Epstein-Barr Virus Associated Lymphoproliferative Lesion Presenting as a Hydroa Vacciniforme-like Eruption / 대한피부과학회지

BACKGROUND: There have been several reports of patients with a severe hydroa vacciniforme (HV)-like eruption from Asia and Latin America. The cutaneous lesions are present in both sun-exposed and nonexposed areas in these patients unlike true HV. Several patients have died of malignant hematologic malignancies. The latent Epstein-Barr virus (EBV) infection has been detected in the skin lesions of the patients. OBJECTIVE: To describe clinical, histological, immunohistochemical, and molecular pathological features of the patients with EBV associated lymphoproliferative lesion presenting as a HV-like eruption. METHODS: The clinical, histological, and immunohistochemical features of 16 patients were reviewed. The presence of T-cell receptor (TCR)-gamma gene rearrangement was investigated using polymerase chain reaction (PCR) technique. Photoprovocation by repetitive UVA exposure was performed in five patients. In situ hybridization was performed to detect mRNA for EBV in the lesional skin biopsy specimen, lymph node biopsy specimen, mucosal biopsy specimen of stomach, and the skin biopsy specimen of photo-provoked site. PCR was performed to detect DNA for EBV in the skin biopsy specimens of 6 patients and peripheral mononuclear cells of 2 patients. RESULTS: The severity of the skin lesion and the clinical course varied among the patients. Skin biopsy specimens obtained from a papule or a vesicle showed perivascular and periadnexal infiltrate of lymphoid cells with T-cell phenotype. However, clonal TCR-gamma gene rearrangement was not detected in all 8 patients. Papules or vesicles were induced by repetitive UVA exposure in 5 patients. A latent EBV infection was demonstrated in all the tested samples, such as lesional skin, lymph node, gastric mucosa, peripheral blood mononuclear cells, and the photo-provoked lesion. CONCLUSION: EBV associated lymphoproliferative lesion presenting as a HV-like eruption is a novel disease that is not related to classic HV. Repetitive irradiation of UVA can induce the skin lesion in some patients with EBV associated lymphoproliferative lesion presenting as a HV-like eruption.

Dermographism ( II ) : The Prevalence in Korean Pregnant and Puerperal Women / 대한피부과학회지

The prevalence of dermographism in 249 Korean pregnant and puerperal women was studied using a dermographic tester. The result was summarized as follows: 1. The prevslence rate of dermographism was 8.0%(20/249). 2. The prevalence rate of dermographism was not significantly correlated with the stages of pregnancy(p>0.1). 3 There was no significant difference in the prevalence rate of dermographism between the pregnant and puerperal women(8.0%) and the control group of Korean women in the same ages(9.5%) (p>0.1).

Multiple Proliferating Trichilemmal Tumors with Ordinary Trichilemmal Cysts: A case report

Proliferating trichilemmal tumor is one of rare benign tumors of the skin appendages, considering as hamartoma of the terminal hair follicle, isthmic segment of the outer root sheath. We report a case of numerous proliferating trichilemmal tumors admixed with ordinary trichilemmal cysts with femilial history in 64-year-old man. He has total 157 lesions, which are chiefly located in scalp (48), back (32), and also face, neck, trunk, extremities. Histologically, the tumor is composed of irregularly arranged and ansatomosed trabeculae, lobules, or sheets of proliferated trichilemmal squamous epithelium with peripheral palisading of the basaloid cells. Several layers of squamoid or ovoid cells have PAS-positive clear cytoplasm. And it also shows abrupt amorphous, trichilemmal keratinization. Some squamous eddies with mild cellular atypism are associated, but definite invasion or other evidence of the malignancy is not found. usual multiple trichilemmal cysts are admixed with tumor and occasionally exhibit connection between them.

Waardenburg's Syndrome in Son and Mother / 대한피부과학회지

We report a case of Waardenburgs syndrome in 18-year-old male patient who has total deafness of the right ear, heterochromia irides, hypopigmented patches on the face and trunk, and disseminated lentigines on the entire body surface since birth. His 51-year-old mother also has total deafness, heterochromia irides, white foreloek, and disseminated lentigines on the entire body surface. The patient with this disorder may complain of pigmentary abnormalities, and we believe dermatologists should pay more attention to systemic signs of the congenital pigmentary disorders.

Dermographism: I . The Prevalence in Korean general Population / 대한피부과학회지

The prevalence of dermographism in the general population of Korea was studied using a dermographic tester on 1, 074 persons(M: 489, F: 585, M/F= 1/l. 2). The result was summarized as follows: 1. The prevalence of dermographism from the pressure of 4, 800 Gm/sq. cm (pressure 2) was 6. 7%. 2. There were no significant difference in the prevalence between male(6.3%) and female(7%) (p>0. 1). 3, The peak age group of dermographism was third(9.4%), fourth and second decades in decreasing order.

Clinical and Histopathological Observation of Porokeratosis / 대한피부과학회지

We observed clinical and histopathological findings of porokeratosis in 16 cases which comprised 2 linear types, 4 superficial disseminat:ed forms, 4 disseminated superficial actinic porokeratosis and 6 plaque types. From the observation, the following results were obtained. l. Clinical results 1) Eleven cases were men, 5 cases were women, and the sex ratio was 2. 2 to 1, age distribution was from 6 to 61 years (mean age: 34. 2years), and the distribution of age of onsets was from 2 to 52 years(mean age of onset' 23. lyears). 2) Four cases had family history of porokeratosis, but linear forms did not. 3) Nine cases had pruritus especially, all cases of DSAP and DSP except 1 case complained pruritus. 4) Tmo cases of DSAP had tendency of summer exacerbations. 2. Histopathological results 1) Cornoid lamellae were prominent in all cases of plaque types and linear types, and minimal in all cases of DSAP and DSP except 1 case. 2) Epidermis between the cornoid lamellae were normal or acanthotic in all cases except 1 case of plaque types and linear types, and were atrophic in 5 cases of 8 cases in DSAP and DSP.

Histopathological Observation of Cafe au Lait Spots / 대한피부과학회지

We observed 17 cases of cafe au lait spots histopathplogi ally. Out of 17 cases, 9 cases were neurofibromatosis with neurofibromas, 6 cases were neurofibromatosis without neurofibroma, and 2 cases were normal healthy persons. The results were as follows. l. In all 17 cases, melanin pigment was increased in melanocytes and keratinocytes. 2. Griant pigment granules were identified only in 3 cases of neurofibromatosis with neurofibromas.