A Clinical and Histopathologic Study of Lichen Sclerosus et Atrophicus / 대한피부과학회지

BACKGROUND: Lichen sclerosus et atrophicus (LS et A) is an uncommon chronic lymphocyte-mediated dermatosis that causes substantial discomfort and morbidity, most commonly in adult women. It occurs predominantly in the anogenital area, and extragenital lesions also occur. OBJECTIVE: The aim of this study is to compare clinical and histopathologic features of genital and extragenital LS et A. METHODS: We retrospectively reviewed the medical records and 31 biopsy specimens of 29 patients who had been diagnosed with LS et A between January 2001 to June 2007 in Asan Medical Center. RESULTS AND CONCLUSION: The male to female ratio of genital LS et A and extragenital LS et A was 1:20, 1:3 respectively. Mean age at onset of genital LS et A and extragenital LS et A was 47.8 and 36.9 respectively. The color of the lesions was mostly white, irrespective of the involved area. Genital LS et A lesions were mostly located on labium majora or minora. Extragenital LS et A lesions were mostly located on the trunk, head and neck. The predominant symptom for genital LS et A was pruritus, whereas extragenital LS et A was mostly asymptomatic. The main treatment regime of LS et A was topical corticosteroid. The combination therapy with topical corticosteroid and pimecrolimus/tacrolimus resulted in symptomatic relief as well as improvement of skin lesions, without significant side effect. Histopathologically all LS et A cases showed vacuolar alteration, papillary dermal hyalinization, and dermal lymphohistiocytic infiltration. Hypergranulosis, exocytosis, red blood cell extravasation, lichenoid inflammatory cell infiltration were more frequently observed in genital LS et A (p<0.05). Follicular plugging, epidermal atrophy and loss of rete ridges were more frequently observed in extragenital LS et A (p<0.05).

A Clinical and Histopathological Study of Parapsoriasis / 대한피부과학회지

BACKGROUND: Parapsoriasis described a group of cutaneous disease that can be characterized by scaly patches or slightly elevated plaques that have a resemblance to psoriasis, hence the nomenclature. Up to now, a uniformly accepted definition of parapsoriasis remains lacking. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathological features of parapsoriasis in Korea. METHODS: The clinical charts, photographs and skin biopsy slides of patients who had been clinically diagnosed as parapsoriasis at the initial visit were reviewed. Then the evaluation of age at onset, sex distribution, disease duration, anatomical distribution, clinical features of the skin lesions, associated symptoms, family history, co-existing disease, and histopathologic features were conducted. Six cases from small plaque parapsoriasis group and four cases from large plaque parapsoriasis group were investigated for the presence of TCR gene rearrangement using a PCR technique. RESULTS: The male to female ratio was 1: 1.2. The average ages of onset in large plaque type and small plaque type were 44.7 and 32.9 years respectively. 1) According to the clinical classification, 7 (29%) of the patients had large plaque type and 17 (71%) had small plaque type. 2) Many lesions of large plaque parapsoriasis group exhibited varying degrees of epidermal atrophy, fine wrinkles and telangiectasia. 3) Large plaque parapsoriasis showed more histologic changes consisting of epidermal atrophy, exocytosis and atypical lymphocytes. 4) Monoclonal T-cell receptor gene rearrangement was not detected in all of 10 patients with large and small plaque parapsoriasis. CONCLUSION: In our study, some of our findings are in agreement with those already in the literature, whereas others, particularly the male to female ratio and constitutional symptoms, differ. Our results also indicate that there are many clinical and pathological differences between large plaque and small plaque parapsoriasis.

A Clinical and Histopathologic Study of Pityriasis Rubra Pilaris / 대한피부과학회지

BACKGROUND: Pityriasis rubra pilaris (PRP)is a rare papulosquamous disorder of unknown etiology. Although the clinical and histopathologic features of PRP are relatively well known in western patients, there are few available studies in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathologic features of pityriasis rubra pilaris(PRP) in Korea METHODS: The clinical charts, photographies and skin biopsy slides of patients clinically diagnosed as PRP at the initial visit were reviewed. Finally 30 patients were diagnosed as PRP. They were classified into 5 groups according to the Griffiths classification. And then the evaluation of age at onset, sex distribution, clinical feature of the skin lesion, associated symptoms, clinical course and histopathologic features were conducted. RESULTS: 1) The male to female ratio was 1:1.14. The age of onset was varied from 1 to 65 years with two peaks in first and fifth decades. The average ages of onset in adult type and juvenile type were 40.1 and 5.1 years respectively. 2) According to the Griffiths classification, 14(47%) of the patients had type I, 4(13%) had type III, 7(23%) had type IV, and 3(10%) had type V. However, 2(7%)patients could not be classified under any of these five types due to atypical clinical feature or insufficient follow-up periods. 3) The common clinical features during the disease course were follicular hyperkeratosis(83%), knee and/or elbow involvement(83%), palm and/or sole hyperkeratosis(80%). Erythroderma was present in 5 patients(17%) during the disease course. 4) There were no specific symptoms in most cases, but 9 patients had ben suffered from mild itching. 5) 3 patients had a positive family history, and were thought to have inherited autosomal dominant trait. 6) The frequent histopathologic findings were alternating parakeratosis and orthokeratosis (100%), superficial perivascular infiltration(100%), broad rete ridge(97%), acanthosis(93%), hypergranulosis (87%), follicular plugging(73%). CONCLUSION: We investigated the clinical and histopathological features of PRP in Korea. In our study, the clinical and histopathological features of PRP were not different from those observed in western patients.

A Clinicopathologic Study on Nevus Sebaceus

BACKGROUND: Nevus sebaceus of Jadassohn is a complex anomaly involving the epidermis, dermis, apocrine glands and hair follicles, as well as the sebaceous glands, and a variety of benign and malignant tumors and neurologic abnormalities may be associated with it; however, only a few studies on it have been reported in the Korean literature. OBJECTIVE: The objectives of this study was to document clinical and histopathologic features of nevus sebaceus in Koreans and compare them to those in the previously reported studies. METHOD: Clinical records and histopathology of 53 cases of nevus sebaceus diagnosed at Ewha Womans University Tongdaemun Hospital during a period of 18 years were reviewed and analyzed. RESULTS: The average incidence rate of nevus sebaceus among new patients was 0.03%. The male-to-female ratio was 1.04 and the average age at visit, 16.5 years. The lesions had been present since birth or had developed before age 10 in 85% of the cases. A11 lesions were situated on the head, and the most common site was the scalp. Linear 1esions (20.8%) were mostly facial lesions. Histopathologically, the incidence of sebaceous and apocrine glandular hyperplasia was most notable in the 13-18 and over-18 age groups. The sebaceous glands showed small punched out defects or "holes" in 24.5%; ectopic apocrine glands were found in 24.5 %. Mature hair follicles were absent or greatly reduced in number in 75.5%. Acanthosis nigricanslike pattern of acanthosis and mild papillomatosis were the dominant changes in the epidermis. Hyperplasias and neoplasms were associated in 15.1%, which included 5 cases of primitive follicular induction and 1 trichoblastoma. CONCLUSION: In this series, no examples of malignant neoplasms were identified; however, an early total excision before age 13 is recommended since a variable "deficit malformation" of the follicles with hyperplasia of the epidermis and sebaceous glands becomes more prominent after that age.

Clinical and Histopathological Study of Calcinosis Cutis / 대한피부과학회지

BACKGROUND: Calcinosis cutis may occur when connective tissue is abnormal (dystrophic), or where calcium or phosphate levels in the blood are high(metastatic); alternatively, there may be no obvious underlying cause(idiopathic). The exact incidence of calcinosis cutis in dermatologic patients is not well-kniown and the pathomechanism of it remains to be elucidated. OBJECTIVE: This study was performed to analyze the incidence, clinical and histopathological features of calcinosis cutis. METHODS: We studied 72 patients with calcinosis cutis who visited our department between January 1985 and December 1996. The patients were classified into 3 types (dystrophic, metastatic, and idiopathic) and were analyzed clinically and histopathologically. Results : The results were summerized as follows; 1. There were 60 cases(83.3%) and 12 cases(16.7%) of the dystrophic and idiopathic types, respectively but the metastatic type was not observed. The frequency of dystrophic calcification in individual disorders was 88.2% in pseudoxanthoma elasticum, 66.7% in trichilemmal cyst and dermatomyositis, and 64.6% in pilomatrichoma. 2. In the idiopathic type, it was common in females aged over 50 years and the mean duration of lesions was 2.8+/-2.0 years. The predilection site was the flank(5 cases) and most of the lesions(7 cases) showed skin-colored nodules 3. Histopathologically, calcified material of the idiopathic type was located predominently in the dermis(10 cases) and was present as large masses surrounded by a foreign body reaction. 4. In the dystrophic type, calcified deposits were noted predominently within the shadow cells of pilomatricoma, in the degenerated elastic fibers of pseudoxanthoma elasticum, and in the keratinized area of basal cell carcinoma, squamous cell carcinoma, trichilemmal cyst, and epidermal cyst. Foreign body reactions with giant cells and mononuclear cell infiltrations were often found around large deposits of calcium. Conclusion : The incidence of calcinosis cutis in dermatologic patients is relatively low, but a variety of disorders can be associated with cutaneous calcification. Therefore, dermatologists should be familiar with the different forms of cutaneous calcification and the dermatoses that manifest them.