Clinical and Histopathological Study of Cutaneous Melanoma in Korea / 대한피부과학회지

BACKGROUND: Malignant melanoma in Korea has been increasing as in other countries, but there is no nationwide survey of malignant melanoma in Korea. OBJECTIVE: The aim of this study was to document the clinical and histopathological features of cutaneous melanoma in Korea. METHODS: The Dermatopathology Research Group conducted a review of nationwide collection of 109 primary cutaneous melanomas, diagnosed at 23 institutes over a recent 3-year period. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS AND CONCLUSION: The peak age was the 7th decade with the mean age of 58.1 years. Korea has a higher rate of acral melanoma and much lower rate of lentigo maligna melanoma. .Major component tumor cell was spindle cell type. Clark level of tumor was III or more and Breslow depth was 2mm or more at the time of the first diagnosis in many cases (62%). Pre-existing melanocytic nevus was present in a few cases (3 cases). All these features suggest that the racial difference between the Korean and the Caucasian is evident. We also suggest that an early detection program is very important to cure this malignant tumor.

Clinical and Histopathological Study of Cutaneous Lymphomas in Korea / 대한피부과학회지

BACKGROUND: The relative frequency and clinicopathologic characteristics of lymphomas vary according to geography and race. Data on the features of cutaneous lymphoma in Korea are limited. OBJECTIVE: The aim of this study was to document the relative occurrence, the clinical and histopathological features of cutaneous lymphomas in Korea. METHODS: The Korean Dermatopathology Research Group conducted a review of nationwide collection of 80 cutaneous lymphomas, diagnosed at 23 institutes over recent 3-year period. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS AND CONCLUSION: Korea has a higher rate of T-cell lymphoma and NK/T cell lymphoma and a much lower rate of cutaneous B cell lymphoma. The relative frequency of the major diagnostic group according to WHO classification was as follows: mycosis fungoides/Sezary syndrome, 42.5%; anaplastic large cell lymphoma, 19%; nasal and nasal type NK/T cell lymphoma, 15%; subcutaneous panniculitis-like T cell lymphoma, 11%; peripheral T cell lymphoma, unspecified, 7.5%; follicular lymphoma, 3%; marginal zone lymphoma, 1%; angioimmunoblastic lymphadenopathy, 1%. Compared with Western countries, the rate of nasal and nasal-type NK/T cell lymphoma and subcutaneous panniculitis-like T cell lymphoma were much higher. Therefore, The EORTC classification is not effective in dealing with Korean cases of cutaneous lymphoma. We consider the principles of the WHO classification are applicable to the Korean cases of cutaneous lymphoma.

Cutaneous T Cell Lymphoma, Pleomorphic, Small/medium-sized with Tissue and Blood Eosinophilia / 대한피부과학회지

Cutaneous T cell lymphoma, pleomorphic, small/medium-sized is a primary cutaneous lymphoma that consists of less than 30% of large, pleomorphic T cells and mostly small/medium-sized, plemorphic T cells stained negatively CD30. This entity presents clinically with one or several red-purple nodules without patches & plaques and histopathologically infiltration of small/medium-sized T cells with a dense nodular or diffuse pattern in the dermis. Usually cellular infiltrations consist of atypical lymphocytes without eosinophils, plasma cells and neutrophils but there are cases infiltrated with atypical lymphocytes and eosinophils. We report a case of cutaneous T cell lymphoma, pleomorphic, small/medium-sized in a 55-year-old male who was presented with hard nodules on both elbows, both ankles, left 1st interdigital web, both wrists and both lateral sides of thigh. Histopathological findings showed diffuse and nodular infiltrates with atypical lymphocytes and eosinophils in the dermis, not involving the epidermis. Bone scan, bone marrow biopsy and CT scan taken to find out involvement of other organs showed normal finding except for eosinophilia at bone marrow biopsy.

Tufted Angioma:Clinicopathologic Surveys and the Response to Intralesional Steroid

BACKGROUND: Tufted angioma is an uncommon slowly progressive vascular tumor found typically in infants and young children with characteristic histologic findings, so called "cannonball" appearance. OBJECTIVE: The purpose of this study was aimed to investigate the clinical and histopathological characteristics of tufted angioma and the response to intralesional steroid. METHODS: Clinical information of 10 patients with tufted angioma diagnosed in Severance hospital and Pundang CHA hospital from 1983 to 1999 was obtained from the medical records and clinical follow-ups. We re-evaluated 10 biopsy specimens obtained from them with routine H&E staining. RESULTS: Five male and five female patients were included. In 9 patients the lesion appeared before 2 months of age. Four had a lesion at birth. The thigh was the most common site. The clinical symptoms were diverse, but characteristically tenderness was present in most cases. In all the patients the lesions had a tendency to spread progressively. Microscopically, numerous, distinct, variably sized, tightly packed capillary and endothelial cellular lobules were scattered in the dermis. There were characteristic semilunar spaces adjacent to the capillary tufts. Six patients received intralesional triamcinolone. This treatment was found to be effective in 5 patients who experienced remarkable improvement. The improved cases had similar histologic findings which were composed of cellular mass more than lumen formation. We classified our specimens into two categories, one with more cellular mass and the other with more lumen formation in relative proportion. The former was different from the latter in that it had more solid appearance and more definite margin. And we realized that it was useful to divide into these two categories since its response to treatment could be different. CONCLUSIONS: Tufted angioma is a relatively uncommon disease with characteristic histopathologic findings. It seems not to regress spontaneously. So early treatment is required to pre-vent further spreading up to the extent. We treated 6 patients with intralesional injection of triamcinolone and 5 patients experienced marked improvement which had more cellular mass more than lumen formation histopathologically.

Epithelioid Hemangioma Associated with Nevus Flammeus / 대한피부과학회지

Epithelioid hemangioma, the term which was first used by Enzinger and Weiss, is synonymous with angiolymphoid hyperplasia with eosinophilia. It defines a lesion occurring usually in the dermal and subcutaneous tissue of the head and neck, characterized by localized, marginated and relatively symmetrical proliferation of the capillaries around a medium-sized vessel with an epithelioid hyperplasia of endothelial cell. There is a marked inflammatory reaction composed of lymphocytes and eosinophils. We report a case of epithelioid hemangioma associated with nevus flammeus in a 53-year-old male. Histology revealed typical feature of epithelioid hemangioma that proliferation of dilated vessels with prominent endothelial cells surrounded by dense lymphoid cell infiltrates but no eosinophils were identified.

Clinical and Histologic Features of Pityriasis Rosea and Pityriasis Lichenoides in Children / 대한피부과학회지

BACKGROUND: Pityriasis rosea is an acute inflammatory dermatosis with an unproven etiology. The typical clinical feature consists of an initial, single herald patch followed by development of smaller, disseminated, papulosquamous, ovoid macules. However, an unusual papular type of pityriasis rosea is relatively common in children and it shows similar clinical characteristics to pityriasis lichenoides. OBJECTIVES: Our purpose was to observe the clinical and histologic features of pityriasis rosea and pityriasis lichenoides, and differentiate two diseases in children. METHODS: Sixteen children with pityriasis rosea and nineteen children with pityriasis lichenoides were investigated clinically and histologically. RESULTS: 1. Clinically, pityriasis rosea may be differentiated from pityriasis lichenoides by the existence of herald patch, short duration, the uniformity of size and shape, and residual pigmentation. 2. Focal parakeratosis, intraepidermal vesicle and papillary derma edema are more common in pityriasis rosea. In contrast, deep dermal infiltration, dyskeratosis, epidermal necrosis and confluent parakeratosis are more common in pityriasis lichenoides. 3. Comparing to the plaque type, a papular type of pityriasis rosea shows more involvement in the extremities with longer duration. Histologically, parakeratosis and papillary dermal edema are less and spongiosis is more commonly observed in papular type. CONCLUSIONS: Pityriasis rosea in children often shows papules especially on the extremities, similar to that of pityriasis lichenoides. However, from the results in this study, we believe that two diseases can be differentiated if both clinical and histologic findings are carefully studied.

Proliferating Nodules Within a Congenital Melanocytic Nevus: Proper criteriae for surgical removal in infantile periods

Congenital melanocytic nevus(CMN) constitute a well-known risk factor in the development of malignant melanoma, but melanoma developing in newborn and infants are extremely rare. We describe a case of proliferating nodules within CMN at the age of 13 months. Like our case it is difficult to manage the proliferating lesions within CMN in infants. So we carefully suggest the indications of preventive excision when proliferating lesions occur within CMN as follows; 1) preventive excision is postponed until the age of two when the proliferating lesions are slowly growing, 2) surgical excision is needed when the lesions are rapidly growing exceeding 1 cm in size even though it is before the age of two.

Pigmented eccrine poroma with squamous differentiation / 대한피부과학회지

Eccrine poroma is a common adnexal neoplasm that differentiated toward the acrosyringium, the intraepidermal portion of the eccrine sweat duct. This disease appear clinically as skin-colored or brownish nodule, but the lesion can be pigmented when it contains melanocytes and melanin. In that case, it can be very similar to melanoma. Futhermore, eccrine poroma rarely shows squamous differentiation histologically. Nevertheless, it should be differentiaed from basalcell carcinoma and seborrheic keratosis when it shows squamous differentiation. We report a case of pigmented eccrine poroma with squamous differentiation in a 42-year-old male.

A Case of multiple cutaneous neurofibromas treated by CO2 laser / 대한피부과학회지

Multiple Cutaneous neurofibromas are the most common skin manifestation of Von Recklinghausen neurofibromatosis(NF-1). The CO2 laser is presented as a useful tool for the removal of large numbers of small- or medium-sized cutaneous neurofibromas. Its advantages include high patient satisfaction with the rapid removal of hundreds of neurofibromas, with minimal morbidity and an enhanced appearance. We report a case of multiple cutaneous neurofibromas treated by CO2 laser in a 40 year-old man whom we eliminated 288 cutaneous neurofibromas out of 788 in total. After 3 months of follow up, the lesions were healed with slight erythemas and minimal scars.

A clinical analysis of 11 patients with chronic actinic dermatitis in Korea

Chronic actinic dermatitis is a rare disease worldwide and also in Korea. However there has been no clinicohistologic and photobiological analysis of chronic actinic dermatitis in Korea. We examined 11 patients who were diagnosed as chronic actinic dermatitis and the results of this study were compared with previous reports. Most patients were elderly men who had erythematous papules or lichenified plaques on sun-exposed areas such as the face, neck, and dorsum of the hands with severe itching sensation. All patients had severe sensitivity to UVB and biopsied specimens showed findings of chronic eczema. Five patients had positive photopatch test materials. The patients were treated with systemic and topical steroid, cyclosporine and antihistamine.

Epidermolysis bullosa simplex (Dowling-Meara type) associated with pyloric atresia and congenital urologic abnormalities

We report a case of epidermolysis bullosa simplex, Dowling-Meara type (EBS-DM), which was associated with congenital pyloric atresia (PA) and various urologic abnormalities, a diagnosis confirmed by immunofluorescence mapping and electron microscopic findings. Immunofluorescent mapping showed the serum from a patient with bullous pemphigoid faintly binding to the floor of the blister, and monoclonal antibodies against type IV and VII collagens were also stained on the floor of the blister. Electron microscopy showed epidermolytic cleavage and prominent clumping of tonofilaments in the basal and suprabasal keratinocytes. An abdominal radiograph and barium swallow showed a complete obstruction at the pyloric channel level. The widespread bullae healed without any scar formation and the bullae formation was localized on the extremities after 3 months of age without any specific treatment. Multiple urologic abnormalities such as bilateral hydronephrosis, hydroureter and a distended bladder with trabeculation were observed at 12 months of age. Currently, with the patient at 4 years of age, bullae still appear on the hands and feet and nail shedding can be observed. The patient's father, a paternal uncle and a paternal aunt had had similar bullous eruptions in infancy, all of which had improved spontaneously by the age of one.

A Case of Primary Cutaneous Adenoid Cystic Carcinoma / 대한피부과학회지

We report a case of primary cutaneous adenoid cystic carcinoma in a 39-year-old male patient. It was a slightly erythematous hard nodule on left chin and composed of cribriform and tubular masses of basaloid cells. Lumina formed by neoplastic cells contained mucin and hyalin, and similar constituents were located between neoplastic cell masses and adjacent dermis. Immunohistochemically, the neoplastic cells stained only focally with S-100 protein and negative with carcinoembryonic antigen(CEA). Therapy should employ wide surgical excision that extends well beyond the clinical confines of the neoplasm.

A Case of Aneurysmal Fibrous Histiocytoma / 대한피부과학회지

Aneurysmal fibrous histiocytoma(AFH) is a histologic variant of dermatofibroma. The importance of the recognition of AFH as a variant of dermatofibroma lies in the differential diagnosis because the histologic appearance of AFH is very similar to dermatofibrosarcoma protuberans and other vascular malignancies, such as Kaposi's sarcoma and angiosarcoma. A 32-year-old man was seen for a nodular mass on the right popliteal fossa of 4 years' duration. The excisional biopsy specimen showed a relatively well circumscribed tumor mass in the dermis. In addition to compact storiform proliferation of spindle cells, there were multiple, irregular, blood-filled, aneurysmal tissue spaces that were devoid of lining of endothelial cells. Also, deposition of hemosiderin was prominent, particularly in the periphery of the aneurysmal spaces. Immunohistochemical stain for CD34 were negative to most tumor cells. After excisional therapy, there was no evidence of recurrence during the period of ten months' follow-up.

Two Cases of Deep Vein Thrombosis Associated with Behcet's Disease / 대한피부과학회지

In Behcet's disease(BD), there is a marked increase in vascular complication. Venous thrombosis is a major feature of the disease, although arterial thrombosis is rarely described. In Behcet's disease, thrombosis occurs in 20 to 30% of patients. We present two cases of Behcet's disease admitted to our hospital whose chief complaint was progressive increases in swelling and pain in their legs. In the first case, routine coagulation tests and sero-immunological tests were within normal limits, however, protein C and S activity were significantly decreased in the patient. So these findings suggest that auto-immune acquired protein S deficiency may be involved in the pathogenesis of thrombotic events in BD.

A Case of Branchial cleft fistula / 대한피부과학회지

Congenital branchial anomalies are developmental defects resulting from incomplete obliteration of branchial clefts, arches and pouches, and usually found as cysts, fistulas and sinuses. The sinus or fistula is usually noted during infancy and young childhood due to recurrent infection. We have experienced a case of branchial cleft fistula in a 12-year-old male. It was a skin colored, nontender nodule which had mucus secreting from a small opening pore. We performed complete surgical removal and histopathologically the lumen was lined by pseudostratified columnar cells and the stroma was composed of aggregates of lymphocytes with many germinal centers. So we finally diagnosed it as branchial cleft fistula of second type branchial anomaly.

Spontaneously Healed Primary Cutaneous Alternariosis: Reports of 2 Cases / 대한의진균학회지

No abstract available.

A Case of Erosive Adenomatosis of the Nipple / 대한피부과학회지

We present a case of erosive adenomatosis of the nipple in a 22-year-old woman. The clinical features of erosive adenomatosis of the nipple are similar to Paget's disease in that it has erythematous erosions with oozing and fissured crusts on one nipple. However, histologic findings are characteristic, consisting of dilated tubular structures that are lined by a peripheral layer of cuboidal cells and a luminal layer of columnar cells showing secretary properties into the lumen. The clinical course is benign, so simple mastectomy is enough for treatment.

Clinical Analysis of 14 Cases of Pyoderma Gangrenosum / 대한피부과학회지

BACKGROUND: Pyoderma gangrenosum is a rare disease in which a painful nodule or pustule breaks down to form a progressive enlarging ulcer. Until now, only 8 cases of pyoderma gangrenosum have been reported in Korea. Therefore, we thought it necessary to perform a clinical analysis of pyoderma gangrenosum in Korea with a review of literature. OBJECTIVE: Our purpose was to find the clinical features of pyoderma gangrenosum in Korea. METHODS: Fourteen cases with pyoderma gangrenosum were investigated by reviewing medical records. Results There were 6 males and 8 females. The onset age was between 4 years and 65 years, and most(9 cases) had developed the condition between the ages of 20 and 60. Thirteen cases involved the extremities and 3 cases had whale body involvement, Seven cases(50%) had multiple lesions. All cases had pain at the lesional sites. Two cases were classified as the bullous type and the others were ulcerative in nature. The histological fmdings were non-specific. Dense inflarnmatory infiltrates composed of lymphocytes and predominant neutrophils were found in the epidermis and dermis associated with ulceration. Two cases were treated only with systemic steroids, and 2 cases with dapsone and steroids, 2 cases with colchicine and steroids, and 1 case with steroids, dapsone and colchicine. One case was treated with colchicine and anti-Tbc drug, 1 case with dapsone, 3 cases with antibotics, 1 case with the anti-Tbc drug and 1 case with anti-cancer drugs. Systemic disease was present in 5D% of the cases. The associated diseases were Behcets disease(3 cases), tuberculosis(2 cases), systemic lupus erythematosus(1 case), pancytopenia(1 case), iron deficiency anemia(1 case), acute leukemia(1 case), and colon adenoma(1 case). Recurrence developed in 2 cases and positive pathergy reactions were observed in 3 cases. CONCLUSION: Pyoderma gangrenosum was eccompanied with systemic disease in 50% of the cases and the most common therapeutic drugs were steroids. It is therefore impartant to detect the presence of any underlying disease and to treat this alongside pyoderma gangrenosum.

Prurigo Pigmentosa:A Report of 5 Cases with a Review of the Korean Literature

Prurigo pigmentosa(PP) is a rare dermatosis with an unknown cause characterized by intensely pruritic papules that resolve with reticulate pigmentation. It is a disease entitity commonly reported in Japan but seemingly rare in other parts of the world. We report 5 cases that clinically and histopathologically fulfilled the characterisitics of PP and were treated with dapsone resulting in significant clinical improvement.

Sweet's Syndrome Associated with Acute Erythema Nodosum

A 44-year-old man had Sweet's syndrome (acute febrile neutrophilic dermatosis), accompanied by erythematous tender subcutaneous nodules resembling erythema nodosum(EN). The EN-like lesions histologically showed a septal panniculitis with predominantly neutrophilic in-filtrates. The association of Sweet's syndrome with EN seems to be uncommon and only a few cases have been reported until the present. We describe a patient with Sweet's syndrome associated with acute EN.