Hyunju Jin

Clinical features and course of generalized pustular psoriasis in Korea

The clinical course of generalized pustular psoriasis (GPP) is variable and unpredictable. Sufficient data on the clinical course of the disease has not been reported due to its rarity. To investigate the clinical features and course of GPP according to its subtypes, medical records of patients diagnosed with GPP from 2002 to 2012 at two tertiary hospitals were reviewed. The data included patient demographics, associated symptoms, aggravating factors, patterns of relapse and prognosis. Thirty‐three patients with GPP were included in our study, with a mean age of 45.6 years and a male : female ratio of 1:1.2. Patients were categorized based on the following subtypes: acute GPP, 21 (63.6%); GPP of pregnancy, two (6.1%); juvenile GPP, three (9.1%); and annular GPP, seven (21.2%). In the acute GPP population, skin lesions cleared within 2 months in 11 (73.3%) patients, and six (40.0%) of these had no relapse. Severe complications, abortion or death, were observed in two patients (100.0%) with GPP of pregnancy. Nineteen (76.0%) of the GPP patients experienced persistence or relapse of skin lesions. The patterns of skin lesions upon relapse included plaques in six patients (31.6%), pustules in eight patients (42.1%), and plaques and pustules in five patients (26.3%). Among acute GPP patients, 16.7% of patients with no relapse had a history of plaque psoriasis. However, 77.8% of patients with persistence and relapse in their clinical course had a history of plaque psoriasis. In conclusion, our study presents the detailed clinical course of GPP by subtype in Korean patients.

Localized argyria: troublesome side-effect of acupuncture

events of PD-1 immunotherapy are being increasingly reported and characterized. Since our initial publication, additional cases of eruptive KAs have been reported, supporting its status as an associated adverse event. It is encouraging to see these reports so that we can better characterize the adverse event profile and gain insight into pathogenesis. It is unclear exactly why KAs paradoxically erupt secondary to a medication used to treat squamous cell carcinomas (SCCs) of the lung and head/neck. It is further confusing in the light of a recent publication reporting very similar PD-L1 expression profiles between KAs and cutaneous SCCs. Nevertheless, the eruption of KAs in PD-1 immunotherapy may prove fortuitous by providing new insight into their unique biology. Although KAs may simply represent a well-differentiated variant of SCC, many consider them a distinct entity – a notion supported on the molecular level. We know PD-1 inhibitors enhance antitumour activity by blocking the immune downregulation that occurs when PD-1 on T cells binds its ligands, often strategically overexpressed on malignant tumour cells. However, antitumour activity is not the only T-cell function ‘unchecked’. Tcell receptor signalling, cytokine production, lymphocyte motility and metabolic programming are all ‘unchecked’ by inhibiting PD-1. Perhaps then KAs are closer related to a reactive proliferation akin to exuberant squamous metaplasia, and a pro-inflammatory cytokine milieu injures predisposed keratinocytes in a way that incites a reactive proliferation that outpaces any enhanced antitumour activity. In the same vein, increased cytokine production (e.g., IL-2, IL-4) could tip a precarious inflammatory balance towards Th1 (lichenoid dermatitis, vitiligo) or Th2 (antibody-related disease) pathways. Further investigation into these immune-related adverse events is certainly needed and might include the immunohistochemical characterization of the inflammatory infiltrate before, during and after immunotherapy. Such information may provide further insight into pathogenesis as well as possible immunohistochemical methods for monitoring treatment efficacy and progress.

Inappropriate amounts of topical tacrolimus applied on Korean patients with eczema

Abstract Background: The limited efficacy of topical tacrolimus may result from insufficient frequency of application or amount applied in eczema patients. Objective: To investigate the frequency of application and amount of use of topical tacrolimus in patients with various types of eczema. Methods: The frequency of application and the applied amount of topical tacrolimus were assessed over two weeks. Results: A total of 200 eczema patients completed this study. The average number of applications per day was 1.75 ± 0.53, despite instructions to apply the topical tacrolimus twice daily. With respect to the frequency of application, 147 (73.5%) and 122 (61.0%) of patients followed the prescription in the first and second weeks, respectively. The average amount applied per 2% of total body surface area (TBSA) was 0.54 ± 0.52 g. Only 53 (26.5%) patients applied between 80 and 120% of expected amount of topical tacrolimus. Limitations: The frequency of application was self-reported, possibly resulting in limited accuracy. Conclusion: Korean patients with eczema tend to apply topical tacrolimus less frequently and in inappropriate amounts. Clear instructions regarding both the frequency and amount of application are needed to improve the therapeutic outcome with treatment with topical tacrolimus.

Arborizing Vessels on Dermoscopy in Various Skin Diseases Other Than Basal Cell Carcinoma

Background Arborizing vessels (AVs) are dermoscopically defined as telangiectasias with distinct treelike branching, and are a characteristic feature of basal cell carcinoma (BCC). However, AVs are observed in various conditions other than BCC. Objective The aim of this study was to investigate skin diseases showing AV and investigates dermoscopic differences between BCC and non-BCC. Methods Dermoscopic images showing AV were prospectively collected and classified into BCC/non-BCC. Non-BCC was further classified into tumors (benign cystic, benign non-cystic, premalignant, and malignant) and non-tumors. We compared AV focusing, widest diameter of stem vessels, widest diameter ratio of stem vessel to first branch, and number of ramifications between groups. Results Among 124 images, 54.0% were BCC and 46.0% were non-BCC. Non-BCC included epidermal cysts, hypertrophic scars/keloids, intradermal nevi, actinic keratoses, etc. The proportion of focused AV in BCC was significantly higher and the proportion of unfocused AV in BCC was lower than that of premalignant and malignant non-BCC. The widest diameter ratio of stem vessel to first branch was higher in non-BCC. Number of ramifications was significantly less in benign cystic non-BCC than BCC. Conclusion Various skin diseases showed AV, so that diagnoses other than BCC should be considered. The findings in this study could help discriminate BCC from non-BCC.

Cutaneous Mucor hiemalis infection diagnosed by molecular methods.

References 1 Navarro V, Pinazo I, Mart ınez E, et al. Facial superficial porokeratosis. Dermatology 2000; 201: 361. 2 Otsuka F, Shima A, Ishibashi Y. Porokeratosis as a premalignant condition of the skin. Cytologic demonstration of abnormal DNA ploidy in cells of the epidermis. Cancer 1989; 63: 891–896. 3 Jung K, Seifert M, Herrling Th, et al. UV-generated free radicals (FR) in skin: their prevention by sunscreens and their induction by self-tanning agents. Spectrochim Acta A Mol Biomol Spectrosc 2008; 69: 1423–1428.

Alitretinoin can be a good treatment option for idiopathic recalcitrant trachyonychia in adults: an open‐label study

Trachyonychia can be refractory to conventional treatments including topical, intralesional or systemic corticosteroids, as well as cyclosporine and retinoids. Therefore, new treatment options are needed for recalcitrant trachyonychia.

The importance of dermoscopy for the diagnosis of acquired bilateral telangiectatic macules: the angioid streak pattern reveals underlying chronic liver disease

Acquired bilateral telangiectatic macules (ABTM) are a newly recognized disease entity, which manifest as multiple telangiectatic pigmented macules confined mostly to the upper arms.

Association between Breslow Thickness and Dermoscopic Findings in Acral Melanoma

Background Dermoscopy is a useful tool for the diagnosis of acral melanomas (AMs). However, little is known about the influence of tumor thickness on the dermoscopic findings of AM. Objective To investigate the affect Breslow thickness (BT) has on the dermoscopic patterns of AM. Methods Data on cases of AM on the glabrous skin were collected from 4 university hospitals. We investigated the frequency of each dermoscopic feature of AM according to the BT. Statistical analysis was performed to investigate the association between the specific dermoscopic patterns and BT. Results Multivariable analysis revealed that the colors red (odds ratio [OR] 16.482, 95% confidence interval [CI] 3.605‐99.016); blue (OR 7.092; 95% CI 1.707‐37.435); and white (OR 5.048, 95% CI 1.152‐22.897) were more common in AM with BT >2 mm than those with BT ≤2 mm. Regarding patterns, atypical vascular (OR 34.589, 95% CI 6.458‐305.852); blue‐white veils (OR 9.605, 95% CI 1.971‐72.062); and ulcers (OR 5.084, 95% CI 1.145‐24.152) were more frequently detected in cases with BT >2 mm than those with BT ≤2 mm. Limitations A retrospective study design and small sample size. Conclusion This study showed an association between dermoscopic patterns and tumor thickness among patients with AM. Dermoscopy can be a useful adjuvant tool for predicting BT in AM.

Pigmentary mosaicism with trisomy 7.

Dear Editor, Hypomelanosis of Ito (HI) is a sporadic pigmentary anomaly along the Blaschko lines, characterized by linear and whorled hypopigmentation. In 1992, Ruiz-Maldonado et al. published a suggestion for diagnostic criteria of HI based on vast clinical experience. Nevertheless, it is important to point out that HI is not a specific diagnosis, but may occur owing to several different chromosomal abnormalities. This group of disorders is better termed “pigmentary mosaicism” (PM). A 21-month-old boy was initially examined because of hypopigmentation that appeared as linear and whorled streaks along the Blaschko lines (Fig. 1a,b). He was delivered at 41 weeks’ gestation with a birthweight of 2880 g. The pedigree showed no evidence of similar abnormalities. Early infant development was normal. By Ruiz-Maldonado’s criteria, he was diagnosed as presumptive HI. Cytogenetic analysis of peripheral blood revealed normal standard karyotype (46, XY). Microarray comparative genomic hybridization (aCGH) did not detect any microdeletion or duplication. Histological examination of the hypopigmented area revealed mildly decreased melanin pigments in the basal layer compared with normal skin (Fig. 1c–f). Karyotype analysis of fibroblast cultures from the hypopigmented lesion revealed a trisomy 7 cell line, 47, XY, +7, and that from normal skin showed 46, XY (Fig. 1g,h). Thus, a diagnosis of PM with trisomy 7 was made, and on recent examination at age 2.5 years, no extracutaneous abnormalities were observed. Although all patients with PM have been hypothesized to have underlying mosaicism, chromosomal mosaicism is found in only 20–30% of the patients. It is caused not only by submicroscopic genetic abnormality, but possibly also because conventional karyotyping of blood lymphocytes or skin fibroblasts cannot always show chromosomal abnormalities. Blood is a conventional specimen for karyotyping. However, it is recommended to karyotype skin fibroblasts as lymphocytes may not demonstrate the abnormal cell line. Such discordance between karyotypes of blood and skin is rare but has been reported. Skin pigmentation is closely related to epidermal–dermal interaction. Although pigmentation is primarily related to the melanocytes, the surrounding keratinocytes and extracellular matrix proteins and fibroblasts in the underlying dermal compartments actively contribute to cutaneous homeostasis. Thus, we suppose that skin biopsy is the best way to obtain an appropriate specimen for karyotyping, and it is better to check both fibroblasts and keratinocytes. Mosaic trisomy 7 is a rare condition. Several pigmentary genes on chromosome 7 were identified (EGFR, GPNMB, HGF, EPHA1, GPDS1) that may affect abnormal pigmentation in HI. Another trisomy mosaicism associated with HI has also been reported. No effective treatment for pigmentary mosaicism has been established; the hypopigmented macules persist throughout life, and the prognosis is influenced by the associated extracutaneous findings such as renal malformation, facial dysmorphism and growth retardation. Ours is the only case of trying to find any submicroscopic abnormalities in blood by using aCGH. However, as in our case, mosaic trisomy 7 has not yet been demonstrated in peripheral blood. Therefore, skin biopsy is crucial for karyotyping and should be performed promptly for chromosome analysis.

High rates of secondary non-adherence causes decreased efficacy of 0.1% topical tacrolimus in adult eczema patients: results from a multicenter clinical trial

Abstract Background: Patients tend to apply topical medications less frequently, in improper amounts. Not only frequency but also application amount may influence treatment outcome. However, studies on relationship between application amount and objective treatment outcome have rarely been conducted. Objective: To assess efficacy of topical agent according to application amount in adult patients, using the finger-tip unit method. Methods: The efficacy of 0.1% topical tacrolimus in adult patients with localised atopic dermatitis was assessed using EASI, TIS, IGA, and PGA scores at baseline, follow-up. Adherence in amount was evaluated after 2 weeks of treatment using the ratio of the actual amount applied to the expected amount applied (A/E). Results: Twenty-seven patients (20.93%) used topical tacrolimus in proper amounts (A/E: 0.8–1.2). However, 86 patients (66.67%) underused topical tacrolimus; 16 (12.40%) patients overused topical tacrolimus. Decreases in EASI scores between baseline and 2 weeks of follow-up in each group (under-amount, proper amount, over-amount) were 1.64, 4.65 and 4.21, respectively. Treatment efficacy increased in accordance with application amount. Further, TIS, IGA, PGA, VAS for Itch and DLQI scores improved concomitantly, exhibiting similar tendencies. Conclusion: Application amount of topical agent is important in increasing treatment efficacy in adult patients with atopic dermatitis.