A Case of Rowell's syndrome / 대한피부과학회지

Rowell's syndrome is believed to be a distinct and rare clinical entity originally descrived as lupus erythematosus associated with erythema multiforme-like skin lesions, immunologic findings of speckled antinuclear antibodies, anti-La antibodies and a positive test for rheumatoid factor. We report a case of systemic lupus erythematosus in a 31-year-old woman with annular erythematous patches on both hands, feet and back. Also, she presented abnormal serologic and immunologic findings. We believed that our patient meets the criteria for this rarely reported entity.

Langerhans Cell Histiocytosis Presenting as a Solitary Nodule / 대한피부과학회지

Langerhans cell histiocytosis, previously called histocytosis X, is a disease of unknown cause characterized by the proliferation of a distinct cell type that is S-100 and CD1a positive and contains cytoplasmic langerhans granules. We report a case of langerhans cell histiocytosis, which a 10-year-old girl presented as a solitary nodule without systemic involvement. Histologic examination of the nodule showed a dense dermal infiltrate of histiocytic cells with bean-shaped nuclei. These cells reacted with antibodies to S-100 and CD1a. Ultrastructural analysis showed Birbeck granules in the cytoplasm of the histiocytic cells. We confirmed langerhans cell histicytosis presenting as a solitary nodule by above findings.

Langerhans Cell Histiocytosis Presenting as a Solitary Nodule / 대한피부과학회지

Langerhans cell histiocytosis, previously called histocytosis X, is a disease of unknown cause characterized by the proliferation of a distinct cell type that is S-100 and CD1a positive and contains cytoplasmic langerhans granules. We report a case of langerhans cell histiocytosis, which a 10-year-old girl presented as a solitary nodule without systemic involvement. Histologic examination of the nodule showed a dense dermal infiltrate of histiocytic cells with bean-shaped nuclei. These cells reacted with antibodies to S-100 and CD1a. Ultrastructural analysis showed Birbeck granules in the cytoplasm of the histiocytic cells. We confirmed langerhans cell histicytosis presenting as a solitary nodule by above findings.

Acute Generalized Exanthematous Pustulosis / 대한피부과학회지

Acute generalized exanthematous pustulosis(AGEP) presents with the abrupt onset of a wide spread pustules on an erythematous base and rapid spontaneous healing. The most possible causes of AGEP seem to be antibiotics, viral infection and exposure to mercury. We report five cases of acute generalized exanthematous pustulosis, all of which presented wide spreaded tiny pustules on an erythematous base. Histopathologic examination showed that there are subcorneal pustules, rete ridge elongations and perivascular infiltration. We suspected that the possible causes of five cases were pharyngitis and various drugs each.

A Case of Trichilemmal Cyst on the Leg / 대한피부과학회지

Trichilemmal cyst is known to be a kind of keratinous cyst with lining cells showing trichilemmal keratinization. About 90% of trichilemmal cysts occur on the scalp. They are only rarely found on the face, neck and trunk. A 25-year-old man had a skin-colored nontender nodule on his left leg. Clinically, we were considered epidermal cyst. However, histopathologic examination showed that there was abrupt keratinization without granular layer, which was compatible with those of trichilemmal cyst.

A Case of Letterer-Siwe Disease in Adult / 대한피부과학회지

Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy, localized bone defect, and fatal outcome. We report a 38-year-old woman with hepatomegaly, lymphadenopathy, scaly papules and petechiae on the trunk. The eletronmicroscopy showed a tennis racquet shaped Birbeck granules in cytoplasm of histiocyte. Systemic treatment with etoposide, cyclophosphamide, prednisone, and vincristine was effective.

A Case of Job's Syndrome / 대한피부과학회지

The Job's syndrome is a relatively rare primary immunodeficiency disorder characterized by recurrent staphylococcal infection and abscess formation, defective neutrophil chemotaxis, and markedly elevated serum IgE level. Clinical features are atopiclike dermatitis, furunculosis, paronychia, pulmonary bacterial pneumonia etc. We reported a case of Job's syndrome in a 12-year-old girl who had recurrent pneumonia, scaly pruritic dermatitis, fissured tongue, and nail dystrophy with elevated serum IgE level.

A Case of Pyoderma Gangrenosum Occurring in Behcet's Disease / 대한피부과학회지

Pyoderma gangrenosum occuring in Behcet's disease have been rarely reported, but there are several clinical and histological similarities between Behcet's disease and pyoderma gangrenosum. We report the case of pyoderma gangrenosum occuring in Behcet's disease and describe the similarities of these diseases.

Expression of Intercellular Adhesion Molecule-l (ICAM-1) in Vascular Endothelium and Keratinocytes of Psoriatic Skin

BACKGROUND: The endothelial expresson and upregulation of ICAM-1 and epidermal keratinocyte expression of ICAM-1 are well documented in psoriasis. ICAM-1 mediates the adhesion and trafficking of circulating activated skin-seeking CD45RO+ memory CD4+ T lymphocytes from the vessel into the dermis and epidermis of psoriatic skin by binding to its ligand LFA-1(lymphocyte function-associated antigen-1) expressed on lymphocyte membranes. OBJECTIVE: The purpose of this study was to investigate the expression of ICAM-1 in vascular endothelium and keratinocyte of psoriatic skin and the difference of ICAM-1 expression between early and fully developed psoriatic lesions. METHODS: We have studied the expression of ICAM-1 in twelve psoriatic patients who had not been treated for psoriatic lesions for 1 month and three normal human skin samples by immunohistochemical staining using monoclonal antibody against ICAM-1. RESULTS: Immunohistochemical staining revealed anti-ICAM-1 antibody positively stained only in the subpapillary endothelial cells of normal skin. But in all psoriatic lesions studied, anti- ICAM-1 antibody was stained positively in the endothelium of papillary and subpapillary plexus, and in fully developed psoriatic lesions, anti-ICAM-1 antibody was stained focally in epidermal keratinocytes. CONCLUSION: The results suggest that ICAM-1 expression on papillary microvessels and keratinocytes may play an important role in the transendothelial and transepidermal migration of lymphocytes from the vessel into the dermis and epidermis of psoriatic skin.

Immunohistochemical Study of TGF- type I and type II receptor Expression in Psoriatic Epidermis / 대한피부과학회지

BACKGROUND: Previous studies have demonstrated the pathogenetic role and expression of TGF-beta in psoriatic lesion. Transforming growth factor s are a family of growth factors with inhibitory effects on epithelial cell proliferation. Their effects are mediated by two interacting receptors, of which type I receptor mediates signal transduction after interaction with type II receptor carrying the TGF ligand. OBJECTIVE: The purpose of this study was to investigate the relationship between development of psoriasis and expression of TGF-beta receptors in psoriatic lesion. METHODS: We have studied the expression of TGF-beta type I and type II receptors in psoriatic lesions of 30 psoriatic patients who had not been treated for 1 month, 5 non-lesional psoriatic skin, and 3 normal human skin by immunohistochemical staining using polyclonal rabit antisera. RESULTS: 1. Immunohistochemical analysis revealed an intense immunoreactivity for TGF-beta type I and type II receptors in the basal and also suprabasal layer of normal epidermis and non-lesional psoriatic skin. 2. Almost all psoriatic lesions studied lacked detectable immunoreactivity of either receptor in the epidermis. CONCLUSION: We suggest the lack of TGF-beta - mediated growth inhibition by down regulation of TGF-beta receptor expression may play an important role in the pathogenesis of psoriasis.

A Case of Eccrine Poroma with A Large Cystic Space / 대한피부과학회지

Eccrine poroma is a benign skin appendage tumor originating from the intradermal part of the sweat duct. It is found commonly on the hairless surface of the feet, although eccrine poroma of the hands, head, trunk, and legs have been reported. Histopathologically, the tumor extends from the lower portion of the epidermis into the dermis as broad, anastomosing bands. The tumor cells are smaller than squamous cells, having a uniform cuboidal appearance and a round, deeply basophilic nucleus, connected by intercellular bridges. In eccrine poromas, narrow ducta1 lumina and occasional cystic spaces are found within the tumor bands, but a large cystic space is very unusual. We report a case of eccrine poroma with a large cystic space extending from epidermis into the reticular dermis.

A Case of Intravenous Pyogenic Granuloma / 대한피부과학회지

An intravenous pyogenic granuloma is a rare, benign, intravascular tumor, which arises from the vein wall and protrudes into the lumen. This is characterized by a lobular proliferation of capillaries similar to the more common cutaneous pyogenic granulomas. We report a case of intravenous pyogenic granuloma which showed lobular capillary proliferation in the perivenous connective tissue.

A Case of Prurigo Pigmentosa Associated with Ketosis / 대한피부과학회지

Prurigo pigmentosa is a distinct type of inflammatory dermatosis characterized by recurrent pruritic erythematous papules that resolve, leaving a peculiar, reticulate hyperpigmentation. Although the cause of prurigo pigmentosa is unknown, exogenous factors, such as friction from clothing, have been suggested as possible triggers in some cases. In other cases, however, systemic conditions such as diabetes mellitus, pregnancy, fasting, and dieting were associated with prurigo pigmentosa, suggesting involvement of endogenous factors in the pathogenesis. There have been reported cases of prurigo pigmentosa associated with ketosis. Because ketosis is commonly observed in association with fasting, dieting, and insulin dependent diabetes melitus, it may be involved in the pathogenesis of prurigo pigmentosa. Here, we describe a patient with prurigo pigmentosa associated with diabetes mellitus and ketosis. In this case, the prurigo pigmentosa improved with the disappearance of the ketosis, but the blood sugar levels did not correlate with the improvement.

Efficacy of Itraconazole Melt-Extrusion Tablet One-week Therapy in Treatment of Hyperkeratotic Type of Tinea Pedis and/or Tinea Manus / 대한피부과학회지

BACKGROUND: Since the bioavailability of itraconazole capsule is influenced by patients gastric acidity, it results in treatment failure due to its low dissolution and subsequent low absorption when administered in fasting. Itraconazole Melt-Extrusion tablet has been lately developed in order to improve its dissolution profile. It is the first clinical study to evaluate the efficacy and safety of itraconazole Melt-Extrusion tablet in Korea. OBJECTIVE: This study was conducted to evaluate the efficacy and safety of itraconazole melt-extrusion tablet 400mg daily for 1 week(pulse therapy) for hyperkeratotic type of tinea pedis and manus. METHODS: A clinical and mycological investigation was made of 812 outpatients with hyperkeratotic type of tinea pedis and/or tinea manus who had visited at 52 general hospitals under the lead of the Korean Dermatological Association from June to December, 1998. Patients confirmed by clinically and microscopically as hyperkeratotic type of tinea pedis and/or tinea manus were administered 2 tablets twice a day for one week and followed up for 8 weeks from the start of the medication. RESULTS: The results were summarized as follows; 1. Clinical symptoms of hyperkeratotic type of tinea pedis and/or tinea mauns were significantly improved at the end of study, week 8(p<0.001). 2. Clinical response rate, defined as more than 50% decrease of the sum of the clinical symptom scores, was 79.3%(512/646). 3. Mycological cure rate, dafined as both culture and KOH negative at week 8, was 78.2%(244 /312). 4. 40(5.5%) patients, of the 727 patients evaluable for drug safety evaluation, were reported to have adverse event. CONCLUSION: Itraconazole Melt-Extrusion tablet 400mg/day for 1 week (pulse therapy) is effective and safe in the treatment of hyperkeratotic type of tinea pedis and/or tinea manus.

A Case of Segmental Neurofibromatosis

Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait spots and neurofibrobmas, or only neurofibroma, limited to one region of the body. Disease-associated systemic involvement is uncommon. Most patients with segmental neurofibromatosis do not have a family history of neurofibromatosis. In Korea, there have been 3 reported cases of segmental neurofibromatosis. None of them had caf6-au-fait spots, systemic involvement, or family history. This report describes a case of segmental neurofibromatosis in a 25-year-old woman who had Becker's nevus-like cafe-au-lait spots and this was linked to the presence of Fanconi's syndrome in a second degree relative of the patient.

Bullous Pemphigoid with Peripheral Blood Hypereosinophilia / 대한피부과학회지

Bullous pemphigoid(BP) is a bullous disease in elderly people characterized by subepidermal bullae on erythematous and normal skin. Peripheral blood easinophilia have been reported in the patients with BP, and blood eosinophilia may be related to disease activity and severity in BP. We report a 70-year old man BP. He showed peripheral blood eosinophilia, and was treated successfully with a combination of low dose steroids & tetracycline-niacinamide(T-N) therapy. The eosinophil counts fell to normal levels as the skin lesion cleared.

The Effects of Ultraviolet Radiation on Aging and p53 Expression in Human Skin / 대한피부과학회지

BACKGROUND: Long-term phototherapy can induce the changes of photoaging and it is reported that there is an increased chance of cutaneous squamous cell carcinomas in patients exposed to large amounts of UV radiation. OBJECTIVE: The main objective was to investigate the degree of photoaging and the presence of p53 mutations in normal skin in patients undergoing long-term phototherapy. METHOD: We performed hematoxylin-eosin and special stains, p53 and p21 immunohistochemical stains and polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) on the normal skin of patients subject to long-term UV therapy. RESULT: 1. The typical features of photoaging were not observed in patients undergoing long-term UV therapy. 2. In p53 immunohistochemical staining performed at 1 week after cessation of long-term PUVA treatment, the patient group with a culmulated UV dosage of more than 1,000J/cm2 demonstrated an increased number of p53 positive epidermal cells compared to exposed as well as unexposed normal skins. 3. The patterns of p21 immunohistochemical staining performed at 1 week after cessation of long-term PUVA and UVB treatments were similar to that of p53 immunohistochemical staining performed at 1 week after cessation of phototherapy. 4. In p53 immunohistochemical staining performed at 4 months after cessation of UV treatment, the number of p53 positive epidermal cells decreased significantly compared to that of p53 positive epidermal cells found at 1 week after cessation of UV treatment. 5. The mutation of p53 genes was not found in PCR-SSCP analysis of biopsied skins done at 1 week after cessation of long-term PUVA and UVB treatment. CONCLUSION: Long-term phototherapy did not induce the typical changes of photoaging and p53 overexpression in the epidermis of UV treated skin was a reactive process. Therefore, UV therapy can be a relatively safe treatment modality, although a closer observation for cutaneous malignancy is warrented in the patients whose cumulated UV dosage is much higher than 1,000J/cm2.

Assessment of Quality of Life in Patient with Toenail Onychomycosis in Korea / 대한의진균학회지

BACKGROUND: Onychomycosis, especially toenail onychomycosis has become one of the common fungal infection and has historically been regarded as a cosmetic rather than medical problem by many patients, even by physicians. Recently, however, there are several reports that this is a refractory disease which may cause a deleterious effect on patients' quality of life (QOL). OBJECTIVE: The purpose of this study was to investigate the impact of toenail onychomycosis on QOL in Korea and to assess the changes of QOL after treatment. METHODS: Total 1004 patients with toenail onychomycosis which was confirmed by clinical findings and KOH preparation were enrolled at 47 dermatologic centers in Korea, and interviewed with standardized QOL questionnaire before and after systemic antifungal treatment. Responses to the questionnaire were scored by f-point scale (0~4) and averaged, and were analyzed for 5 dimensions of emotional impact, social impact, symptom and functional impact, patients' views concerning treatment, and relationship with doctor. RESULTS: 1. Before and after treatment, the most serious impact was emotional dimension showing 1.90 and 1.30 in average score (AS), and social (AS: 1.14 and 0.83) and symptom and functional impact (AS: 1.05 and 0.92) was also affected. 2. In female rather than male, statistically more significant impact on patients' QOL was observed in all dimensions. 3. After treatment, 3 of 5 dimensions were improved significantly - emotional dimension (AS: from 1.90 to 1.30), social dimension (AS: from 1.14 to 0.83), patients' view concerning treatment(AS: from 1.34 to 1.02) 4. The degree of patients' satisfaction at the therapeutic effect was very high - 62.4% (immediately after. treatment) and 65.8% (9 months after initiation of treatment) of patients answered excellent or good. CONCLUSION: This study confirms that toenail onychomycosis has significant Impact on the overall QOL of patients. Also the effect of antifungal therapy on patients' QOL were satisfactory. Therefore, both doctor and patient should pay more attention to the treatment of onychomycosis.

A Case of Sjogren-Larsson Syndrome / 대한피부과학회지

Sjogren-Larsson syndrome is a rare autosomal recessive neurocutaneous disorder due to a deficiency of the fatty aldehyde dehydrogenase. It consists of ichthyosis, spastic di- or quardriplegia and mental retardation. We report a case of a 9-year-old boy who was suspected to have Sjogren-Larsson syndrome. He had congenital ichthyosis, spastic diplegia and mental retardation. In addition, there was macular degeneration on the fundus. His neurological symptoms were improved after selective posterior rhizotomy and rehabilitation therapy. However, the dermatological problem did not subside with conservative treatment.

The Rate of Recovery after Disruption of the Skin Barrier and the composition of Human Surface Lipids at Different Anatomic Sites of Skin / 대한피부과학회지

BACKGROUND: Lipids in the stratum corneum which acts as a barrier prevents transepidermal water loss and percutaneous absorption of a substance differs at different anatomic sites of skin. No study has yet been done on the recovery rate of the skin barrier after its destruction in relation to anatomic sites. OBJECTIVES: The purpose of this study was to measure the recovery rate of the skin barrier after its disruption in relation to anatomic sites and also to examine the lipid composition of skin surface lipids at different anatomic sites of skin. METHODS: The study population consisted of 20 healthy male subjects. We used 3M Scotch tapes to remove the stratum corneum and examined the recovery rate by measuring the TEWL using an evaporimeter after 2.5, 6, 10, 24, 48 and 72 hours at eight different anatomic sites consisting of the post-auricular area, chest, abdomen, back, forearm, thigh and calf. The skin surface lipids were extracted using 99% ethanol at ten different anatomic sites including the face, chest, abdomen, back, upper arm, forearm, palm, thigh, shin and sole. The composition of skin surface lipids was determined by thin layer chromatography. RESULTS: 1. The transepidermal water loss was highest in the post-auricular area and ahdomen, back, thigh, forearm, upper arm, thieh and chest in a decreasing order, although there was no statistical significance(p>0.05). 2. The recovery rate was highest in the post-auricular area after 2.5 hours with a statistical significance compared to other sites except for the abdomen(p<0.05). 3. The recovery rate was also highest in the post-auricular area after 6, 10, 24, 48 and 72hrs compared with other sites with a statistical significance (p<0.01). 4. Lipid analysis of ten anatomic sites demonstrated that the concentration of cholesterol sulfate was highest on the face, that of cholesterol was highest on the back and that of sphingolipid was highest on the abdomen but there was no statistical significance. CONCLUSION: The post-auricular area showed the fastest recovery rate after its barrier disruption which can be applied in the percutaneous absorption of substances. Also the composition of human skin surface lipids, especially epidermal lipids at different anatomic sites was not statistically different.