Cutaneous leishmaniasis is a skin infection caused by the Leishmania species, an intracellular protozoan parasite that is transmitted by various species of female sandflies. According to the geographic distribution and vectors, leishmaniasis is classified as Old World or New World cutaneous leishmaniasis. In Korea, 24 cases of Old World cutaneous leishmaniasis have been reported, but New World cutaneous leishmaniasis has not been reported as yet. A 37-year-old man presented with a 3-month history of a painful and erythematous nodule with two satellite papules on the left postauricular area and a papule on the left arm after traveling to the Amazon region in Brazil. After we performed skin biopsies of the lesions, diagnosis of cutaneous leishmaniasis was made by the histopathological findings. After intralesional injection of sodium stibogluconate (Pentostam®, GlaxoSmithKline) twice a week for 4 weeks, the lesions improved with scarring. Herein, we discuss this case of New World cutaneous leishmaniasis that was successfully treated with intralesional injection of sodium stibogluconate (Pentostam®) in Korea.
Cutaneous leishmaniasis; Leishmaniasis; Sodium stibogluconate (Pentostam®)
Acrodermatitis enteropathica (AE) is an uncommon autosomal recessive genetic disorder of zinc malabsorption. The acquired form may be associated with inadequate intake, impaired absorption, and increased excretion of zinc. Those afflicted present with diarrhea, stomatitis, psychiatric symptoms, non-scarring alopecia, and nail dystrophy accompanied by erythematous which appears as scaly patches with erosion vesicles and pustules mostly affecting the extremities, perineal, and periorificial areas. Due to the variable findings of most case reports, the clinical and histopathological features of AE are often regarded as non-specific. We report an unusual case of bullous AE secondary to total parenteral nutrition for the treatment of acute pancreatitis occurring in a six-year-old male with acute lymphocytic leukemia who underwent chemotherapy. He presented with periorificial, reddish, eroded bullae with multiple vesicles and blisters on his fingers, toes, and buttock, showing necrotic keratinocytes with multiple intraepidermal vesicles and perivascular infiltration with predominant lymphocytes and few neutrophils within the dermis. To the best of our knowledge, this is the first case report of bullous AE in the Korean dermatologic literature.
Bullous acrodermatitis enteropathica; Chemotherapy; Total parenteral nutrition
Pincer nail is a nail deformity characterized by transverse overcurvature of the nail plate. Pincer nail can affect a patient's quality of life due to its chronic, recurrent course; however, there have been no clinical studies on the pincer nail condition in Korean patients.
The purpose of this study was to characterize the clinical findings and treatment of pincer nail. In addition, possible etiological factors were considered, and treatment efficacy was evaluated.
The medical records and clinical photographs of 35 patients (12 males, 23 females) who were diagnosed with pincer nail between August 1, 2005 and July 31, 2009 were studied.
Patient age ranged from 10 to 77 (52.09±17.26) years, and there was a predominance of female (23 out of 35 patients, F:M=2:1). The mean duration of the disorder was 7.45 years (range 0.25~40); 85% had pincer nail for at least 1 year. In addition, 40% had a history of previous treatment and recurrence. There were 82.8% patients with the common type of pincer nails. The most commonly involved nails were both great toenails. Among 35 patients, nail grinding was started in 30 patients, and 25 patients showed clinical improvement with nail grinding. The width index increased and the height index decreased after treatment. The mean follow up period was 8.42 months (range 1~27), and 7 patients showed recurrence after 8.8 months (range 2~20). Among 35 patients, 5 patients were treated with nail extraction with matricectomy, and the symptoms resolved immediately. The mean follow up period was 7.6 months (range 0~19), and recurrence was not observed. Onychomycosis was also present in 37.1% of patients, and itraconazole pulse therapy for 3 months was added.
The results of this study demonstrate the clinical features of pincer nail in Korean patients. The findings show that the common type of pincer nail was most common, and nail grinding as a conservative treatment greatly improved pincer nails despite a risk of recurrence. When onychomycosis was also present, oral antifungal therapy added to nail grinding resulted in a more rapid change in nail thickness and clinical improvement.
Nail grinding; Onychomycosis; Pincer nail
A 58-year-old man presented with whitish patches on both great toenails for four weeks prior to visiting our hospital; the patches spread rapidly to other finger- and toe-nails. Prior to presentation, the patient had been diagnosed with idiopathic thrombocytopenic purpura two months ago and Kaposi's sarcoma three weeks ago. The patient was treated with human immunoglobulin for five days, and then received prednisolone 40 mg bid. Serology showed that the patient was negative for HIV and results of other laboratory tests were normal. The KOH slide preparation of the nail scraping showed long septated hyphae and numerous arthrospores. The fungus culture revealed whitish downy colonies on the front side and wine-red reverse pigmentation on Sabouraud's dextrose agar. Trichophyton rubrum was isolated on fungus culture and slide culture. The internal transcribed space (ITS) regions of ribosomal DNA of the cultured fungus were identical to Trichophyton rubrum. Proximal subungual onychomycosis (PSO) is the rarest form of onychomycosis. PSO initially presents as whitish patch(es) on the proximal side of the nail plate(s). Because PSO shows whitish to yellowish patches on the nail plate, the result of KOH examination of nail scrapings from the nail plate is almost always negative. Herein, we report on a case of multiple PSO in a patient with classic Kaposi sarcoma and suggest a method for easy KOH scraping on PSO.
Classic kaposi sarcoma; Proximal subungual onychomycosis; Trichophyton rubrum
Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP. However, this transformation rarely occurs in patients younger than 20 years of age. Here, we present the first known pediatric patient in Korea, a 12-year-old boy who developed a subcutaneous nodule on the scrotum 13 months after papulonecrotic lesions of LyP were identified on both lower extremities and face. Histological and immunohistochemical examination of the subcutaneous nodule revealed anaplastic large cell lymphoma (ALCL). A T-cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in the two specimens, indicating that a common T-cell clone had proliferated over time in both the LyP and ALCL lesions.
Anaplastic large cell lymphoma; Lymphomatoid papulosis
Localized involutional lipoatrophy (LIL) is a rare distinctive idiopathic form of localized lipoatrophy. The characteristic features in histopathology of LIL are diminutive fat lobules composed of small adipocyte resembling fetal fat tissue. LIL is not a well-known disorder, there have been only a few reports on LIL in the English literature. We herein report 2 cases of LIL and review the previously published cases.
Idiopathic; Involutional; Lipoatrophy; Localized
Porokeratosis is an abnormal disease of keratinization of epidermis. It is clinically characterized by margins covered with keratin layer and it typically has an atrophied macule with a protruded, circular form. Histopathologically, it shows the findings of cornoid lamella. Risk factors for its development include organ transplantation, long-term use of corticosteroids, immunocompromised status, including AIDS, and exposure to ultraviolet light. We herein report a case of atypical porokeratosis in a 38-year-old man who developed porokeratosis involving multiple sites following bone marrow transplantation for myelodysplastic syndrome.
Bone marrow transplantation; Myelodysplastic syndrome; Porokeratosis
Kaposi sarcoma (KS) is a multifocal vascular neoplasm most commonly seen in association with human immunodeficiency virus infection. However, KS has also been reported in patients treated with immunosuppressive agents. However, it is very rare to find KS in association with idiopathic thrombocytopenic purpura (ITP). Here we describe a 58-year-old male patient suffering from ITP treated with corticosteroid therapy who developed KS and present a review of the literature.
Corticosteroid; Idiopathic thrombocytopenic purpura; Kaposi sarcoma
Graft versus host disease (GVHD) has traditionally been divided into acute GVHD and chronic GVHD based on the period it occurs after transplantation. Chronic cutaneous GVHD has traditionally been classified into the lichenoid and scleroderma-like forms. However, unusual clinical forms have been reported such as dermatomyositis, lupus erythematosus and exfoliative dermatitis. A 35-year-old woman presented with a 2 week history of a pruritic maculopapular rash on the whole body. The rash rapidly progressed to confluent erythematous scaly patches and plaques with micaceous scales and this finally led to a generalized exfoliative dermatitis in a 1 month period. Here we present an unusual case of chronic cutaneous GVHD with the clinical features of exfoliative dermatitis. The histopathologic examination demonstrated the lichenoid features of chronic cutaneous GVHD.
Chronic graft versus host disease; Exfoliative dermatitis
Leukemia cutis is the cutaneous involvement of leukemic neoplastic cells. It is an uncommon feature of systemic leukemia, and is associated with a poor prognosis. A 20-year-old man presented with a solitary, 2 cm domeshaped, firm, erythematous nodule on the right medial malleolus that was present for 3 months. The patient had a history of venous catheter insertion at the right medial malleolus area 3 months previously. The history was significant for acute lymphoblastic leukemia (ALL) for 4 years; an allogenic bone marrow transplantation was performed 3 years earlier. The histologc examination of the lesion revealed diffuse infiltration of leukemic cells in the dermis and subcutis. Herein we report a case of leukemia cutis at the site of a prior catheter insertion in a patient with ALL.
Acute lymphocytic leukemia; Leukemia cutis; Site of catheter insertion
We report here on a 63-year-old woman who had several small, yellowish papules on the scalp for the previous 2 years. There was no family history of similar lesions. Yellowish, creamy material was expressed from a papule during punch biopsy. Histologic examination from the lesion revealed the typical features of steatocystoma multiplex. We report here on this rare variant of steatocystoma multiplex that was limited to the scalp.
Scalp; Steatocystoma multiplex
Contact with coral may cause a rare type of contact dermatitis, and the resulting skin reaction can be divided into different reactions: the acute, delayed and chronic types of coelenterate dermatitis. Granulomas of delayed skin reactions have rarely been reported. Herein, we report on a rare case of a delayed reaction of the skin to coral injury, and the patient displayed superficial granulomas and atypical CD30+ lymphocytes.
Contact dermatitis; Coral; Granuloma
Cholinergic urticaria is a type of physical urticaria characterized by heat-associated wheals. Several reports are available about cholinergic urticaria; however, the clinical manifestations and pathogenesis are incompletely understood.
The purpose of this study was to investigate the clinical characteristics of cholinergic urticaria in Korea.
We performed a retrospective study of 92 patients with cholinergic urticaria who were contacted by phone and whose diagnoses were confirmed by the exercise provocation test among those who had visited The Catholic University of Korea, Catholic Medical Center from January 2001 to November 2010.
All 92 patients were male, and their average age was 27.8 years (range, 17~51 years). Most of the patients had onset of the disease in their 20s and 30s. Non-follicular wheals were located on the trunk and upper extremities of many patients, and the symptoms were aggravated by exercise. Eight patients showed general urticaria symptoms and 15 had accompanying atopic disease. Forty-three patients complained of seasonal aggravation. Most patients were treated with first and second-generation antihistamines.
Dermatologists should consider these characteristics in patients with cholinergic urticaria. Further investigation and follow-up studies are necessary to better understand the epidemiological and clinical findings of cholinergic urticaria.
Cholinergic; Epidemiology; Phenotype; Signs and symptoms; Urticaria
Epithelial keratinocyte proliferation is an essential element of wound repair, and abnormal epithelial proliferation is an intrinsic element in the skin disorder psoriasis. The factors that trigger epithelial proliferation in these inflammatory processes are incompletely understood. Here we have shown that regenerating islet-derived protein 3-alpha (REG3A) is highly expressed in keratinocytes during psoriasis and wound repair and in imiquimod-induced psoriatic skin lesions. The expression of REG3A by kerati-nocytes is induced by interleukin-17 (IL-17) via activation of keratinocyte-encoded IL-17 receptor A (IL-17RA) and feeds back on keratinocytes to inhibit terminal differentiation and increase cell proliferation by binding to exostosin-like 3 (EXTL3) followed by activation of phosphatidylinositol 3 kinase (PI3K) and the kinase AKT. These findings reveal that REG3A, a secreted intestinal antimicrobial protein, can promote skin keratinocyte proliferation and can be induced by IL-17. This observation suggests that REG3A may mediate the epidermal hyperproliferation observed in normal wound repair and in psoriasis.
The incidence of overall cancer has increased over time. The incidence of top-ranking cancers has changed in the 1990s and the 2000s. However, few studies have evaluated the trends in metastatic skin cancers during this period. We evaluated the recent trends in incidence, peak age and location of metastatic skin cancers from 1991 to 2010. This 20-yr survey was divided into two decades to determine the trends by comparing the statistics. Out of 694,466 outpatients (1991-2010), 174 (0.025%) were diagnosed with metastatic skin cancer. The incidence of metastatic skin cancer increased significantly from 20.64 per 100,000 outpatients in the 1990s to 28.70 per 100,000 outpatients in the 2000s (P = 0.030). The peak age of skin metastasis shifted from the 40s to the 50s in women, and from the 50s to the 60s in men. The percentage of metastatic skin cancers originating from intra-abdominal organs increased from 10% in the 1990s to 23.1% in the 2000s (P = 0.027). The percentage of metastatic skin cancers located on the abdomen increased from 7.1% in the 1990s to 15.4% in the 2000s (P = 0.011). The higher proportion of metastatic skin cancers located on the abdomen may be related to the increase in skin metastases from intra-abdominal organs.
Metastatic Skin Cancers Incidence; Abdomen; Intra-Abdominal Organs
Leukemia cutis (LC) is defined as a neoplastic leukocytic infiltration of the skin. Few clinical studies are available on recent trends of LC in Korea. The purpose of this study was to analyze the clinical features and prognosis of LC in Korea and to compare findings with previous studies. We performed a retrospective study of 75 patients with LC and evaluated the patients' age and sex, clinical features and skin lesion distribution according to the type of leukemia, interval between the diagnosis of leukemia and the development of LC, and prognosis. The male to female ratio was 2:1, and the mean age at diagnosis was 37.6 yr. The most common cutaneous lesions were nodules. The most commonly affected site was the extremities in acute myelocytic leukemia and chronic myelocytic leukemia except for acute lymphocytic leukemia. Compared with previous studies, there was an increasing tendency in the proportion of males and nodular lesions, and LC most often occurred in the extremities. The prognosis of LC was still poor within 1 yr, which was similar to the results of previous studies. These results suggest that there is a difference in the clinical characteristics and predilection sites according to type of leukemia.
Clinical Characteristics; Leukemia Cutis; Prognosis
The pathogenesis of psoriasis may involve the interleukin (IL)-23 and Th17-mediated immune responses. Th17 cells secret IL-17 and IL-22, which mediates dermal inflammation and acanthosis.
As inhibitor of nuclear factor κB kinase-α (IKKα) has been previously identified as a primary regulator of keratinocyte differentiation and proliferation, we proposed that IL-17 and IL-22 might affect keratinocyte differentiation by changing the expression of IKKα.
We employed HaCaT cells
maintained culture medium at a low calcium concentration (0.06 mM) and induced differentiation by switching to the high concentration (2.8 mM) media with IL-17 or IL-22, then compared the IKKα expression and the cell cycle. We employed reconstituted human epidermal skin (Neoderm) and mice ears for the in vivo studies.
calcium concentration induced IKKα expression and terminal differentiation with cell cycle arrest in HaCaT cell cultures. Moreover, IL-17 and IL-22 treatment also induced IKKα in HaCaT cells and reconstituted human epidermis. IKKα induction was also noted, following the injection of IL-17 and IL-22 into mice ears.
Although the induction of IKKα was accompanied by keratinocyte differentiation, IL-17 and IL-22 did not affect calcium-mediated differentiation or the cell cycle. Rather, IL-17 and IL-22 appear to contribute to the inflammation occurring via the induction of IKKα from keratinocytes or skin layers.
Cell differentiation; IKK alpha; Inflammation; Interleukin-17; Interleukin-22; Keratinocytes
To evaluate the safety and technical success rate of an ultrasound-guided fiducial marker implantation in preparation for CyberKnife radiation therapy.
Materials and Methods
We retrospectively reviewed 270 percutaneous ultrasound-guided fiducial marker implantations in 77 patients, which were performed from June 2008 through March 2011. Of 270 implantations, 104 were implanted in metastatic lymph nodes, 96 were in the liver, 39 were in the pancreas, and 31 were in the prostate. During and after the implantation, major and minor procedure-related complications were documented. We defined technical success as the implantation enabling adequate treatment planning and CT simulation.
The major and minor complication rates were 1% and 21%, respectively. One patient who had an implantation in the liver suffered severe abdominal pain, biloma, and pleural effusion, which were considered as major complication. Abdominal pain was the most common complication in 11 patients (14%). Among nine patients who had markers inserted in the prostate, one had transient hematuria for less than 24 hours, and the other experienced transient voiding difficulty. Of the 270 implantations, 261 were successful (97%). The reasons for unsuccessful implantations included migration of fiducial markers (five implantations, 2%) and failure to discriminate the fiducial markers (three implantations, 1%). Among the unsuccessful implantation cases, six patients required additional procedures (8%).
The symptomatic complications following ultrasound-guided percutaneous implantation of fiducial markers are relatively low. However, careful consideration of the relatively higher rate of migration and discrimination failure is needed when performing ultrasound-guided percutaneous implantations of fiducial markers.
Fiducial marker; Radiation oncology; Ultrasonography; interventional; Imaging-guided radiation therapy
In this report, we describe a Korean patient with May-Hegglin anomaly from a mutation of the MYH9 gene. The proband was a 21-year-old man with thrombocytopenia. He did not have a bleeding tendency. His neutrophil count was normal at 7490/mm3; however, the neutrophils contained abnormal basophilic inclusions in their cytoplasm. The platelet count was decreased at 15000/mm3 with giant platelets. Coagulation test results were not remarkable. Direct sequencing of MYH9 revealed that he was heterozygous for a mutation in exon 1, which was a 97T>A substitution mutation affecting codon 33, substituting tryptophan with arginine (Trp33Arg). Family study showed that both of his parents had normal phenotype and genotypes, indicating a de novo occurrence of the mutation in the proband.
May-Hegglin anomaly; MYH9; thrombocytopenia; Korean