摘要
Sweet syndrome is a disorder characterized by painful, erythematous, cutaneous plaques and nodules of rapid onset accompanied by fever, leukocytosis, and neutrophilia. We encountered a case of a recurrent histiocytoid Sweet syndrome in a patient with acute myeloid leukemia (AML). A 64-year-old Korean man was diagnosed with complete remission of AML and had symptomatic nodules and plaques on the dorsal sides of both hands. Approximately 3 years prior, he also had symptomatic plaques at the same site and had been diagnosed with MDS (Myelodysplastic syndrome). We performed a biopsy and diagnosed this case as a malignancy-associated histiocytoid Sweet syndrome. Most Sweet syndrome cases are acute; in contrast, this case was chronic with a relapse. In addition, histopathologic examination showed a dense histiocytic infiltration. These histiocytoid cells are usually misinterpreted as histiocytes; however, they are actually immature myeloid cells. Herein, we report a case of a recurrent malignancy-associated histiocytoid Sweet syndrome in a patient with a hematologic disorder.
Subject(s)
Humans , Middle Aged , Biopsy , Fever , Hand , Histiocytes , Leukemia, Myeloid, Acute , Leukocytosis , Myeloid Cells , Recurrence , Sweet Syndrome摘要
BACKGROUND: Alopecia areata (AA) is a T cell-mediated autoimmune disease that targets hair follicles and interrupts hair regrowth. The microenvironment of the effector T cells and their related cytokines may affect immunopathogenesis around the hair bulb/bulge. OBJECTIVE: To determine the contributory roles of the effector T cell subsets and related cytokines to the pathogenesis of AA. METHODS: We investigated the correlation between histopathological grades and four clinical prognostic factors in 331 patients with AA, and analyzed the topography of T cell infiltrates and related cytokines around the hair bulb/bulge according to histopathological grades through immunohistochemical and double immunofluorescence studies on a subset of AA specimens. RESULTS: First, the groups with more severe histopathological grades were associated with earlier onset, longer duration, more hair loss, as well as poorer therapeutic outcomes. Second, the pattern of CD4 and CD8 expression around the hair bulb/bulge varied by histopathological grade, with staining density decreasing in the following order: type 1>type 2>type 3. In addition, interferon-γ and transforming growth factor-β1 expression appeared denser in the peribulbar area. Interestingly, the denser CCR6⁺ cells (Th17 cells) showed more infiltration than CCR5⁺ cells (Th1 cells) around the hair bulb/bulge as histopathological grade worsened. CONCLUSION: The insidious destruction of bulge stem cells and hair bulb matrix stem cells results in more severe hair loss in patients with chronic AA, which is mediated by Th17 lymphocyte and cytotoxic T lymphocyte infiltration. Furthermore, Th17 lymphocytes may play an even more important role than cytotoxic T cells in the development of AA.
Subject(s)
Humans , Alopecia Areata , Alopecia , Autoimmune Diseases , Cytokines , Fluorescent Antibody Technique , Hair Follicle , Hair , Lymphocytes , Stem Cells , T-Lymphocyte Subsets , T-Lymphocytes , Th17 Cells摘要
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a newly defined entity that includes various clinical entities. Histopathologically, this disease is characterized by a granulomatous inflammation with or without leukocytoclastic vasculitis. PNGD shows vasculitic lesions in the early stage, with palisaded granulomatous lesions and dermal fibrosis with minimal leukocytoclastic debris appearing in the late stage. It is frequently associated with rheumatoid arthritis, lupus erythematosus, and other autoimmune diseases. A 14-year old Korean girl presented with multiple erythematous nodules and plaques on both elbows and knees that were present for 2 years prior to the initial visit. Clinically, she had multiple arthritis with morning stiffness and decreased C3 levels, as well as positive results for antinuclear antibodies, the lupus anticoagulant test, and anti-beta 2 glycoprotein I (IgG). Skin biopsy findings from the skin lesion indicated that the foci of degenerated collagens were palisaded with histiocytes throughout the dermis and neutrophils with leukocytoclasia infiltrated with fibrin, with mucin at the center of the lesion. Based on the SLICC 2012 criteria, we diagnosed her condition as PNGD in systemic lupus erythematosus. She was treated with hydroxychloroquine and topical application of 0.1% tacrolimus ointment, which resulted in the remarkable flattening of the skin lesions after 6 months of follow-up. Herein, we report a case of PNGD that may have been an indicator of systemic lupus erythematosus.
Subject(s)
Female , Humans , Antibodies, Antinuclear , Arthritis , Arthritis, Rheumatoid , Autoimmune Diseases , Biopsy , Collagen , Dermatitis , Dermis , Elbow , Fibrin , Fibrosis , Follow-Up Studies , Glycoproteins , Histiocytes , Hydroxychloroquine , Inflammation , Knee , Lupus Coagulation Inhibitor , Lupus Erythematosus, Systemic , Mucins , Neutrophils , Skin , Tacrolimus , Vasculitis摘要
BACKGROUND: The global prevalence of premalignant lesions has been continuously increasing in recent years, but there has been little research regarding the distribution and incidence of cutaneous premalignant lesions in Korean populations. OBJECTIVE: We conducted this retrospective study to analyze recent trends in the incidence and clinical patterns of cutaneous premalignant lesions in the Korean population. METHODS: We reviewed 1,292 cases (3,651 lesions) of patients with cutaneous premalignant lesions, including actinic keratosis (AK) and Bowen's disease (BD), from the Department of Dermatology at Dong-A University Hospital (January 1995 to December 2013). RESULTS: The average cutaneous premalignant lesion annual incidence was 1.82%, and the incidence continuously increased from 0.70% to 4.25% over the study period. The most common cutaneous premalignant lesion was AK (75.85%), followed by BD (24.15%). The mean age of onset was 68.76 years (men, 70.89 years; women, 65.56 years), and the male:female ratio of patients was 1:1.52. Major skin cancers, including squamous cell carcinoma (SCC, 8.90%), basal cell carcinoma (BCC, 6.42%), and malignant melanoma (MM, 0.70%), were detected in 15.79% of patients with cutaneous premalignant lesions. Three patients (0.23%) were previously diagnosed with both SCC and BCC. In addition, 59.13% of patients had a single lesion, while 40.87% had multiple lesions. Patient age, history of previous skin cancers, and occupation-related exposure to ultraviolet radiation were more common in patients with multiple lesions. CONCLUSION: Cutaneous premalignant lesion incidence has gradually increased in the Korean population.
Subject(s)
Female , Humans , Age of Onset , Bowen's Disease , Carcinoma, Basal Cell , Carcinoma, Squamous Cell , Dermatology , Incidence , Keratosis, Actinic , Korea , Melanoma , Precancerous Conditions , Prevalence , Retrospective Studies , Skin Neoplasms摘要
Pyoderma gangrenosum (PG) is rare chronic painful ulcerative skin disorder usually associated with various systemic diseases, the most common of which is inflammatory bowel disease (IBD). PG shows a particularly stronger correlation with ulcerative colitis than Crohn's disease. It generally occurs at sites of trauma (pathergy), butspontaneous lesions with no evident trauma are not uncommon. PG is classified into four major clinical types: ulcerative, pustular, bullous, and vegetating. The ulcerative type is mostly associated with IBD. On the other hand, the bullous type is known to be associated with lymphoproliferative disorders. We report a rare case of PG of the bullous types forming multiple lesions on the face of a 21-year-old Korean woman with ulcerative colitis.
Subject(s)
Female , Humans , Young Adult , Chronic Pain , Colitis, Ulcerative , Crohn Disease , Hand , Inflammatory Bowel Diseases , Lymphoproliferative Disorders , Pyoderma Gangrenosum , Pyoderma , Skin , Ulcer摘要
BACKGROUND: In the treatment of vitiligo, topical corticosteroids are known to be effective, but are associated with serious adverse effects. Many studies have shown that topical calcipotriol is a promising therapeutic modality in vitiligo. In some studies, combined calcipotriol and betamethasone dipropionate ointment has been shown to be a more effective and well tolerated treatment for vitiligo. The combination therapy seems to synergistically act as an immunosuppressive and a pigment restorative agent. OBJECTIVE: We investigated the clinical efficacy of CCB (Combination Calcipotriol and Betamethasone dipropionate) gel compared with that of betamethasone dipropionate alone in the repigmentation of vitiligo. METHODS: In an intraindividual right-left comparison study (n=20), a CCB gel was applied once daily to a lesion on one side, and betamethasone dipropionate cream was applied to a lesion on the other side. The degree of repigmentation was assessed according to the Vitiligo Area Scoring Index (VASI) at baseline, 4, 12, 24, and 48 weeks. RESULTS: The CCB gel treated group showed a remarkably improved therapeutic outcome compared to the betamethasone dipropionate monotherapy group: the percentages of VASI relative to the baseline at CCB gel treated sites were 82.73+/-8.17%, 70.45+/-14.05%, 62.73+/-17.52%, and 56.24+/-18.49% at 4, 12, 24, and 48 weeks after treatment, respectively; while those of the other sites receiving betamethasone dipropionate were 89.55+/-7.24%, 84.55+/-10.60%, 77.73+/-14.38%, and 73.48+/-12.93%. Adverse effects such as atrophy and burning sensations were much less after CCB gel treatment than after betamethasone monotherapy. CONCLUSION: CCB gel is more effective and tolerable than betamethasone dipropionate monotherapy in repigmentation therapy for vitiligo.
Subject(s)
Adrenal Cortex Hormones , Atrophy , Betamethasone , Burns , Sensation , Vitiligo摘要
Mastocytosis is characterized by an accumulation of mast cells in various organs, most frequently in the skin. A solitary mastocytoma is a clinical variant of cutaneous mastocytosis. It is defined as a localized collection of mast cells in the skin without evidence of extracutaneous organ involvement. Here we report on a 2-year-old female patient presenting with Solitary erythematous bulla on her lower back. The patient had a history of spinal tap on the lower back for evaluation of meningitis at 5 months of age, which resulted in trauma at the site. Histopathology showed mast cells infiltrating the papillary and reticular dermis and metachromatic purple cytoplasmic granules seen with Giemsa staining. As a result, the patient was diagnosed with a solitary bullous mastocytoma and administered antihistamine. The patient showed complete remission at 3 months. Herein, we report a rare case of solitary bullous mastocytoma occurring at a trauma site.
Subject(s)
Child, Preschool , Female , Humans , Azure Stains , Cytoplasmic Granules , Dermis , Mast Cells , Mastocytoma , Mastocytosis , Mastocytosis, Cutaneous , Meningitis , Skin , Spinal Puncture摘要
No abstract available.
Subject(s)
Humans , Colon , Colonic Neoplasms , Sparganosis , Ultrasonography摘要
OBJECTIVES: The purpose of this study was to determine a separation method for each arsenic metabolite in urine by using a high performance liquid chromatography (HPLC)- inductively coupled plasma-mass spectrometer (ICP-MS). METHODS: Separation of the arsenic metabolites was conducted in urine by using a polymeric anion-exchange (Hamilton PRP X-100, 4.6 mm x 150 mm, 5 mum) column on Agilent Technologies 1260 Infinity LC system coupled to Agilent Technologies 7700 series ICP/MS equipment using argon as the plasma gas. RESULTS: All five important arsenic metabolites in urine were separated within 16 minutes in the order of arsenobetaine, arsenite, dimethylarsinate, monomethylarsonate and arsenate with detection limits ranging from 0.15 to 0.27 mug/L (40 muL injection). We used GEQUAS No. 52, the German external quality assessment scheme and standard reference material 2669, National Institute of Standard and Technology, to validate our analyses. CONCLUSIONS: The method for separation of arsenic metabolites in urine was established by using HPLC-ICP-MS. This method contributes to the evaluation of arsenic exposure, health effect assessment and other bio-monitoring studies for arsenic exposure in South Korea.
Subject(s)
Argon , Arsenic , Cacodylic Acid , Chromatography, Liquid , Environmental Monitoring , Korea , Limit of Detection , Plasma , Polymers , Spectrum Analysis摘要
BACKGROUND: In the majority of melanomas, the RAS/RAF/MEK/ERK signaling pathway is constitutively activated, due to oncogenic mutations in the BRAF and NRAS genes. The BRAF mutation has been mainly described in Caucasian melanomas. However, there is a lack of study evaluating the status, and the clinical significance, of BRAF mutation in the Asian population. OBJECTIVE: This study was aimed to determine the frequency of BRAF mutation, and to evaluate the correlation of BRAF status with clinicopathologic features and outcomes, in Korean primary acral lentiginous melanoma (ALM) patients. METHODS: ALM samples (n=36) were analyzed for the BRAF V600E mutation, by dual-priming oligonucleotide (DPO) based real-time polymerase chain reaction. The clinicopathologic features and prognosis of the patients were analyzed with BRAF mutation status. RESULTS: The incidence of BRAF V600E mutation was 19.4% (7/36). The BRAF V600E mutations were not associated with clinicopathologic features, except for the age factor. All of the BRAF-mutant patients survived without recurrence or metastasis, and have a better clinical outcome than BRAF wild-type patients. CONCLUSION: In Korean primary ALM, a low frequency of BRAF mutation was shown; and BRAF mutation presented with a favorable prognosis. These results indicate that other distinctive genetic mechanisms may have more important roles in the development and progression of disease. Further multicenter study with large sample size is firmly needed, to confirm the results of our preliminary study.
Subject(s)
Humans , Age Factors , Asian People , Incidence , Melanoma , Neoplasm Metastasis , Prognosis , Real-Time Polymerase Chain Reaction , Recurrence , Sample Size摘要
BACKGROUND: In cutaneous malignant melanoma (MM) with clinically uninvolved regional lymph nodes, sentinel lymph node (SLN) status is the most powerful indicator of both overall survival (OS) and disease-free survival (DFS). However, no studies on the long-term survival and clinical follow-up of Korean patients with acral lentiginous MM (ALM) undergoing SLN biopsy (SLNB) have been published. OBJECTIVE: The purpose of this study was to investigate the clinical prognosis and long-term survival of Korean patients with ALM according to SLN status. METHODS: Thirty-four ALM patients undergoing SLNB were included in this study. We evaluated clinical and histopathological follow-up data such as the stage of disease, treatment, recurrence, and metastasis, and analyzed OS and DFS according to SLN status. RESULTS: The median follow-up time was 60.5 months (range 3~127 months). Positive SLNs were noted in 14 patients (41.2%). Patients with negative SLNs had better OS and DFS than those with positive SLNs (p<0.05). Increased Breslow thickness was associated with short OS and DFS (p<0.05), and female patients showed better DFS than male patients (p<0.05). CONCLUSION: To our knowledge, this is the first study on the long-term survival and clinical follow-up of patients undergoing SLNB for ALM in Korea. Our findings show that SLN status is an important prognostic factor for predicting OS and DFS.
Subject(s)
Female , Humans , Male , Biopsy , Disease-Free Survival , Follow-Up Studies , Korea , Lymph Nodes , Melanoma , Neoplasm Metastasis , Prognosis , Recurrence , Sentinel Lymph Node Biopsy , Survival Analysis摘要
BACKGROUND: Certain epidermal appendage tumors, including hyperplasias (hamartomas), adenomas, benign epitheliomas, primordial epitheliomas, and malignant tumors, can exhibit any stage of differentiation. Several molecules associated with tumorigenesis, such as Gli-1, pleckstrin homology-like domain, family A, member 1 (PHLDA-1), transforming growth factor (TGF)-beta1, TGF-beta2, and p63, are associated with tumor grade and aggressive behavior in follicular and sebaceous tumors in ways that are not well understood. OBJECTIVE: The aim of this study was to elucidate the expression of Gli-1, PHLDA-1, TGF-beta1/beta2, and p63 in benign and malignant tumors of the hair and sebaceous glands and to determine their importance in the degree of tumor differentiation. METHODS: Immunohistochemistry was performed in follicular and sebaceous tumors using antibodies against Gli-1 (sebaceous tumor marker), PHLDA-1 (hair follicle outer root sheath [ORS] cell marker), p63, TGF-beta1, and TGF-beta2. RESULTS: Gli-1 was expressed in basaloid cells, sebocytes, and sebaceous carcinoma cells, and expression levels decreased as differentiation progressed. PHLDA-1 was expressed in ORS cells and some follicular tumor cells. Expression of p63 was observed in the nuclei of the outermost basaloid cells (seboblasts), poorly differentiated sebaceous carcinoma cells, and tumor cells toward the direction of the hair. Remarkably, TGF-beta1 was expressed exclusively in the nuclei of benign and malignant follicular (hair) tumors, but not in sebaceous tumors, at levels that correlated with the degree of differentiation. CONCLUSION: We propose that p63 and/or TGF-beta1 are useful for predicting the degree of differentiation and malignant potential of sebaceous and follicular tumors and for distinguishing trichilemmal carcinoma from sebaceous carcinoma.