No abstract available.

Hailey-Hailey disease(benign familial chronic pemphigus) is a rare autosomal dominant disorder characterized by blisters at sites of friction such as the neck, axillae and groin which are caused by suprabasal epidermal acantholysis. We report two cases of Hailey-Hailey disease in the one family. One of the two cases has asymptomatic multiple longitudinal white bands in the fingernails associated with typical skin lesions. The nail lesions have not been described until reported by Burge in 1992 and it may be a characteristic finding in Hailey-Hailey disease.
Acantholysis ; Axilla ; Blister ; Friction ; Groin ; Humans ; Nails ; Neck ; Pemphigus, Benign Familial* ; Skin

The monoclonal antibody Ki-1(CD30) was first described in 1982 and was reported to react selectively with Reed-Sternberg cells in Hodgkins disease, highly activated B and T cells and large cell lymphomas of both T-and B-cell origin. Lymphomatoid papulosis(LyP) is characterized by recurrent erythematous papules or nodules that undergo spontaneous healing with hyperpigmentation and scarring. Histologically, two major types of LyP can be distinguished: type A is characterized by the presence of variable numbers of Ki-1+ large, atypical lymphocytes with some Reed-Sternberg like cells and type B is characterized by Ki-1- atypical cerebriform mononuclear cells similar to those in mycosis fungoides. We report a case of Ki-1+ lymphomatoid papulosis which was histopathologically recognized as a type B lesion but represented a Ki-1 positive reaction.
B-Lymphocytes ; Cicatrix ; Hodgkin Disease ; Hyperpigmentation ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Mycosis Fungoides ; Reed-Sternberg Cells ; T-Lymphocytes

Kaposis sarcoma is a multicentric neoplastic vascular tumor involving the skin or internal organs. We report a case of classic Kaposis sarcoma in a 69-year-old male who had positive serum cytomegalovirus antibodies and disseminated multiple erythematous to purplish colored confluent papules and edema on both lower legs. The histopathological finding showed abnormally proliferated and dilated vessels, vascular spaces, spindle cells, and extravasated erythrocytes. He was treated with radiotherapy, but died due to ventricular tachycardia.
Aged ; Antibodies ; Cytomegalovirus ; Edema ; Erythrocytes ; Humans ; Leg* ; Male ; Radiotherapy ; Sarcoma, Kaposi* ; Skin ; Tachycardia, Ventricular

We report two cases of recurrent extramarnmary Pagets disease after wide local excision. Both cases involved the scrotum and the penoserotal junction, respectively. On histopathological examination, we could observed many Paget cells confined to the epidermis and the hair follicle, but not invading the underlying dermis in both cases. There was no evidence of internal malignancy. Both cases were treated with wide local reexcision.
Dermis ; Epidermis ; Hair Follicle ; Paget Disease, Extramammary* ; Scrotum

A 5-year-old girl was diagnosed with neurofibromatosis type 2 (NF-2) due to multiple neurofibromas, cafe-au-lait spots, and schwannomas of the brain. During ophthalmologic evaluation, a posterior subcapsular cataract and a gray-green colored subretinal lesion were found in right eye. Fluorescein angiography (FA) revealed a combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). At age 9, she underwent cataract surgery. At this time FA and spectral-domain optical coherence tomography (SD-OCT) were taken. The SD-OCT showed an elevated hyperreflective mass in the retina with prominent attenuation of the inner and outer retina, but minimal attenuation in the photoreceptor layers. The underlying retina appeared to be disorganized and thick (791 microm). This is the first case report of SD-OCT imaging of a CHRRPE associated with NF-2 in a pediatric patient. By using SD-OCT in this patient, we could obtain detailed tumor characteristics, and SD-OCT may be helpful in the diagnosis and management of CHRRPE.
Child, Preschool ; Diagnosis, Differential ; Female ; Hamartoma/complications/*diagnosis ; Humans ; Neurofibromatosis 2/*complications/diagnosis ; Retinal Diseases/complications/*diagnosis ; Retinal Pigment Epithelium/*pathology ; Tomography, Optical Coherence/*methods ; Visual Acuity

No abstract available.

We report a case of Darier's disease with typical cutaneous and nail lesions in a 50-year-old male patient. He has brownish hyperkeratotic papules on the ant.chest, back, both axilla and groins and verrucous surfaced hypertrophic plaques on the scalp, forehead and both postauricular areas, which were accompanied by a severe itching sensation and malodor. Also he has finger nail lesions such as subungual hyperkeratotic papules, distal wedge shaped notching and multiple longitudinal red lines on the nail plates. Histopathologic features taken from the inguinal area revealed hyperkeratosis, suprabasal clefts and lacuna, protruded villi lined with one layer of basal cells into the cavity and dyskeratotic cells such as corps ronds in the epidermis. Hyperkeratotic papules on the ant.chest, back, both axilla and groins disappeared after systemic treatment withe a low dose of oral etretinate but hypertrophic plaques on the scalp and both postauricular areas still remained clinically and histopathologically. We then the treated postauricular hypertrophic plaques with CO2 laser vaporization and severe malodor with oral isotretinoin and it resulted in a marked improvement.
Acitretin* ; Axilla ; Darier Disease* ; Epidermis ; Etretinate* ; Fingers ; Forehead ; Groin ; Humans ; Isotretinoin* ; Lasers, Gas* ; Male ; Middle Aged ; Pruritus ; Scalp ; Sensation ; Volatilization*

Among the various types of congenital meniscal anomalies, the double-layered lateral meniscus is extremely rare. The double-layered meniscus consists of both the upper additional and the lower normal meniscus. As the upper additional meniscus is mobile, it can be easily displaced, while the lower lateral meniscus is usually normal in shape and volume. A 42-year-old woman suffering from pain and locking of her left knee underwent Magnetic resonance imaging (MRI) examination and an arthroscopic surgery. A rare meniscal abnormality was seen in her left knee, which presented as a double-layered lateral meniscus with displacement. It was remarkable that the upper additional meniscus was displaced over the intercondylar eminence of the tibia and it mimicked a bucket-handle tear. Even though it is rare, it is necessary to consider the possibility of displaced double-layered meniscus in the differential diagnosis of a bucket-handle tear. Here, we report the MRI and arthroscopic findings of a displaced double-layered lateral meniscus, which was similar to the bucket-handle tear.
Adult ; Arthroscopy ; Diagnosis, Differential ; Female ; Humans ; Knee ; Magnetic Resonance Imaging ; Menisci, Tibial* ; Tears* ; Tibia

Background: Atopic dermatitis (AD) is characterized by chronic, relapsing skin inflammation (eczema) with itchy sensation. Keratinocytes, which are located at the outermost part of our body, are supposed to play important roles at the early phase of type 2 inflammation including AD pathogenesis. Objective: The purpose of this study was to evaluate whether keratinocytes-derived reactive oxygen species (ROS) could be produced by the allergens or non-allergens, and the keratinocytes-derived ROS could modulate a set of biomarkers for type 2 inflammation of the skin. Methods: Normal human epidermal keratinocytes (NHEKs) were treated with an allergen of house dust mites (HDM) or a non-allergen of compound 48/80 (C48/80). Then, biomarkers for type 2 inflammation of the skin including those for neurogenic inflammation were checked by reverse transcriptase-polymerase chain reaction and western immunoblot experiments. Results: HDM or C48/80 was found to upregulate expression levels of our tested biomarkers, including type 2 T helper-driving pathway (KLK5, PAR2, and NF κ B), epithelial-cell-derived cytokines (thymic stromal lymphopoietin, interleukin [IL]-25, IL-33), and neurogenic inflammation (NGF, CGRP). The HDMor C-48/80-induced expression levels of the biomarkers could be blocked by an antioxidant treatment with 5 mM N-acetyl-cysteine. In contrast, pro-oxidant treatment with 1 mM H2O2 could upregulate expression levels of the tested biomarkers in NHEKs. Conclusion Our results reveal that keratinocytes-derived ROS, irrespective to their origins from allergens or non-allergens, have a potential to induce type 2 inflammation of AD skin.