Clinical, Dermoscopic, and Histological Characteristics of Laugier-Hunziker Syndrome / 대한피부과학회지

Background@#Laugier-Hunziker syndrome (LHS) is a rare, benign, acquired disorder that is usually characterized by hyperpigmentation of the oral cavity and lips, along with longitudinal melanonychia. Till date, its characteristics have not been investigated in a large number of patients in a single-center study. @*Objective@#This study aimed to investigate the clinical, dermoscopic, and histological characteristics of LHS. @*Methods@#We retrospectively reviewed the medical records, biopsy specimens, and clinical and dermoscopic photographs of 21 patients diagnosed with LHS at Pusan National University Hospital (Busan and Yangsan) over a period of 15 years (2008∼2022). @*Results@#Among the 21 patients, 20 patients (95.2%) were female and one patient (4.8%) was male, and the mean age was 56.0 years (range: 18∼75 years). The lower lip (95.2%) was the most frequently affected oral mucosa, followed by the upper lip (81.0%), buccal mucosa (66.7%), tongue (57.1%), gingiva (42.9%), and palate (14.3%). The nails were the most commonly affected extraoral site, and in our study, eight of 21 patients (38.1%) presented with periungual lesions. Based on the dermoscopic findings, a globular pattern was the most common. Histologically, the lesions showed epithelial acanthosis and increased basal keratinocyte melanin without an increase in the number of melanocytes. @*Conclusion@#This is the first study to investigate the clinical, dermoscopic, and histological characteristics of patients with LHS. The results of this study could be helpful in understanding LHS.

Congenital Smooth Muscle Hamartoma Characterized by Hypopigmented Appearance / 대한피부과학회지

Congenital smooth muscle hamartoma is a benign proliferation of smooth muscles within the dermis. The classic form presents as well-defined, skin-colored, or hyperpigmented plaques associated with hypertrichosis. However, there have been reports of atypical forms, including a follicular spotted appearance, linear atrophic plaques, and morphea-like forms. In such cases, distinguishing congenital smooth muscle hamartomas from other cutaneous diseases can be challenging. Herein, we report on a 16-month-old boy who presented with a hypopigmented patch and hypertrichosis on his back since birth. Histopathological examination revealed mild acanthosis and well-defined smooth muscle bundles haphazardly oriented in the dermis. These bundles stained positively with Masson’s trichrome stain. Based on these findings, a definitive diagnosis of congenital smooth muscle hamartoma was established. In conclusion, an exceptionally rare case of congenital smooth muscle hamartoma with a hypopigmented appearance is reported.

Effectiveness and Safety of Low-Dose Oral Minoxidil in Female Pattern Hair Loss:A Single-Center Study of 44 Patients / 대한피부과학회지

Background@#Female-pattern hair loss (FPHL) is characterized by diffuse hair thinning in the mid-frontal scalp and increased hair shedding. Although the use of low-dose oral minoxidil (LDOM) is increasing significantly in Korea, data on its use are limited. @*Objective@#To determine the efficacy and safety of LDOM in Korean patients with FPHL. @*Methods@#This retrospective, single-center study was conducted at Pusan National University Hospital. The study included female patients with pattern hair loss who received treatment with LDOM at 1.25 mg/d for a minimum of 6 months in combination with other treatments. Patients were eligible for LDOM addition if their previous treatments showed a limited response and the previous treatment regimen remained unchanged throughout the evaluation period. Clinical response to treatment was evaluated using the Sinclair hair loss severity scale. @*Results@#The study included 44 females with FPHL. Before treatment, the mean Sinclair scale score was 2.77, which decreased to 2.27 after treatment. By the 3rd month, one patient (2.3%) experienced worsening, while seven patients (15.9%) showed slight improvement. By the 6th month, the treatment response was as follows: stabilization in 30 patients (68.2%), slight improvement in 13 patients (29.5%), and substantial improvement in one patient (2.3%). Adverse effects, predominantly hypertrichosis, were observed in nine patients. All adverse effects improved upon discontinuation of LDOM and no life-threatening adverse effects were observed during the study. @*Conclusion@#This study provides evidence that LDOM can be an effective therapeutic option with a good safety profile for FPHL.

Clinical Findings and Severity of Periorificial Dermatitis:Comparison between Children and Adults / 대한피부과학회지

Background@#Periorificial dermatitis (POD) is an acneiform or rosacea-like eruption that occurs in children and adults. Although POD is not rare, studies investigating its clinical characteristics and severity, particularly during childhood, have not been well conducted. @*Objective@#This study aimed to investigate the clinical findings and severity of POD and the differences between childhood and adult POD. @*Methods@#We retrospectively reviewed the medical records and clinical photographs of 131 patients diagnosed with POD in the Pusan National University Hospital and Pusan National University Yangsan Hospital over a 17-year period (2003∼2019). @*Results@#Among 131 patients, 23 (17.6%) were children and 108 (82.4%) were adults. The mean age was 9.0 years (2.4∼17 years) and 43.9 years (19∼79 years), respectively. The male-to-female ratio was lower in adults with POD (1:2.6) than in children with POD (1:0.9). The involvement of the periocular area was more frequent in childhood POD (47.8%) than in adult POD (17.6%), although the involvement rates of the perinasal, perioral, and extrafacial areas were not different. When we checked the PeriOral Dermatitis Severity Index (PODSI), it was higher in adults (4.3±1.5) than in children (3.2±2.0). The clinical course according to age, sex, treatment, and severity did not differ between the groups. @*Conclusion@#Although the sample size of childhood POD was small, this study identified that the periocular area was more frequently involved and PODSI was lower in childhood POD than in adult POD.

Prescription Patterns of Topical Medications in Patients with Atopic Dermatitis: An Investigative Review Using Big Data from the National Health Insurance Corporation

Background@#Topical medications play a crucial role in the treatment of atopic dermatitis (AD). Topical corticosteroids (TCSs) remain the main treatment of choice and topical antibiotics have also been used. However, with the new topical calcineurin inhibitors (TCIs), the prescription patterns of topical agents have changed over time. @*Objective@#To characterize the prescription patterns of topical medications in Korean patients with AD. @*Methods@#We investigated topical medications prescribed to Korean patients with AD using the National Health Insurance Sharing System (NHISS) database over a 14-year period (2002~2015). Additionally, the potency of prescribed TCSs was compared with AD and psoriasis patients. @*Results@#The annual prescription of TCSs showed a slightly decreasing trend without significant change. In particular, in terms of steroid class, prescription of moderate-to-low potency TCSs were increased and the use of high potency TCSs were decreased. TCSs were the most commonly prescribed topical medications for AD. Tertiary hospitals had a higher prescription rate for TCIs than secondary or primary hospitals (16.2%, 3.1%, and 1.9%, respectively). Additionally, dermatologists prescribed TCIs more frequently than pediatricians and internists (4.3%, 1.2%, and 0.6%, respectively). Among TCSs, Class 5 was prescribed the most (40.6%) followed by Class 7, 6, 4, 3, 1, and 2. When we compared the potency of TCSs prescribed for AD with psoriasis patients, moderate-to-low-potency TCSs were more commonly prescribed in AD. @*Conclusion@#Prescription patterns of topical medications had changed from 2002 to 2015 and differed according to the type of institution and specialty of the physician.

A Case of Variant Turner Syndrome with Multiple Café-au-lait Spots / 대한피부과학회지

The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.

Change in the Usage of Complementary and Alternative Medicine in the Era of Biologics Among Korean Patients With Psoriasis

Background@#Patients with long-standing psoriasis who are not treated with conventional medicine seek complementary and alternative medicine (CAM). The biological revolution in the field of psoriasis since the late 2000s has progressed, expecting clearance or almost clearance of the disease. The frequency and type of CAM usage may have changed after these advances. We aimed to investigate changes in CAM use in Korean patients with psoriasis before and after the prevalent use of biologics. @*Methods@#Patients with psoriasis who visited Pusan National University Hospitals (Busan and Yangsan) between March 2020 and June 2022 were made to complete a face-to-face structured questionnaire. These results were compared with our previous study conducted approximately 10 years ago. @*Results@#In total, 207 patients were included. Compared with the previous results, the frequency of CAM use (67.6%) increased (P < 0.001). Oriental medicine (67.1%) has most commonly been used, followed by health supplements and bath therapy. The biggest reason for using CAM was “to try all the potential treatments.” Meanwhile, negative concerns about conventional medicine (13.5%) significantly decreased during the 10-year period (P < 0.001). @*Conclusion@#Although treatment efficacy has increased with biologics development, CAM usage remains prevalent among Korean patients with psoriasis. Therefore, dermatologists need more efforts to improve patients’ understanding of conventional medicine, including biologics.

Clinical Efficacy and Safety of Dupilumab for the Treatment of Moderate to Severe Atopic Dermatitis in Korea: A Real-World Data in a Single Medical Center / 대한피부과학회지

Background@#Dupilumab is a human monoclonal antibody against interleukin-4 receptor α. Several clinical trials have demonstrated the rapid and excellent therapeutic effects of dupilumab. Although a growing number of studies have reported data on the real-world efficacy and safety of dupilumab for the treatment of atopic dermatitis, data on real-world experiences in Korea are limited. @*Objective@#To evaluate the real-world efficacy and safety of dupilumab for the treatment of moderate-to-severe atopic dermatitis in Korean patients. @*Methods@#This was a retrospective, single-center study. A total of 179 patients treated with dupilumab for at least 16 weeks were enrolled in this study. Based on electronic medical records, the clinical characteristics, Eczema Area and Severity Index (EASI) score, and adverse events were investigated. @*Results@#The mean baseline EASI score (26.5±7.2) significantly decreased at weeks 16, 40, 52, and 112 (p＜0.05). All and 55.2% of patients achieved EASI75 and EASI90 responses at week 52, and all and 75.0% of patients achieved EASI75 and EASI90 responses at week 112, respectively. Common adverse events were facial erythema (31.8%), conjunctivitis (24.0%), and herpes simplex virus infection (11.2%). Three serious adverse events of severe conjunctivitis, mycosis fungoides, and mesenteric venous thrombosis resulted in discontinuation of dupilumab. @*Conclusion@#Dupilumab was effective in real-world clinical practice with a favorable safety profile in Korean patients with moderate-to-severe atopic dermatitis.

A Case of Hystrix-Like Ichthyosis and Deafness Syndrome with a Rare Variant of Gap Junction Protein Beta 2 Gene Mutation / 대한피부과학회지

Hystrix-like ichthyosis and deafness (HID) syndrome is a rare ectodermal dysplasia characterized by erythrokeratoderma and hearing impairments. HID syndrome is a nonocular variant of keratitis ichthyosis deafness (KID) syndrome caused by an autosomal dominant mutation in the gap junction protein β 2 (GJB2) gene. The GJB2 gene encodes connexin 26, a transmembrane protein involved in cell–cell attachment in almost all tissues. We report a case of a 25-year-old man with generalized hyperkeratotic plaques, diffuse palmoplantar keratoderma, and nail deformities since birth. The patient also had a history of recurrent bacterial skin infections in the existing hyperkeratotic lesions. Histopathological examination revealed compact hyperkeratosis and irregular acanthosis in the epidermis, along with upper dermal lymphocytic infiltration. Audiometry revealed high-frequency sensorineural hearing loss. Genetic analysis revealed a missense mutation in the GJB2 gene. Based on clinicopathological findings and genetic testing, HID syndrome was diagnosed.

A Case of Weathering Nodules of the Ear / 대한피부과학회지

Weathering nodules are benign skin conditions of the ear typically occurring in patients with a history of persistent sun exposure. Clinically, they appear as multiple localized, skin-colored to whitish papules or nodules in the ear helix. Histopathologically, weathering nodules are characterized by chondroid metaplasia with spurs of fibrous tissue extending upward from the disrupted perichondrium of the underlying pinna cartilage. We report the case of a 19-year-old man who presented with multiple localized whitish papules on the right ear helix for a month. The lesions were asymptomatic and accompanied by a blanch sign. Histopathological examination revealed chondroid metaplasia in the dermis, separate from the pinna cartilage. Based on the clinicopathological findings, weathering nodules of the ear was diagnosed.

Clinical Efficacy and Safety of Risankizumab for the Treatment of Moderate to Severe Psoriasis in Korea / 대한피부과학회지

Background@#Risankizumab, human immunoglobulin G1λ monoclonal antibody, selectively blocks the p19 subunit of interleukin-23-mediated signaling pathway. Risankizumab has emerged as an effective and well-tolerated therapeutic option for moderate-to-severe psoriasis. However, real-world data on treatment outcomes of risankizumab in Korean patients with psoriasis are limited. @*Objective@#To investigate the efficacy and safety of risankizumab in Korean patients with moderate-to-severe plaque psoriasis. @*Methods@#We retrospectively reviewed patients with moderate-to-severe psoriasis who were treated with risankizumab in our hospital from July 2020 to December 2022. A total of 45 patients with psoriasis who were treated with risankizumab for at least 16 weeks were included in this study. Patient demographics, Psoriasis Area and Severity Index (PASI) scores, Investigator’s Global Assessment scores, and adverse events were assessed. @*Results@#The mean baseline PASI score (13.1±7.7) was significantly decreased after 16 weeks of risankizumab treatment (0.9±1.4) (p＜0.05). Of the 45 patients, 40 showed a PASI 90 response at the last follow-up visit. No serious adverse events were observed. Three patients (6.7%) experienced mild adverse events such as injection site reaction, easy bruising, and headache. @*Conclusion@#Our real-world data demonstrated that risankizumab treatment is effective and safe in Korean patients with moderate-to-severe plaque psoriasis.

A Case of Plaque Psoriasis Associated with Myasthenia Gravis and Graves’ Disease / 대한피부과학회지

Psoriasis is a common immune-mediated skin disease. Myasthenia gravis and Graves’ disease are autoimmune diseases caused by autoantibodies against receptors located at the neuromuscular junction and thyroid-stimulating hormone receptor, respectively. A 29-year-old male presented with recurrent multiple erythematous scaly papules and plaques on his legs that had persisted for 3 years. A diagnosis of plaque psoriasis was made based on clinicopathological findings. The patient had a 26-year history of ocular myasthenia gravis, for which he had not been received any specific treatment. He also had a long history of Graves’ disease. To the best of our knowledge, psoriasis with myasthenia gravis and Graves’ disease has not been reported previously in the literature. Herein, we describe our examination of the causes of concurrent psoriasis, myasthenia gravis, and Graves’ disease.

A Case of Acanthosis Nigricans with Hypochondroplasia due to FGFR3 Gene (p.Lys650Thr) Mutation / 대한피부과학회지

Acanthosis nigricans (AN) is characterized by velvety hyperpigmented plaques, usually observed in skin folds. The different types of the condition include obesity-associated, syndromic, drug-induced, malignancy-related, and other types of AN. AN, an FGFR3 gene mutation-related disease, is associated with varying degrees of skeletal disorders. FGFR3 gene mutations are known to cause excessive inhibition of chondrocyte growth and keratinocyte proliferation, which is responsible for AN development. To our knowledge, only a small number of cases of AN with hypochondroplasia due to FGFR3 gene mutation (p.Lys650Thr) have been described in the literature. However, there are no reports of genetically confirmed AN with hypochondroplasia in Korea. Physicians should consider syndromic AN when symptoms develop at an early age or when associated skeletal anomalies are present.

Two Cases of Mycosis Fungoides Diagnosed during Dupilumab Treatment / 대한피부과학회지

Dupilumab, a human monoclonal antibody targeting the interleukin-4 receptor α, has exhibited rapid and remarkable therapeutic efficacy in numerous clinical trials focusing on moderate-to-severe atopic dermatitis and prurigo nodularis. Nonetheless, instances of mycosis fungoides have been reported in patients undergoing dupilumab treatment for atopic dermatitis. We present two cases: a 36-year-old woman and a 64-year-old man who presented to our dermatology clinic with erythematous papules and plaques. Following skin biopsy, both patients were diagnosed with chronic eczematous dermatitis with prurigo. Erythematous plaques notably increased after 9 months and 1 month of dupilumab treatment, respectively. Subsequent biopsies confirmed histopathological markers consistent with mycosis fungoides. The first patient underwent chemotherapy for lymph node metastasis, while the second received oral acitretin, narrow-band ultraviolet B, and topical corticosteroids.

Pigmented Fungiform Papillae of the Tongue: A Single-center Experience and Review of Literature

Background@#Pigmented fungiform papillae of the tongue (PFPT) is a rare benign pigmentary disorder of the tongue. In dark-skinned individuals, PFPT appears to be relatively common. However, limited data exist on PFPT in Korean patients. @*Objective@#We aimed to investigate the clinical characteristics of PFPT in Korean patients. @*Methods@#Patients diagnosed with PFPT between 1995 and 2021 at the Pusan National University Hospital were included. Clinical characteristics of PFPT, dermoscopic findings, and comorbidities were reviewed. @*Results@#A total of 19 patients diagnosed with PFPT were enrolled. The male to female ratio was approximately 1:5. The mean age at diagnosis was 41.1 years (range, 8~67 years). According to Holzwanger’s classification, Type I was the most common (89.5%). PFPT was commonly concomitant with pigmentary disorders, including mucosal melanotic macules, Laugier-Hunziker syndrome, melasma, and melanonychia (6/19, 31.6%). Preceding oral infection or inflammatory lesions were found in four patients (21.1%), and systemic diseases and infectious diseases existed in two patients (10.5%). Dermoscopic examination was performed in seven patients; pigmented border with dichotomized vessels (rose petal pattern, 71.4%) and diffuse pigmentation (cobblestone pattern, 71.4%) were common findings. @*Conclusion@#Our study shows PFPT can coexist with pigmentary disorders. Concomitant pigmentary disorder shows an association with sex hormone or susceptibility to abnormal pigmentation may be a possible cause of PFPT.

Multiple Cutaneous Rosai-Dorfman Disease on the Face Treated by Electrosurgery / 대한피부과학회지

Cutaneous Rosai-Dorfman disease (RDD) is a rare, benign condition mainly affecting the lymph nodes and sometimes involving the skin. RDD is notorious for its poor response to medication, and surgery is often the preferred treatment option for limited lesions. Herein, we present a case of cutaneous RDD in a 49-year-old woman with multiple facial nodules that showed minimal response to treatment with steroids, isotretinoin, and methotrexate.The diagnosis of cutaneous RDD was confirmed based on histopathological examination. The patient showed minimal response to medication; however, electrosurgery resulted in a relatively good cosmetic response. The present case demonstrates that electrosurgery can be an effective treatment option for cutaneous RDD, especially in case of patients with multiple lesions. The rarity of this disease and the limited reports on its management highlights the need for further research on the efficacy of different surgical modalities for cutaneous RDD.

Differences in Dysphagia between Patients with Stroke or Amyotrophic Lateral Sclerosis / 대한연하장애학회지

Objective@#Dysphagia is a common symptom of stroke and affects 23–50% of such patients. In addition, bulbar involvement, which causes dysphagia, is the primary initial symptom in approximately 25–30% of amyotrophic lateral sclerosis (ALS) patients. The purpose of this study was to compare patterns of swallowing difficulties in stroke and ALS patients. @*Methods@#We retrospectively recruited 84 ALS patients with dysphagia and 294 stroke patients with dysphagia between January 2017 and December 2019. Swallowing processes were reviewed by videofluoroscopic swallowing studies (VFSSs). The presence of oral residues and oral transit times (OTTs) were measured in the oral phase, and the presence of penetration and aspiration and residues in valleculae or pyriform sinuses were evaluated. Statistical analysis was performed using SPSS 25.0 and comparisons using the Chi-square test. @*Results@#ALS patients more frequently had delayed OTTs and oral residues than stroke patients, and stroke patients more frequently experienced aspiration and had delayed thin liquid pharyngeal transit times (PTTs). However, no significant intergroup difference was observed for the presence of penetration, residues in valleculae or pyriform sinuses, or thick liquid PTTs. @*Conclusion@#The study shows that ALS patients exhibit slower food processing in the oral cavity and more significant bulbar muscle weakness than stroke patients. On the other hand, stroke patients had greater thin liquid aspiration rates than ALS patients. These findings should be considered when choosing treatments for ALS and stroke.

Clinical Practice Guideline for Postoperative Rehabilitation in Older Patients With Hip Fractures

Objective@#The incidence of hip fractures is increasing worldwide with the aging population, causing a challenge to healthcare systems due to the associated morbidities and high risk of mortality. After hip fractures in frail geriatric patients, existing comorbidities worsen and new complications are prone to occur. Comprehensive rehabilitation is essential for promoting physical function recovery and minimizing complications, which can be achieved through a multidisciplinary approach. Recommendations are required to assist healthcare providers in making decisions on rehabilitation post-surgery. Clinical practice guidelines regarding rehabilitation (physical and occupational therapies) and management of comorbidities/complications in the postoperative phase of hip fractures have not been developed. This guideline aimed to provide evidence-based recommendations for various treatment items required for proper recovery after hip fracture surgeries. Methods Reflecting the complex perspectives associated with rehabilitation post-hip surgeries, 15 key questions (KQs) reflecting the complex perspectives associated with post-hip surgery rehabilitation were categorized into four areas: multidisciplinary, rehabilitation, community-care, and comorbidities/complications. Relevant literature from four databases (PubMed, EMBASE, Cochrane Library, and KoreaMed) was searched for articles published up to February 2020. The evidence level and recommended grade were determined according to the grade of recommendation assessment, development, and evaluation method. Results A multidisciplinary approach, progressive resistance exercises, and balance training are strongly recommended. Early ambulation, weigh-bearing exercises, activities of daily living training, community-level rehabilitation, management of comorbidities/complication prevention, and nutritional support were also suggested. This multidisciplinary approach reduced the total healthcare cost. @*Conclusion@#This guideline presents comprehensive recommendations for the rehabilitation of adult patients after hip fracture surgery.

Clinical Practice Guideline for Postoperative Rehabilitation in Older Patients With Hip Fractures

Objective@#The incidence of hip fractures is increasing worldwide with the aging population, causing a challenge to healthcare systems due to the associated morbidities and high risk of mortality. After hip fractures in frail geriatric patients, existing comorbidities worsen and new complications are prone to occur. Comprehensive rehabilitation is essential for promoting physical function recovery and minimizing complications, which can be achieved through a multidisciplinary approach. Recommendations are required to assist healthcare providers in making decisions on rehabilitation post-surgery. Clinical practice guidelines regarding rehabilitation (physical and occupational therapies) and management of comorbidities/complications in the postoperative phase of hip fractures have not been developed. This guideline aimed to provide evidence-based recommendations for various treatment items required for proper recovery after hip fracture surgeries. Methods Reflecting the complex perspectives associated with rehabilitation post-hip surgeries, 15 key questions (KQs) reflecting the complex perspectives associated with post-hip surgery rehabilitation were categorized into four areas: multidisciplinary, rehabilitation, community-care, and comorbidities/complications. Relevant literature from four databases (PubMed, EMBASE, Cochrane Library, and KoreaMed) was searched for articles published up to February 2020. The evidence level and recommended grade were determined according to the grade of recommendation assessment, development, and evaluation method. Results A multidisciplinary approach, progressive resistance exercises, and balance training are strongly recommended. Early ambulation, weigh-bearing exercises, activities of daily living training, community-level rehabilitation, management of comorbidities/complication prevention, and nutritional support were also suggested. This multidisciplinary approach reduced the total healthcare cost. @*Conclusion@#This guideline presents comprehensive recommendations for the rehabilitation of adult patients after hip fracture surgery.

Post-Traumatic Guillain-Barre Syndrome / 대한신경근육질환학회지

Guillain-Barre syndrome (GBS) is acute inflammatory demyelinating polyradiculoneuropathy, which is often related to post-infectious etiology. However, GBS has also been reported to be caused by non-infectious factors such as trauma. This report describes a rare case of post-traumatic GBS with dramatic response to immunoglobulin therapy. And here, we also discussed about the importance of differential diagnosis with critical illness polyneuropathy.