Jimyung Seo

Cutaneous Serratia marcescens infections in Korea: A retrospective analysis of 13 patients

Serratia marcescens is a Gram‐negative bacillus belonging to the Enterobacteriaceae family. Because of increasing reports of antimicrobial resistance, this bacterium has received considerable attention and has emerged as an important pathogen. In order to reveal clinical and microbiological characteristics of S. marcescens cutaneous infection and to suggest appropriate antibiotic treatment, we retrospectively analyzed 17 strains isolated from wound swabs of Korean patients between November 2005 and March 2014. A total of 13 patients (five men and eight women) were included in our study, with a mean age of 46.3 years (range, 21–82). Based on medical history, seven patients were classified as immunocompromised. Prior predisposing factors for infections were noted in 12 patients, including pre‐existing leg ulcers or dermatitis (5/13), preceding cancer surgeries (2/13), plastic surgeries and filler injection (2/13), traumas (2/13) and medical procedures following cutaneous abscess (1/13). Cutaneous infections showed various clinical presentations, including spontaneous dermal abscess, fingernail change, painful nodules and papular erosions. We found that third‐ and fourth‐generation cephalosporins, gentamicin, levofloxacin and meropenem appeared active against all 17 strains in vitro. Clinically, all patients treated with empirical first‐generation cephalosporin showed treatment resistance, and oral quinolone monotherapy was the most preferred antibiotic regimen without treatment failure, with an average treatment duration of 25 days (range, 14–42). This study demonstrates the various clinical presentations and treatment responses for cutaneous S. marcescens infection. Moreover, we suggest that initial antibiotic coverage should be broad enough to account for multidrug resistance in this rare pathogen.

Intramuscular triamcinolone acetonide: An undervalued option for refractory alopecia areata.

Severe alopecia areata (AA) can have an unpredictable clinical course and become refractory to contact immunotherapy. Novel treatment options include low‐dose interleukin‐2 and Janus kinase inhibitors; however, these treatments are still under investigation. Therefore, we evaluated the efficacy and safety of intramuscular (i.m.) triamcinolone acetonide (TAC) as a rescue therapy for refractory AA. We retrospectively analysed efficacy, adverse effects and relapse rate of i.m. TAC monthly in 27 patients with refractory AA. We defined AA as refractory if the patient showed an unsatisfactory response to both systemic treatment (not i.m. TAC) and the consecutive diphenylcyclopropenone immunotherapy. The initial systemic treatment of other forms of corticosteroids and/or cyclosporin was used to control extensive AA involving more than 25% of the scalp. Administration of i.m. TAC for 3–6 months resulted in a 63.0% response rate, and all patients showed inactive disease after treatment. Final hair regrowth negatively correlated with initial scalp involvement (Spearman r = −0.595, P = 0.001). All patients showed complete recovery of adrenocortical reserve within 3 months after the last injection. Adverse effects of systemic steroid therapy were observed only in female patients (dysmenorrhea and osteoporosis). i.m. TAC may provide a valuable therapeutic option to manage active hair loss and facilitate hair regrowth in refractory AA, especially in male patients.

Clinical significance of serum YKL-40 in Behçet disease.

Serum YKL‐40 is an inflammatory biomarker of endothelial dysfunction and may play a role in the inflammatory process of Behçet disease (BD).

Multiple milia formation induced by dovitinib

Dovitinib is a novel multi‐target tyrosine kinase inhibitor that inhibits vascular endothelial growth factor receptor‐1–3, platelet‐derived growth factor receptor‐β, Fms‐like tyrosine kinase 3, c‐Kit and fibroblast growth factor receptor‐1–3. This compound is currently being assessed clinically for treatment of various malignancies. In phase I and II clinical trials of dovitinib treatment for renal cell carcinoma, 20% of patients experienced cutaneous adverse events, although the specific type of skin rash was not documented. Here, we report two cases of multiple milia formation induced by dovitinib. We believe our cases are the first report mainly showing non‐inflammatory cystic structure.

Serial trichoscopy vs. modified hair pull test for monitoring the disease activity and treatment response of localized alopecia areata

Editor Because localized alopecia areata (AA) can resolve spontaneously, continuing treatment until hair loss stops may be a reasonable approach for managing the disease. Disease activity and progression of AA are readily discernible by the hair pull test (HPT). However, HPT results can vary, depending upon the physician’s skill and patient’s condition. Inui et al. identified characteristic trichoscopic findings of active AA: black dots, exclamation mark hairs and broken hairs. In this study, we evaluated serial trichoscopy vs. modified HPT for monitoring localized AA. A total of 104 patients with AA and localized alopecic patches (<10% scalp area) were treated with triamcinolone acetonide injection monotherapy at Severance Hospital in Seoul, Korea. Only round or oval lesions <4 cm diameter were included. One to three representative lesions from each participant were selected for analysis. To assess therapeutic response, the patients underwent serial assessments at each office visit: 49 underwent modified HPTs and 55 underwent trichoscopic evaluation (DermLite DL3 , 3Gen, San Juan Capistrano, CA, USA). All patients were advised to avoid shampooing 24 h before their examinations. We defined a positive modified HPT as >10% hairs removed after repeated light pulls from four sides of each alopecic patch. Trichoscopic examination assessing characteristic AA features was performed in the centre and along four sides (at the margin) of each alopecic patch. Hair regrowth was evaluated using the Severity of Alopecia Tool (SALT) at every visit. Multiple 0.1-mL injections, 1.0–1.5 cm apart, of triamcinolone acetonide (5 mg/mL) were administered every 3–4 weeks in all alopecic patches during active disease. In the HPT group, active AA was defined as a positive HPT. Treatment was discontinued when the HPT became negative. In the trichoscopy group, active AA was defined as alopecic patches with black dots, exclamation marks or broken hairs. Triamcinolone acetonide was injected selectively into areas containing any of these markers until they disappeared. Response (≥75% regrowth in the affected area) and relapse rates were assessed 3 months after the last treatment. Relapse is defined as having an abnormal increase in hair loss in the treated alopecic patch. A total of 155 AA patches from 104 patients were analysed (Table 1). Trichoscopic evaluation of 82 patches revealed the following before treatment: yellow dots (93%), black dots (85%), broken hairs (76%), exclamation marks (47%) and upright regrowing hairs (42%). At the end of treatment, upright regrowing hairs (95%) and yellow dots (31%) were observed. Response and relapse rates did not differ between HPT and trichoscopic groups. However, the HPT group underwent more triamcinolone injections than the trichoscopy group (P < 0.001). When we assessed both HPT and trichoscopic

Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis. Immunoblotting and enzyme-linked immunosorbent assays revealed that the patient had multiple autoantibodies against desmoglein 1, desmocollin 1, 2, 3, laminin gamma-1, envoplakin, and periplakin. The skin lesions completely healed following thymectomy and systemic corticosteroid therapy, but the oral ulcerations persisted through a follow-up period of over 2 years.

Mucocutaneous Telangiectasia as a Diagnostic Clue of Hereditary Hemorrhagic Telangiectasia: An Activin Receptor-Like Kinase-1 Mutation in a Korean Patient

Dear Editor: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant vascular disorder manifested by variable-sized arteriovenous malformations (AVMs) of the skin, central nervous system, and respiratory, gastrointestinal, and urogenital tracts1,2. A 43-year-old woman visited our dermatology clinic with a 3-year history of increasing numbers of purpuric, punctate, and tiny macules of the lip, oral cavity, and fingers (Fig. 1A~C). The patient had no history of recurrent epistaxis; however, her mother had recurrent epistaxis and brain AVMs. No other abnormalities were detected in laboratory workups, particularly to detect visceral AVMs, including computed tomography scans of the chest and abdomen, brain magnetic resonance imaging, and gastrointestinal endoscopy. Under clinical suspicion, we performed a mutation analysis for HHT in the patient and her mother. The mutation c.940C>T (p.His314Tyr) in activin receptor-like kinase-1 (ACVRL1) was confirmed in both the patient and her mother, confirming the diagnosis of HHT (Fig. 1D). Interestingly, this genetic alteration, previously reported in a Western pedigree3, is a novel point mutation in a Korean family. The cutaneous lesions were well improved by pulsed dye laser (Fig. 2). Fig. 1 (A~C) Typical cutaneous telangiectasias at characteristic sites: lip, oral cavity, and fingers. (D) Presence of the mutation c.940C>T (p.His314Tyr) in activin receptor-like kinase-1. Fig. 2 Skin lesions on the tongue showed excellent response after a single treatment using pulsed dye laser (Vbeam Perfecta; Candela Laser Corporation, Wayland, MA, USA): (A) before treatment and (B) after treatment. Clinically, HHT is diagnosed by the Curacao criteria, which include spontaneous and recurrent epistaxis, mucocutaneous telangiectasias, visceral AVMs, and family history of HHT in a first-degree relative1,3. HHT is definitively diagnosed when three of the criteria are satisfied, whereas a suspected diagnosis is given when two of the criteria are met. In molecular diagnosis, mutations are found in approximately 85% of HHT families; most of these cases involve mutations in the endoglin (HHT1) or ACVRL1 (HHT2) genes4. Mutations of the endoglin gene on chromosome 9, which cause HHT1, were not detected in this family. The prevalence of specific vascular abnormalities varies according to genotype; patients with HHT1 have a higher prevalence of pulmonary and cerebral AVMs, while hepatic AVMs occur more commonly in HHT2 patients4. In our case, although the patient and her mother did not fulfill the criteria for a definitive diagnosis, HHT was strongly suspected because of the patients typical mucocutaneous lesions. Therefore, targeted genetic testing for the endoglin and ACVRL1 genes should be performed to establish an early diagnosis if there are characteristic cutaneous features and/or a family history of HHT. The cutaneous stigmata, which are characterized by an accumulation of small-caliber and superficial vessels, can cause cosmetic concerns and a risk for bleeding1,2,5. In our case, treatment with pulsed dye laser resulted in dramatic improvement of the skin lesions, similar to results reported by Halachmi et al.2. Multiple treatments and follow-up visits can be necessary because of the lower response of HHT than non-HHT telangiectasia and the possible accumulation of new vascular lesions2. In conclusion, to initially rule out an HHT diagnosis, genetic study is an available option. Because of the superficial nature of vascular lesions, shorter wavelength vascular lasers such as the pulsed dye laser can be considered an effective and safe treatment option for HHT.

Palmar melanoma: a tertiary centre experience

after the procedure. At 12 weeks, all patients (n = 5) had appreciable degree of increase in hair counts, confirmed with investigator-evaluated improvement in GPI (40%–75%; average of 57%) (Fig. 2), trichoscopic hair count increment (48-93 HFU/cm; average of 67 HFU/cm) and patient satisfaction evaluated with VAS ranging from 4 to 8 with a mean of 6 (Table 1). Except for mild pain experienced during thread insertion (n = 5), and mild transient swelling (n = 2), the procedure was very well tolerated by all. There was no case of significant bleeding, ecchymosis, persistent pain, headache or postprocedure infection. Polydioxanone (PDO) filament, a synthetic absorbable suture prepared from polyester, poly (p-dioxanone) has high flexibility and high retention strength, is non-allergenic and has a slow absorption rate (6–8 months). The risk of bacterial colonization or infection is minimal. As a non-surgical face lift modality, threads stimulate neocollagenesis within 2–3 weeks with clinical results expected to last for 2–3 years. We preferred monofilament threads, instead of barbed/screwed. Although the exact mechanism of action of hair growth stimulation by PDO threads remains speculative, it is likely to be similar to that of microneedling, involving enhanced expression of hair-related genes, release of growth factors like PDGF and direct activation of stem cells in the hair bulge area. A randomized trial by Dhurat et al.had indeed displayed the superiority of the combination of microneedling with minoxidil over minoxidil alone in MPHL. Although this pilot study seems to offer scalp threading as a novel efficacious and safe non-surgical approach to hair regrowth, the limitations of this study including small number of cases, limited follow-up period and lack of scalp histological analysis warrant further research with controlled trials with a larger cohort. Further, the persistence of the hair growth-stimulating effect of the threads, and theoretical possibility of foreign body granuloma formation in the long term remain to be explored.

Increased serum antibody titer against HPV-16 antigen in patients with Behçet's disease

Quadrivalent human papillomavirus (HPV) vaccine has been reported to be significantly associated with Behçets disease (BD). However, no reports have described HPV infection as a possible cause for the development of BD. The objective of this study was to evaluate whether anti-HPV immunoglobulin G (IgG) antibody titer is increased in BD. Serum samples from 93 Korean BD patients, who fulfilled the diagnostic criteria of the International Study Group for BD, were used in an enzyme-linked immunosorbent assay (ELISA). The clinical activity of BD was evaluated at the time of blood sampling. HPV-16 L1 virus-like particle (VLP) antigen was used in this study for the ELISA. Patients with BD had significantly higher antibody titers against HPV-16 (optical density [OD], 0.210–3.675; mean 0.992) than that of healthy controls (OD, 0.248–0.762; mean 0.517; P < 0.001). Using a receiver operating characteristic (ROC) analysis, a cut-off value of 0.578 OD for the anti-HPV antibody titer was determined that differentiated BD patients from healthy controls. When we compared the clinical features of BD between the 2 groups, articular involvement of BD was more likely in patients with an anti-HPV-16 antibody titer < 0.578 OD (P = 0.035). In addition, patients with an anti-HPV-16 antibody titer < 0.578 were significantly younger than those with a titer ≥ 0.578 OD. HPV itself may be a possible extrinsic triggering infectious agent causing the development of BD.

Unilateral linear basaloid follicular hamartoma: Its diagnosis and successful treatment with surgical excision and adjuvant dermabrasion

Dear Editor, Basaloid follicular hamartoma (BFH) is a rare tumor of hair follicle origin that usually occurs in the head and neck area. There are several clinical subtypes of BFH, but the unilateral linear form has rarely been reported. A 14-year-old male presented with skin lesions of the face, trunk and extremities since childhood (Fig. 1a,b). The patient had no family history of tumorous or genetic diseases. Physical examination revealed subcutaneous masses and nodules localized on left side of the body. Laboratory tests including complete blood count, serum chemistry, autoimmune antibodies and C3/C4 complements were within normal limits. Histological examination revealed basaloid cell proliferation forming irregular buds and anastomosing cords with peripheral palisading (Fig. 1d,e). There was no cleft between the nests and stroma. The immunohistochemical stain for CD10 was positive for peritumoral stroma (Fig. 1f). The diagnosis of unilateral linear BFH was established. Surgical removal had been performed starting from the exposed site. Large lesions were surgically excised and reconstructed by local flap or full-thickness skin graft. Scar revision and fractional carbon oxide laser therapy were performed to minimize scarring. For papules of the nose and glabella, two sessions of dermabrasion resulted in marked improvement without excision. All facial lesions had been completely removed and no single lesions had recurred during 11 years of follow up. The surgical treatment had led to cosmetic improvement with minimal scars (Fig. 1c). There have been only approximately 10 cases of unilateral linear form since first reported by Carney in 1952. Although the generalized type is known to be often associated with myasthenia gravis, there was no other associated abnormality in this unilateral case. Previous reports hypothesized that genetic mosaicism could cause the disease to be lateralized. However, the pathogenesis of linear unilateral BFH has not yet been well established. Differentiating BFH from BCC can be challenging due to histological similarity, but is of the utmost importance to avoid inappropriate treatment. Histopathological features of BFH include basaloid proliferation with