ABSTRACT
Xanthomas are localized lipid deposits within organs that may manifest as papules, plaques, or nodules in skin. They are commonly associated with all types of hyperlipidemia. Xanthoma striatum palmare characterized by xanthomas of the palmar creases is a rare but important diagnostic physical sign of dysbetalipoproteinemia, also known as type III hyperlipoproteinemia. Type III hyperlipoproteinemia is characterized by the early onset of cardiovascular disease and peripheral vascular disease. We describe herein the case of a 51-year-old female patient affected by xanthoma striatum palmare associated with elevated plasma levels of triglycerides and cholesterol and a lipoprotein electrophoresis pattern consistent with type III hyperlipoproteinemia.
Subject(s)
Female , Humans , Middle Aged , Cardiovascular Diseases , Cholesterol , Electrophoresis , Hyperlipidemias , Hyperlipoproteinemia Type III , Lipoproteins , Peripheral Vascular Diseases , Plasma , Skin , Triglycerides , XanthomatosisABSTRACT
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 SyndromeABSTRACT
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 CellsABSTRACT
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 , VasculitisABSTRACT
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 , UlcerABSTRACT
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 NeoplasmsABSTRACT
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 PunctureABSTRACT
No abstract available.
Subject(s)
Humans , Colon , Colonic Neoplasms , Sparganosis , UltrasonographyABSTRACT
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is characterized histopathologically by the presence of granulomatous inflammation with or without leukocytoclastic vasculitis. It has been associated with multiple systemic diseases, particularly autoimmune conditions like rheumatoid arthritis and Behcet's disease. We report a 52-year-old woman with rheumatoid arthritis who presented with a single, non-tender, rapidly growing nodule on her right elbow. Histologic examination of the nodule revealed degenerated collagen, neutrophils, neutrophilic dust, fibrin, and mucin present in the centers of the palisades of histiocytes throughout the dermis. Herein, we report the abrupt onset of PNGD in a patient with rheumatoid arthritis.