-Falco M. Hereditary PalmoplantarKeratodermas. J Dtsch Dermatol Ges2009; 7: 971–984.

Figure 10: (a–d) Clinical findings preoperative and approximately 4 years postoperative. No sign ofrecurrence.