Clinicopathological and immunohistochemical characteristics of bullous pilomatricoma: a retrospective, single-center study, and comparison with ordinary pilomatricoma.

BACKGROUND: Bullous pilomatricoma is a rare variant of pilomatricoma. As it has been published in sporadic case reports, a limited understanding of its clinicopathological characteristics restricts its effective diagnosis and treatment. OBJECTIVES: This study aimed to analyze the clinicopathological and immunohistochemical characteristics of bullous pilomatricoma to better understand the bullous transformation of pilomatricoma. METHODS: The authors conducted a retrospective study of 12 patients with bullous pilomatricoma and compared their clinical, histopathological, and immunohistochemical data with those of patients with ordinary pilomatricoma. RESULTS: Bullous pilomatricoma showed no sex preference, with a mean onset age of 31.2 years. The common sites were the upper extremities and trunk. Bullous pilomatricoma had a shorter disease duration, a larger diameter, and a greater tendency to increase in size than those of ordinary pilomatricoma. Histopathologically, bullous pilomatricoma had a shorter duration, lesser calcification, more mitotic figures, and distinct dermal features from those of ordinary pilomatricoma. Immunohistochemically, the expression of Matrix Metalloprotease (MMP)-2, MMP-9, vascular endothelial growth factor receptor-3 (VEGFR-3), and VEGF-C was elevated. STUDY LIMITATIONS: The study was retrospective, and the sample size was small. CONCLUSION: The distinctive features of bullous pilomatricoma potentially result from dermal changes associated with the release of angiogenic factors and proteolytic enzymes. This comprehensive analysis provides novel insights into the clinical features and pathogenesis of bullous pilomatricoma.

Clinicopathological and immunohistochemical characteristics of bullous pilomatricoma: a retrospective, single-center study, and comparison with ordinary pilomatricoma

Abstract Background Bullous pilomatricoma is a rare variant of pilomatricoma. As it has been published in sporadic case reports, a limited understanding of its clinicopathological characteristics restricts its effective diagnosis and treatment. Objectives This study aimed to analyze the clinicopathological and immunohistochemical characteristics of bullous pilomatricoma to better understand the bullous transformation of pilomatricoma. Methods The authors conducted a retrospective study of 12 patients with bullous pilomatricoma and compared their clinical, histopathological, and immunohistochemical data with those of patients with ordinary pilomatricoma. Results Bullous pilomatricoma showed no sex preference, with a mean onset age of 31.2 years. The common sites were the upper extremities and trunk. Bullous pilomatricoma had a shorter disease duration, a larger diameter, and a greater tendency to increase in size than those of ordinary pilomatricoma. Histopathologically, bullous pilomatricoma had a shorter duration, lesser calcification, more mitotic figures, and distinct dermal features from those of ordinary pilomatricoma. Immunohistochemically, the expression of Matrix Metalloprotease (MMP)-2, MMP-9, vascular endothelial growth factor receptor-3 (VEGFR-3), and VEGF-C was elevated. Study limitations The study was retrospective, and the sample size was small. Conclusion The distinctive features of bullous pilomatricoma potentially result from dermal changes associated with the release of angiogenic factors and proteolytic enzymes. This comprehensive analysis provides novel insights into the clinical features and pathogenesis of bullous pilomatricoma.

SIRT1 downregulation provokes immune-inflammatory responses in hair follicle outer root sheath cells and may contribute to development of alopecia areata.

BACKGROUND: Silent information regulator 1 (SIRT1), a type III histone deacetylase, is involved in various cutaneous and systemic autoimmune diseases including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis. However, little is known about the role of SIRT1 in the development of alopecia areata (AA). OBJECTIVES: This study investigated whether SIRT1 regulates the hair follicle immune system and is involved in AA pathogenesis. METHODS: SIRT1 expression in human scalp tissue was analyzed using immunohistochemical staining, qPCR, and western blotting. The regulatory effect of SIRT1 was evaluated after stimulation with the double-stranded RNA mimic polyinosinic:polycytidylic acid (poly I:C) in hair follicle outer root sheath (ORS) cells and C3H/HeJ mice. RESULTS: SIRT1 expression was significantly reduced in the AA scalp compared to the normal scalp. SIRT1 inhibition upregulated MHC class I polypeptide-related sequence A and UL16 binding protein 3 in hair follicle ORS cells. SIRT1 inhibition also promoted the production of Th1 cytokines (IFN-γ and TNF-α), IFN-inducible chemokines (CXCL9 and CXCL10), and T cell migration in ORS cells. Conversely, SIRT1 activation suppressed the autoreactive inflammatory responses. The counteractive effect of the immune response by SIRT1 was mediated through the deacetylation of NF-κB and phosphorylation of STAT3. CONCLUSION: SIRT1 downregulation induces immune-inflammatory responses in hair follicle ORS cells and may contribute to AA development.

Intravascular Papillary Endothelial Hyperplasia in Association with Eccrine Angiokeratomatous Hamartoma.

Eccrine angiokeratomatous hamartoma is a variant of eccrine angiomatous hamartoma. Histopathologically, it shows both features of eccrine angiomatous hamartoma with components of angiokeratoma. Eccrine angiokeratomatous hamartoma is extremely rare. Eccrine angiokeratomatous hamartoma in our case co-existed with intravascular papillary endothelial hyperplasia. This is the first reported case.

Association of adenotonsillar disease and adenotonsillectomy with the development of vitiligo: A nationwide population-based cohort study.

Background: Vitiligo is a common acquired skin depigmentation disorder and is associated with various other autoimmune diseases which include thyroid disease and rheumatoid arthritis. Similarly, adenotonsillar disease (ATD) may induce inflammatory or autoimmune diseases in other organs which include the skin. However, the influence of ATD on the development of vitiligo has not been studied. Objectives: To determine the association between ATD and adenotonsillectomy, and the development of vitiligo. Design and methods: Using data from the National Health Insurance Service database, patients diagnosed with ATD between 2008 and 2010 were included in the study. We performed two rounds of 1:1 propensity score matching in the ATD and adenotonsillectomy groups. The ATD and non-ATD groups both included 206,514 individuals. Among the ATD group, the adenotonsillectomy and non-adenotonsillectomy groups both included 23,354 individuals. Each individual was monitored until 2019. The primary end point was the risk of vitiligo. Using the Cox Proportional Hazards model, the incidence of vitiligo and the hazard ratio (HR) were calculated. Results: The incidence of vitiligo was 1.16-fold higher in the ATD group than in the non-ATD group [adjusted HR (aHR), 1.16; 95% confidence interval (CI), 1.09-1.24] and 0.82-fold lower in the adenotonsillectomy group than in the non-adenotonsillectomy group (aHR, 0.82; 95% CI, 0.68-0.99). Additionally, the other risk factors for developing vitiligo included thyroid disease (aHR, 1.48; 95% CI, 1.11-1.98), age younger than 30 years (aHR, 1.18; 95% CI, 1.09-1.27), and age over 60 years (aHR, 1.22; 95% CI, 1.06-1.41), whereas factors including rural residency (aHR, 0.91; 95% CI, 0.85-0.98) and low economic status (aHR 0.87; 95% CI, 0.82-0.93) were associated with decreased incidence of vitiligo. Conclusion: In this study, ATD increases the risk of vitiligo and adenotonsillectomy attenuates its development. Clinicians should consider ATD as a pathogenic factor for vitiligo and the potential effect of adenotonsillectomy in its management.

First report of Tulip Virus X infecting tulip (Tulipa gesneriana) in Korea.

Tulip virus X (tulip virus X, TVX) is a member of the genus Potexvirus (family Alphaflexiviridae) and is a positive single-stranded RNA virus. TVX was described first in Scotland (Mowat 1982), followed by several countries (Yamaji et al. 2001; Tzanetakis et al. 2005; Ward et al. 2008; Dees et al. 2011; Sochacki and Komorowska 2012; Wylie et al. 2019). In April 2021, 86 whole tulip plants showing viral symptoms in leaves (mosaic, yellowing, and malformation) and flowers (color breaking) were collected in Chilgok, Chuncheon, Goseong, Yecheon and Yesan, Korea. Furthermore, high-throughput sequencing was performed to identify viruses that infect tulips in Korea. Total RNA was extracted from pooled the leaves and petals using a Maxwell® 16 LEV Plant RNA Kit (Promega, Madison, USA). We constructed a single library using the TruSeq Stranded Total RNA LT Sample Prep Kit for Plant (Illumina, San Diego, USA). The library was 100 bp paired-end sequenced using Illumina's NovaSeq 6000 (Macrogen, Seoul, Korea) and was assembled de novo using Trinity software version trinityrnaseq_r20140717, with default parameters. The contigs were annotated as in previous study (Lee et al. 2020), revealing a single contig each related to TVX, lily symptomless virus (LSV), and tulip breaking virus (TBV) was generated from 648 million total reads. The TVX-related contig (GenBank ON205948) consisting of 6,076 bp showed 99.52% nucleotide identity (6027/6056 bp) with TVX-J (GenBank AB066288). We conducted an RT-PCR assay to validate the presence of viruses with specific primers as TVX-F5093/R5624 (5'-CTATCCGGACTCATTCTACTTC/GTGCGTTCCAGATAAGCTTG-3'), LSV-F7013/R7338 (5'-CTTGGTCGACAGGGACATAAC/GATTGGAATTGTGCTTTTCAGC-3'), and TBV-F7515/R8116 (5'-GTGTGTCATGGATGATTGTTG/CAACTGATTTGCTACCGCTAG-3'). Consequently, TVX were detected in 13 of 86 samples. Moreover, LSV and TBV were detected in 15 and 26 samples, respectively. However, the yellowing and mosaic observed in the TVX infected samples were not observed in the LSV and TBV infected samples. Subsequently, two TVX amplicons were selected, cloned and sequenced. The obtained sequences were 532 bp and were named YS24 and YS38 (GenBank LC664027 and LC664028), respectively. The Korean isolates showed 98.68% (525/532 bp) and 99.62% (530/532 bp) identity with Australian isolate (GenBank MH886522) in BLASTn analysis. To bioassay for TVX, the infected tulip leaf tissue from which YS24 was obtained was used to sap-inoculate, in triplicates, 15 species of indicator plants (Nicotiana benthamiana, N. clevelandii, N. debneyi, N. glutinosa, N. rustica, N. tabacum, Datura stramonium, Glycine max, Phaseolus vulgaris, Chenopodium amaranticolor, C. quinoa, Cucumis sativus, Cu. melo, Gomphrena globosa, and Tetragonia tetragonioides). After 14 days of inoculation, we observed distinct chlorotic spots on inoculated and upper leaves of C. quinoa, but no symptoms were observed in other indicator plants. In RT-PCR assay using TVX-specific primers, only C. quinoa showed a positive reaction. In previous studies, C. amaranticolor, C. quinoa, G. globosa, and N. benthamiana were known as the experimental host of TVX (Dees et al. 2011; Tzanetakis et al. 2005), but only C. quinoa was confirmed to be susceptible to the Korean isolate. Furthermore, transmission electron microscopy revealed typical flexuous rod-shaped viral particles in the inoculated C. quinoa. To our knowledge, this is the first report of TVX infecting tulips in Korea.

A Clinicohistopathological Analysis of Cutaneous Fibrous Histiocytomas of the Finger.

BACKGROUND: Cutaneous fibrous histiocytoma (CFH) is a common, benign skin tumor predominantly occurring on the extremities or trunk. However, CFH on the finger is rare. OBJECTIVE: This study was undertaken to examine the clinicohistopathological features of CFH of the finger. MATERIALS AND METHODS: This is a retrospective study of 12 CFHs located on fingers in a tertiary hospital in Korea. All case slides were retrieved from saved files. RESULTS: Ages of the CHF of the finger affected individuals ranged from 9 to 48 years with a male-to-female ratio of 1.4:1. Picker's nodule or wart was the most common clinical diagnosis. In only 2 out of the 12 cases was the pre-biopsy diagnosis of CFH ventured. Fibrocollagenous type was the most common histological type. Majority of the cases were mitotically inactive, exhibiting only 0-1 mitoses per high-power field and there was no recurrence. Tumor cells were uniformly CD34 negative. CONCLUSION: Because CFH can resemble malignancies including dermatofibrosarcoma protuberans, a lack of familiarity with the occurrence of CFH of the finger may lead to more aggressive treatment. Dermatologists should include CFH in their differential diagnosis of circumscribed nodules on the fingers to ensure proper management.

Cutaneous Carcinosarcoma: a Clinicopathologic and Immunohistochemical Analysis of 11 Korean Cases.

BACKGROUND: Cutaneous carcinosarcoma is a rare biphasic tumor comprising malignant epithelial and heterologous mesenchymal elements. Data on the clinical and histopathologic characteristics of this tumor in Asian populations are not available. The purpose of this study was to investigate the clinicopathologic and immunohistochemical features of cutaneous carcinosarcoma in the Korean population. METHODS: We retrospectively reviewed the records of 11 patients with cutaneous carcinosarcoma who were diagnosed from 2006 to 2016. RESULTS: The mean patient age at diagnosis was 71.5 years (range, 43-96 years) and there was a men predilection. The most common site of cutaneous carcinosarcoma was the head and neck (8/11, 72.7%). Histopathologically, most tumors showed a characteristic morphology consisting of two types of tumor cells, varied differentiated epithelial cells (such as basal or squamous cells) and spindle cells with transition zones between the two components. These two cell types also demonstrated variable immunohistochemical characteristics. CONCLUSION: Although the number of cases in this study was limited, our results provide valuable insight into the clinical and histopathologic characteristics of cutaneous carcinosarcoma in the Korean population.

Biological evaluation of novel derivatives of the orange pigments from Monascus sp. as inhibitors of melanogenesis.

Nitrogenous derivatives of the two orange pigments from Monascus sp. with anti-melanogenic activities were prepared using fermentation and chemical synthesis. The pigments were produced in a 5 l jar fermentor. A total of 33 derivatives were synthesized via incorporation of L-amino acids and amines into the pigments. Two derivatives with high inhibitory melanin-synthesizing activities and low cell toxicities were selected based on testing using B16F10 cells. Glutamic acid and (S)-(+)-1-amino-2-propanol derivatives showed high inhibitory activities against melanogenesis. Both the reaction and expression of tyrosinase, an important enzyme in the melanin-synthesizing pathway, were inhibited by the glutamic acid derivative in a dose-dependent manner. The (S)-(+)-1-amino-2-propanol derivative inhibited expression of tyrosinase in cells, but not the tyrosinase reaction. TRP1 and TRP2, other important proteins in melanin-synthesis, were not affected by the two derivatives.

Clinical and histopathological characteristics of extra-facial basal cell carcinoma: analysis of 35 patients at the Chonbuk National University Hospital in Korea.

Basal cell carcinoma (BCC) primarily develops in the head and neck region, with 74-83 per cent of BCC occurring in this region. Unfortunately, most published studies on BCC were conducted in Caucasian populations, and analytic data on extra-facial BCC in Asian and Korean patients, in particular, are not readily available. Here, we report on a retrospective analysis of extra-facial BCC in Korean patients. Thirty-five patients (16 men, 19 women) diagnosed with extra-facial BCC at Chonbuk National University Hospital between January 1981 and December 2008 were evaluated. Their average age was 62.3 years and most of the patients (11 of 35, 31%) were in their fifties. The relative tumour density (RTD) was the highest in the genitalia (0.769), followed by the axilla (0.481). Other regions such as the trunk, buttocks and upper and lower extremities exhibited a much lower RTD (average: 0.1). Histopathological examinations showed that 16 tumours were nodular (46%), eight were superficial (23%) and seven were mixed (20%). Additionally, potential predisposing factors were identified in seven cases. In five patients the use of Asian medicine, including acupuncture and herbal medication, was ascertained. To the best of our knowledge, the present study is the first to analyse the clinical and histopathological characteristics of extra-facial BCC in Korean patients. Our results indicate that the incidence of extra-facial BCC is higher in the axilla and genitalia than at other locations, although these sites are frequently overlooked during routine skin examinations.

Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus.

Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5-148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.

The three dimensional conformal radiotherapy for hyperkeratotic plantar mycosis fungoides.

The localized early-stage of Mycosis fungoides (MF) (stage IA-IIA) is usually treated with topical agents, such as nitrogen mustard, steroids, and phototherapy (UVB/PUVA) as first line therapy; response to these initial treatments is usually good. However, hyperkeratotic plantar lesions are clinically rare and have decreased responsiveness to topical agents. For such cases, physicians may consider local radiotherapy. Here, a case of an 18-year-old Korean woman who was treated with three-dimensional conformal radiotherapy (3D-CRT) for hyperkeratotic plantar lesions that were refractory to UVA-1, methotrexate, and topical steroids is reported. Complete remission was attained after radiotherapy. During the one-year follow-up period, there has been no evidence of disease recurrence and no chronic complications have been observed.

Mycosis fungoides as an ichthyosiform eruption.

Ichthyosiform eruption as a specific manifestation of mycosis fungoides is very rare and only a few such cases have currently been reported in the medical literature. A 63-year-old Korean man presented with a 4-year history of a pruritic ichthyotic eruption. There was no personal or family history of ichthyosis or atopy. The ichthyosiform skin changes involved the abdomen, arms, thighs and shins. The face, palms and soles were spared. There was no peripheral lymphadenopathy or organomegaly. The typical lesions of mycosis fungoides were not present. The results of the routine investigations were normal or negative. A skin biopsy specimen revealed the findings of early mycosis fungoides. He was successfully treated with photochemotherapy.