Fibroblast Growth Factor-Impregnated Collagen-Gelatin Sponge Improves Keratinocyte Sheet Survival.

Commercially available cultured epithelial keratinocyte sheets (KSs) have played an essential role in wound healing over the past four decades. Despite the initial uptake by the dermal elements, the survival rate of KS on the dermis-like tissue generated by conventional artificial dermis (AD) is low, making this method unsuitable for standard treatments. Therefore, an innovative AD such as collagen-gelatin sponge (CGS) that maintains the release of human recombinant basic fibroblast growth factor (bFGF) may promote wound healing. In this study, we examined whether combination therapy with KSs and CGS with bFGF (bFGF-CGS) could enhance KS survival by heterologous grafting by transplantation of human-derived KSs in an athymic nude rat wound model of staged skin reconstruction. The CGSs were implanted into skin defect wounds on athymic nude rats, which were then divided into two experimental groups: the bFGF group (CGSs containing bFGF, n = 8) and the control group (CGSs with saline, n = 8). Two weeks after implantation, human epithelial cell-derived KSs were grafted onto the dermis-like tissue, followed by assessment of the survival and morphology at 1 week later using digital imaging, histology (hematoxylin and eosin and Masson's trichrome staining), immunohistology (von Willebrand factor), immunohistochemistry (cytokeratin 1-5-6, Ki-67), and immunofluorescence (collagen IV, pan-cytokeratins) analyses. The bFGF group showed a significantly higher KS survival area (86 ± 58 mm2 vs. 32 ± 22 mm2; p < 0.05) and increased epidermal thickness (158 ± 66 µm vs. 86 ± 40 µm; p < 0.05) compared with the control group, along with higher dermis-like tissue regeneration, neovascularization, epidermal maturation, and basement membrane development. These results indicate that the survival rate of KSs in the dermis-like tissue formed by bFGF-CGS was significantly increased. Therefore, combination treatment of bFGF-CGS and KSs shows potential for full-thickness skin defect reconstruction in clinical situations. Impact statement This study highlights how using a combination of cultures, keratinocyte sheets, and collagen-gelatin sponge containing basic fibroblast growth factors can significantly improve cell survival in athymic nude rats with staged skin reconstruction. Our study makes a significant contribution to the literature because it highlights a novel and improved strategy for treating a very common condition such as skin wounds arising from many conditions. Clinical translation of this study may be useful for treating skin wounds.

A Three-dimensionally Printed Acrylonitrile Butadiene Styrene Model for the Reduction of Nasomaxillary Fracture.

The successful reduction of a nasomaxillary fracture was performed using a three-dimensional printed model. A 16-year-old boy was struck in the left orbit by a baseball; subsequently, he was diagnosed with the nasal bone fracture at a hospital, and was referred to the authors' department. A left nasomaxillary fracture and nasal bone fracture were diagnosed by computed tomography. Standard triangulated language data for the mirror image of the frontal process of the right maxilla were obtained from digital imaging and communications in medicine data for preparing a three-dimensional printed acrylonitrile butadiene styrene model. On postinjury day 13, the frontal process fracture was reduced via transconjunctival and intraoral approaches. After the reduction of the fracture, an absorbable plate fitting to the shape of three-dimensional printed acrylonitrile butadiene styrene model was molded, and the maxillary frontal process and infraorbital rim were reduced and fixed with an absorbable plate and screws. Postoperative computed tomography demonstrated a favorable reduction. The intraoperative use of the 3D printed acrylonitrile butadiene styrene model was helpful in the nasomaxillary fracture reduction and fixation.

Facial nerve regeneration with bioabsorbable collagen conduits filled with collagen filaments: An experimental study.

INTRODUCTION: A bioabsorbable collagen conduit (Renerve™) filled with collagen filaments is currently approved as an artificial nerve conduit in Japan and is mainly used for connecting and repairing peripheral nerves after traumatic nerve injury. However, there are few reports on its applications for reconstructing and repairing the facial nerve. The present study evaluated the efficacy of the conduit on promoting nerve regeneration in a murine model with a nerve defect at the buccal branch of the facial nerve. METHODS: Under inhalational anesthesia and microscopic guidance, the buccal branch of the left facial nerve in an 8-week-old Lewis rat was exposed, and a 7 mm gap was created in the nerve. The gap was then connected with either the nerve conduits (NC group) or an autologous nerve graft (the autograft group). At 13 weeks after the procedure, we compared the histological and physiological regenerations in the both groups. RESULTS: We found compound muscle action potential amplitude is significantly larger in the autograft group (2.8 ± 1.4 mV) than in NC group (1.3 ± 0.5 mV) (p < 0.05). The number of myelinated fibers of the autograft group was higher (3634 ± 1645) than that of NC group (1112 ± 490) (p < 0.01). The fiber diameter of the autograft group (4.8 ± 1.9 µm) was larger than that of NC group (3.8 ± 1.4 µm) (p < 0.05). The myelin thickness of the autograft group was thicker than that of NC group (0.6 ± 0.3 µm vs. 0.4 ± 0.1 µm) (p < 0.05). G-ratio of the autograft group (0.74 ± 0.19) was lower than that of NC group (0.79 ± 0.10) (p < 0.05). CONCLUSION: This study demonstrated the efficacy of collagen nerve conduit for facial nerve reconstruction following nerve injury. However, the effectiveness of the conduit on the promotion of nerve regeneration was inferior to that of the autograft.

"Elephant-trunk" negative pressure wound therapy for fixing artificial dermis with basic fibroblast growth factor for critical limb ischemia.

INTRODUCTION: The treatment of intractable toe ulcer with critical limb ischemia (CLI) is a challenge because of its poor blood flow and the wound. Here, a novel fixation technique for artificial dermis with negative pressure wound therapy (NPWT) was reported. METHOD: After the amputation of toe, artificial dermis made of collagen-gelatin sponge (CGS) was grafted onto the wound where human recombinant basic fibroblast growth factor (bFGF) was sprayed. The foot was put on adhesive iodine-impregnated drape, the artificial-dermis area was covered with a sponge dressing of which another end reached to the drape, and the vacuum port was applied on the dressing sponge sandwiched with two drapes and connected to an NPWT system. Since the shape of sponge-dressing was similar to that of elephant-trunk, the technique in this study was named an "Elephant-trunk" technique. RESULT: During NPWT period, no complications such as air leakage, skin erosion, ischemic around tissue were confirmed. The artificial dermis was engrafted completely at one week after surgery, and the wound was confirmed to close completely. CONCLUSION: This NPWT technique with bFGF and CGS accelerated the healing of wound treated conservatively with artificial dermis in CLI patients.

Spare Parts Surgery with a Free Posterior Interosseous Artery Perforator Flap for Thumb Tip.

Replantation is an ideal method for treating fingertip amputation. However, in some cases, replantation is known to be a challenging issue. This report described a successful thumb tip reconstruction performed with bone and nail bed salvaged as spare parts, and a free posterior interosseous artery perforator (PIAP) flap. A 75-year-old man accidentally amputated his left thumb with an electric saw, and emergency replantation was started under brachial plexus block. However, the distal stump of digital artery was unable to be identified, forcing the initial plan to change to flap reconstruction. After vascular anastomosis, complex tissue containing nail bed and side nail fold was grafted on the adipofascial tissue of PIAP flap. Both PIAP flap and the complex tissue survived completely. At 12 months after surgery, only a slight deformity in the nail plate was observed. Spare parts surgery is a surgical procedure effectively salvaging and utilizing tissue that is going to be discarded in severe limb trauma. This idea can be applied to treatment for the finger amputation. In this case, replantation would be difficult in the thumb tip amputation, so spare parts surgery was performed with a PIAP flap. The innervated PIAP flap is reported, including the posterior antebrachial cutaneous nerve. In this case, the cutaneous nerve was able to be identified, neurorrhaphy was performed, and sufficient sensory recovery was obtained. Surgical procedure with PIAP flaps was found to be a useful method for immediate reconstruction with salvaged spare parts after fingertip replantation was considered to be difficult intraoperatively.

Superficial Temporal Artery Pseudoaneurysm following Midface Thread-lift.

Facial thread-lifting has been popular because of its ease and safety with short down time. However, many physicians perform the procedure in cosmetic clinics, which can result in several complications. This report describes the surgical treatment of iatrogenic superficial temporal artery pseudoaneurysm (STAP) following thread-lifting. A 27-year-old man developed a painless, pulsating soft mass in the pre-auricular region after undergoing a thread-lift in a private cosmetic clinic 3 months before being referred to the authors' hospital. The mass was diagnosed as a STAP, using magnetic resonance imaging. The pseudoaneurysm was resected completely, and the superficial temporal artery was microsurgically reconstructed. Although there are some surgical procedures for treating STAP, such as surgical resection and embolization, the former is considered the first choice. Physicians should be trained before performing thread-lifting and must know the possibility of an iatrogenic STAP appearing after the procedure and the face and neck anatomy to prevent complications.

A Case of Traumatic Intractable Leg Ulcer with Lymphorrhea Diagnosed Using ICG Lymphography.

We report a case of traumatic lower extremity ulcers with lymphorrhea in a 37-year-old woman. She presented intractable ulcers without lymphedema for nearly 6 months after a traumatic injury. She was treated conservatively for 6 months at a referral hospital. Unfortunately, during conservative treatment, cellulitis was developed. Even though the inflammation resolved, the ulcer did not heal. We suspected persistent lymphorrhea as the cause of refractory ulcer, and for that reason, we performed indocyanine green lymphography. The lymphatic vessel damage site was identified; also, a collateral lymphatic vessel connected toward the central side was recognized. The lymph fluid drainage site was selectively ligated. The lymphorrhea and ulcers healed 1 month after ligation. Ulcers with lymphorrhea can be persistent and refractory to treatment. Indocyanine green lymphography can be a feasible diagnostic technique. It can identify the site of leakage and is thus useful for treatment. There are fewer reports on the evaluation of lymphorrhea with video presentation. Here, we report a case with video presentation.

Lacrimal Duct Obstruction Caused by Nasomaxillary Fracture: A Retrospective Analysis of Consecutive 12 Patients by Computed Tomographic Dacryocystography.

ABSTRACT: Nasomaxillary fracture is a characteristic mid-facial fracture, and there are no reports showing the fracture damaging the nasolacrimal system (NLS). This report described nasomaxillary fracture cases with NLS damages, which were assessed by computed tomographic dacryocystography (CT-DCG). A retrospective cohort study of nasomaxillary fractures diagnosed by CT was conducted from 2007 to 2015. Twelve patients (mean age: 27.5 years) were found, and their clinical symptoms were as follows: nasal deformity in 10 patients, infra-orbital hypoesthesia in 7, epiphora in 5, and diplopia in one. CT-DCG was performed for 2 patients who complained epiphora, and obstruction was found in 1 patient. All patients underwent open reduction and internal fixation (ORIF), and epiphora in 5 patients was improved. One patient, however, complained epiphora postoperatively, which was supposed to be due to the unsuitable screw insertion. Since nasomaxillary fracture could give NLS obstruction, CT-DCG is useful for diagnosis. Although ORIF is an optimal treatment, attention is needed to avoid the lacrimal canal in screwing on the nasomaxillary buttress.

Prevention of denervated muscle atrophy with accelerated nerve-regeneration by babysitter procedure in rat facial nerve paralysis model.

PURPOSE: The "babysitter" procedure is a reconstruction technique for facial nerve complete paralysis and uses the movement source from the healthy facial nerve with a cross-nerve graft. First, an end-to-side neurorrhaphy is performed between the affected facial nerve trunk and hypoglossal nerve for continuously delivering stimuli to the mimetic muscles for preventing the atrophy of mimetic muscles. Despite favorable clinical results, histological and physiological mechanisms remain unknown. This study attempted to establish a model for the "babysitter" procedure and find its efficacy in rats with facial nerve complete paralysis. MATERIALS AND METHODS: A total of 16 Lewis rats were used and divided into 2 groups; cross nerve graft (n = 8) and babysitter groups (n = 8). The facial nerve trunk was transected in both groups. Babysitter group underwent a two-stage procedure. Cross nerve graft group underwent only the transfer of nerve graft from the healthy side to affected side. The animals were assessed physiologically by compound muscle action potential (CMAP), and the regenerated nerve tissues were evaluated histopathologically at 13 weeks after surgery. RESULTS: Facial nucleus stained with retrograde tracers proved the re-innervation of affected facial muscle by the babysitter procedure. In CMAP, the amplitude of babysitter group was significantly higher than that of the cross-facial nerve graft group (p < .05). Histological examination found a significant difference in myelin g-ratio between two groups (p < .05). CONCLUSION: This study investigated the "babysitter" procedure for rat facial nerve palsy. Babysitter procedure shortened the denervation period without mimic muscle atrophy.

Hydrosurgical Debridement Allows Effective Wound Bed Preparation of Pressure Injuries: A Prospective Case Series.

BACKGROUND: Pressure injuries (PIs) are common in hospitalized patients, with incidence exceeding 50% in high-risk patients. Immobilization causes a prolonged compression of vascular networks in tissues overlying bony prominences, leading to ischemia and ulceration. Traditionally, PIs are treated with a combination of surgical debridement and reconstruction. This approach can be invasive for debilitated patients who cannot tolerate prolonged surgeries and extensive tissue resection. Hydrosurgery uses high-pressure irrigation to low-invasively debride and cleanse wounds; its use has shown positive outcomes in burn and chronic wounds care. Here, we hypothesize that hydrosurgery allows low-invasive yet effective wound bed preparation in truncal PIs. METHODS: We conducted a single-center, prospective, uncontrolled case series. Inclusion criteria for this study were presence of a truncal PI (stage III or IV) and an American Society of Anesthesiologists physical status of ≥2 (no exclusion criteria). Measured outcomes included duration of hydrosurgery, postsurgical local (dehiscence, infection, seroma) or systemic complications in the first 30 days, and PI recurrence rate (6-month follow-up). RESULTS: Seven patients (3 sacral, 2 greater trochanteric, and 2 ischial tuberosity PIs) were enrolled for this study. Average duration of hydrosurgery was 12 minutes (±3.1). No local or systemic complications were observed at a 30-day follow-up (0/7, 0%). All flaps (6/7, 86%) and graft (1/7, 14%) reconstructions successfully survived, and no PI recurrence was reported within a 6-month follow-up (0/7, 0%). CONCLUSIONS: Hydrosurgery seems to allow safe, low-invasive, and effective wound bed preparation in truncal PIs. Larger controlled trials are needed to confirm this preliminary evidence, to guide its broader adoption for improved care of high-risk patients with PIs.

Corrigendum to "Dedifferentiated fat cells in polyglycolic acid-collagen nerve conduits promote rat facial nerve regeneration" [Regen Ther 11 (2019) 240-248].

[This corrects the article DOI: 10.1016/j.reth.2019.08.004.].

Accelerated outgrowth in cross-facial nerve grafts wrapped with adipose-derived stem cell sheets.

In this study, we devised a novel cross-facial nerve grafting (CFNG) procedure using an autologous nerve graft wrapped in an adipose-derived stem cell (ADSC) sheet that was formed on a temperature-responsive dish and examined its therapeutic effect in a rat model of facial palsy. The rat model of facial paralysis was prepared by ligating and transecting the main trunk of the left facial nerve. The sciatic nerve was used for CFNG, connecting the marginal mandibular branch of the left facial nerve and the marginal mandibular branch of the right facial nerve. CFNG alone, CFNG coated with an ADSC suspension, and CFNG wrapped in an ADSC sheet were transplanted in eight rats each, designated the CFNG, suspension, and sheet group, respectively. Nerve regeneration was compared histologically and physiologically. The time to reinnervation, assessed by a facial palsy scoring system, was significantly shorter in the sheet group than in the other two groups. Evoked compound electromyography showed a significantly higher amplitude in the sheet group (4.2 ± 1.3 mV) than in the suspension (1.7 ± 1.2 mV) or CFNG group (1.6 ± 0.8 mV; p < .01). Toluidine blue staining showed that the number of myelinated fibers was significantly higher in the sheet group (2,450 ± 687) than in the suspension (1,645 ± 659) or CFNG group (1,049 ± 307; p < .05). CFNG in combination with ADSC sheets, prepared using temperature-responsive dishes, promoted axonal outgrowth in autologous nerve grafts and reduced the time to reinnervation.

Usefulness of avulsed fingertip skin for reconstruction after digital amputation.

We present four cases of fingertip amputation treated with local flap where the avulsed fingertip skin could be effectively utilized for these donor sites. The avulsed finger skin may be utilized even when replantation is not possible. This approach may serve as a new treatment option after fingertip amputation.

Dedifferentiated fat cells in polyglycolic acid-collagen nerve conduits promote rat facial nerve regeneration.

INTRODUCTION: Polyglycolic acid (PGA) nerve conduits, an artificial biodegradable nerve regeneration-inducing tube currently used in clinical practice, are effective in regenerating peripheral nerves. Dedifferentiated fat (DFAT) cells differentiate into various cells including adipocytes, osteoblasts, chondrocytes, skeletal muscle cells, and myofibroblasts, when cultured in appropriate differentiation-inducing conditioned culture medium. This study made a hybrid artificial nerve conduit by filling a PGA conduit with DFAT cells, applied the conduit to a rat facial nerve defect model, and investigated the facial nerve regenerative ability of the conduit. METHODS: Under inhalational anesthesia, the buccal branch of the facial nerve in Lewis rats was exposed, and a 7-mm nerve defect was created. PGA nerve conduits were filled with DFAT cells, which were prepared from rat subcutaneous adipose tissue with type I collagen as a scaffold, and then grafted into the nerve defect sites in rats with a microscope (DFAT group) (n = 10). In other rats, PGA artificial nerve conduits alone were similarly grafted into the nerve defect sites (the control group) (n = 10). Reinnervation was confirmed at 13 weeks postoperatively by a retrograde tracer, followed by histological and physiological comparative studies. RESULTS: The mean number of myelinated fibers was significantly higher in DFAT group (1605 ± 806.23) than in the control group (543.6 ± 478.66). Myelin thickness was also significantly lager in DFAT group (0.57 ± 0.17 µm) than in the control group.(0.46 ± 0.14 µm). Although no significant difference was found in the amplitude of compound muscle action potential (CMAP) between DFAT group (2.84 ± 2.47 mV) and the control group (0.88 ± 0.56 mV), whisker motion was lager in DFAT group (9.22° ± 0.65°) than in the control group (1.9° ± 0.84°). CONCLUSIONS: DFAT cell-filled PGA conduits were found to promote nerve regeneration in an experimental rat facial nerve defect model.

Keratinocyte sheets prepared with temperature-responsive dishes show enhanced survival after in vivo grafting on acellular dermal matrices in a rat model of staged bi-layered skin reconstruction.

INTRODUCTION: Bi-layered skin reconstruction can be achieved by staged grafting of acellular dermal matrices (ADMs) and cultured epithelial keratinocyte sheets (KSs). Both KSs and ADMs have been used for long; yet, their combined use has shown poor effectiveness. This outcome has been related to the enzymatic treatment used in the preparation of KSs, which impairs their adhesion potential to ADMs and the formation of a basement membrane (BM). Temperature-responsive (TR) culture dishes allow for enzyme-free preparation of KSs with preservation of BMs and intercellular adhesion proteins; yet, their use has not been previously applied to staged bi-layered skin reconstruction. Using an in vivo rat model, we tested the hypothesis that TR cultures enhance KSs survival and BM preservation after sequential grafting on ADMs. METHODS: In nude rats (n = 9/group), a 9-cm [2] full-thickness dorsal skin defect was repaired with a commercial ADM. At 2 weeks after surgery, we grafted the ADM with KSs (circular, 25 mm diameter), prepared from human cells either by enzymatic Dispase treatment (DT control group) or a TR culture dish (TR experimental group). KSs survival and BMs preservation was assessed one week later by digital imaging, histology (hematoxylin & eosin), immunohistochemistry (collagen IV, pancytokeratins) and immunofluorescence (cytokeratin 1-5-6, laminin). RESULTS: The TR group showed a significantly higher KSs survival (120 ± 49 vs. 63 ± 42 mm2; p < 0.05) and epidermal thickness (165 ± 79 vs. 65 ± 54 µm; p < 0.01) compared with the control DT group, as well as higher epidermal maturation (cytokeratin) and a denser laminin and Collagen IV expression in the BMs in vitro and in vivo. CONCLUSION: These findings suggest that KSs prepared with TR culture dishes have significantly enhanced survival when grafted on ADMs; these outcomes could help improve current clinical strategies in wound care by skin reconstruction.

One-stage reconstruction by dual-innervated double muscle flap transplantation with the neural interconnection between the ipsilateral masseter and contralateral facial nerve for reanimating established facial paralysis: A report of 2 cases.

The authors developed a one-stage double-muscle reconstruction technique for facial paralysis using a latissimus dorsi (LD) flap and a serratus anterior (SA) flap, which were dually reinnervated by the contralateral facial nerve (FN) and ipsilateral masseter nerve (MN). The procedure was performed for 61-year-old man 3-years after resection of a malignant tumor and a 24-year-old woman 10-years after temporal fracture with facial paralysis. A double-muscle flap comprising left LD and SA flaps was harvested, a 15-cm thoracodorsal nerve (TN) section was attached to the LD flap, and 5-cm and 1-cm sections of the long thoracic nerve (LTN) were attached to the proximal and distal sides of SA flap. The LD flap and SA flap were sutured along the direction of motion of the zygomaticus major and risorius muscles, respectively. The contralateral FN and ipsilateral MN were interconnected by nerve suturing: the medial branch of TN to the distal end of LTN, the proximal end of LTN to the ipsilateral MN, and the buccal branch of contralateral FN to the main trunk of TN. After surgery, good contraction of the transferred flaps resulted in reanimation of a natural symmetrical smile; no complications were observed during the 12-month follow-up period.

Axonal supercharged interpositional jump-graft with a hybrid artificial nerve conduit containing adipose-derived stem cells in facial nerve paresis rat model.

PURPOSE: Interpositional jump-graft (IPJG) technique with the hypoglossal nerve for supercharging can be applied in a facial nerve paresis case. In IPJG, an autologous nerve is required, and the donor site morbidity is unavoidable. Biodegradable nerve conduits are made from polyglycolic acid (PGA) and used recently without donor site complications after providing autologous grafts. Hybrid artificial nerve conduits with adipose-derived stem cells (ASCs) also attract attention as a nerve-regeneration enhancing agent. This study examined the effect of hybrid artificial nerve conduit on IPJG. MATERIALS AND METHODS: A total of 34 Lewis rats were used and divided into 4 groups by the bridge materials: autograft (n = 8), PGA nerve conduit (n = 8), hybrid PGA nerve conduit with ASCs (n = 8), and the nontreated control groups (n = 8). ASCs were collected from 2 rats and cultured. The animals were assessed physiologically and histopathologically at 13 weeks after surgery. RESULTS: In compound muscle action potential, the amplitude of hybrid PGA group (3,222 ± 1,779 µV) was significantly higher than that of PGA group (1,961 ± 445 µV, P < .05), and no significant difference between hybrid PGA and autograft group. All treated groups showed a myelinated nerve regeneration with double innervation in hypoglossal and facial nerve nuclei for vibrissal muscle. CONCLUSION: This study showed the effectiveness of IPJG with a hybrid PGA conduit especially in physiological examination.

Adipose-derived stem cells and the stromal vascular fraction in polyglycolic acid-collagen nerve conduits promote rat facial nerve regeneration.

Adipose-derived stem cells (ADSCs) and the stromal vascular fraction (SVF) promote nerve regeneration. Biodegradable nerve conduits are used to treat peripheral nerve injuries, but their efficiencies are lower than those of autologous nerve grafts. This study developed biodegradable nerve conduits containing ADSCs and SVF and evaluated their facial nerve regenerating abilities in a rat model with a 7-mm nerve defect. SVF and ADSCs were individually poured into nerve conduits with polyglycolic acid-type I collagen as a scaffold (ADSCs and SVF groups). The conduits were grafted on to the nerve defects. As the control, the defect was bridged with polyglycolic acid-collagen nerve conduits without cells. At 13 weeks, after transplantation, the regenerated nerves were evaluated physiologically and histologically. The compound muscle action potential of the SVF group was significantly higher in amplitude than that of the control group. Electron microscopy showed that the axon diameter of the SVF group was the largest, followed by the ADSC group and control group with significant differences among them. The SVF group had the largest fiber diameter, followed by the ADSC group and control group with significant differences among them. The ADSC group had the highest myelin thickness, followed by the SVF group and control group with significant differences among them. Identical excellent promoting effects on nerve regeneration were observed in both the ADSC and SVF groups. Using SVF in conduits was more practical than using ADSCs because only the enzymatic process was required to prepare SVF, indicating that SVF could be more suitable to induce nerve regeneration.

Multiple pilomatricomas in Kabuki syndrome.

Pilomatricoma is a benign tumor of the hair matrix cell that presents predominantly in childhood. Although pilomatricoma occurs spontaneously, multiple pilomatricomas have been described in association with several inherited syndromes. We report on a 28-year-old man with Kabuki syndrome with three pilomatricomas in his head and thigh. Although several reports describe multiple pilomatricomas associated with Turner syndrome, there are no reports of multiple pilomatricomas combined with Kabuki syndrome. Ectodermal abnormalities such as hair abnormality and hirsutism are symptoms of Kabuki syndrome, and pilomatricomas are frequently associated with the mutations of beta-catenin in hair follicle development. The predisposition of pilomatricomas may be not merely a coincidental finding, but an added association with Kabuki syndrome.