A case of dedifferentiated liposarcoma

( Kihyuk Shin ),( Sang Hyeon Won ),( Woo Il Kim ),( Min Young Yang ),( Won Ku Lee ),( Gun Wook Kim ),( Hoon Soo Kim ),( Hyun Chang Ko,),( Byung Soo Kim ),( Moon Bum Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Liposarcoma accounts for 20% of sarcoma in adults, and it is the most common soft tissue sarcoma. Dedifferentiated liposarcoma is a subtype of liposarcoma which is characterized by the coexistence of poorly differentiated, pleomorphic sarcoma juxtaposed to well-differentiated liposarcoma, and it is known to be associated with more aggressive feature. A 68-year-old woman presented with 5.9 cm sized dome shaped subcutaneous mass on the chest wall, which was growing rapidly for 2 months. She was diagnosed previously as non-small cell lung cancer with brain metastasis 8 months ago. A biopsy was performed and histopathology revealed that well-differentiated areas consist of mature adipocytes and lipoblasts were comingled with dedifferentiated areas composed of poorly differentiated non-lipogenic round cells with myxoid stroma. Immunohistochemically, the round cells were positive for vimentin and smooth muscle actin, and negative for cytokeratin, CD34, and S100. A diagnosis of dedifferentiated liposarcoma was established based on clinicopathologic features. Wide excision was performed, and the patient was transferred to the radiation oncology department for radiation therapy. We have described a patient with dedifferentiated liposarcoma which is a rare clinicopathologic variant of liposarcoma. To our knowledge, this is the second report of dedifferentiated liposarcoma in Korean dermatologic literature.

Cuticle as a predictor of the thumbnail growth rate

( Kihyuk Shin ),( Jun-oh Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Hoon-soo Kim ),( Hyun-chang Ko ),( Byung-soo Kim ),( Moon-bum Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2

Background: Growth rate of nails is affected by various conditions. However, little is known about the anatomic factor which can predict the nail growth rate. Objectives: To investigate the anatomic factors that could predict the growth rate of thumbnails. Methods: Twenty-four healthy young adults were enrolled in the study. We marked a line with ultrapulse CO₂ laser on thumbnails at the baseline. Thumbnails were photographed with ruler at the baseline and six weeks after marking. The distance from the mark to the proximal nailfold, and the length of lunula and cuticle was measured by analyzing magnified photos with ImageJ. Results: The female to male ratio was 1.4:1 and the mean age was 30.3 years (24-39 years). Growth rate of dominant hand thumbnails was faster than that of non-dominant hand thumbnails (3.85 vs. 3.28 mm/ month, P < 0.0001). Cuticle length was positively correlated with thumbnail growth rate (r=0.3946 and P=0.0055), and cuticle length of dominant hand thumbnails was longer than that of non-dominant hand thumbnails (P=0.0002). Age, sex, height, weight, body mass index (BMI), length of lunula were not significantly associated with thumbnail growth rate. Conclusion: This study highlights the role of cuticle in predicting thumbnail growth rate. These results could be helpful in the treatment of nail disorders as well as for investigation on medication affecting nail growth rate.

A case of primary cutaneous extraskeletal osteosarcoma on the back

( Kihyuk Shin ),( Sang Hyeon Won ),( Woo Il Kim ),( Min Young Yang ),( Won Ku Lee ),( Gun Wook Kim ),( Hoon Soo Kim ),( Hyun Chang Ko ),( Byung Soo Kim ),( Moon Bum Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Extraskeletal osteosarcoma (ESOS) is a malignant mesenchymal neoplasm composed of tumor cells producing bone, osteoid, and/or chondroid material without attachment to skeletal structures. Despites most reports of ESOS were located in deep soft tissue, primary cutaneous SFT is extremely rare. A 77-year-old man presented with subcutaneous mass on the left lower back, which had been growing slowly for 1.5 years. The patient did not complain of lesionassociated pain and itching. A biopsy was performed, and the tumor was composed of proliferating atypical and pleomorphic spindle cells with focal osteoid formation. On immunohistochemical examination, the tumor cells were positive for vimentin, and negative for cytokeratin, CD68, CD31 and CD34. Beyond histopathological examination, computed tomography and positron emission tomography-computed tomography revealed no other skeletal involvement. These clinicopathologic findings were consistent with primary cutaneous ESOS. The tumor was excised, and the patient was transferred to the oncology department for the chemotherapy. To our knowledge, this is the second report of primary cutaneous ESOS in Korean dermatologic literature. Despite its rarity, ESOS should be considered in the differential diagnoses when osteoid is found on the skin.

A case of malignant solitary fibrous tumor of the skin

( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Tae-wook Kim ),( Sung-min Park ),( Gun-wook Kim ),( Hoon-soo Kim ),( Hyun-chang Ko ),( Byung-soo Kim ),( Moon-bum Kim ),( Hyun-joo Le 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Solitary fibrous tumor (SFT) is uncommon fibroblastic mesenchymal tumor which commonly affects pleura and peritoneum. Despites multiple reports in in various organs, cutaneous SFT is rare with limited case reports. Furthermore, malignant cutaneous SFT is exceptional. A 59-year-old man presented with protruding mass on the right shoulder, which was growing slowly for 10 years. A 5.0 X 4.3 cm sized tumor was relatively hard, and telangiectasia was observed on the surface of the tumor. The patient did not complain of lesion-associated pain and itching. An excisional biopsy was performed, and histopathologic examination revealed well-circumscribed and unencapsulated hypercellular tumor which was composed of proliferating spindle cells with a haphazard pattern embedded in fibrotic stroma. The spindle cells were characterized by pleomorphism and numerous mitotic figures. On immunohistochemical examination, the tumor cells were strongly positive for CD34 and vimentin, and negative for cytokeratin, desmin, S100, smooth muscle actin. These clinico-histopathologic findings were consistent with malignant SFT of the skin and the patient was transferred to the oncology department for the chemotherapy. To our knowledge, this is the second report of the malignant SFT of the skin in the literature.

Acral calcified angioleiomyoma: a rare clinicopathologic variant of cutaneous angioleiomyoma

( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Tae-wook Kim ),( Sung-min Park ),( Hyun-joo Lee ),( Hoon-soo Kim ),( Hyun-chang Ko ),( Byung-soo Kim ),( Moon-bum Kim ),( Gun-wook Ki 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Cutaneous angioleiomyomas are benign tumors with vascular smooth muscle differentiation. The tumor usually involves the lower extremity, but acral sites such as toes, fingers, feet, and hands are rarely affected. Leiomyoma of deep soft tissue commonly shows regressive changes including prominent fibrosis, and calcification, however, cutaneous angioleiomyoma rarely manifests these changes. A 61-year-old female presented with subcutaneous nodule on the right heel. The lesion began to develop 3 months ago without history of trauma or infection, and the patient reported no subjective symptom. The lesion was totally excised, and histopathology revealed a well-circumscribed, non-encapsulated tumor composed of interlacing fascicles of spindled cells with elongated blunt-ended nuclei. A major finding was extensive calcifications, which dominated over the cellular component. On immunohistochemical examination, the spindle cells expressed strong immunoreactivity for α -smooth muscle actin. Based on these findings, a diagnosis of acral calcified angioleiomyoma was made. We have described a rare clinicopathologic variant of angioleiomyoma which has a predilection for acral locations and shows extensive calcifications predominating over the tumor itself. To our knowledge, this is the first report of a patient with acral calcified angioleiomyoma in Korean dermatologic literature.

Acral lentiginous melanoma developed in Papillon-Lefevre syndrome patient

( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Tae-wook Kim ),( Sung-min Park ),( Hyun-joo Lee ),( Gun-wook Kim ),( Hyun-chang Ko ),( Byung-soo Kim ),( Moon-bum Kim ),( Hoon-soo Ki 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Papillon-Lefevre syndrome is an extremely rare autosomal recessive genodermatosis characterized by diffuse palmoplantar keratoderma and severe early-onset periodontitis. The malignant cutaneous neoplasms developed within the hyperkeratotic lesions of Papillon-Lefevre syndrome patient are exceptional, and the association of Papillon-Lefevre syndrome with malignant melanoma had not been elucidated yet. A 46-year-old woman presented with 7.0 X 6.0 cm sized ulcerative pigmented patch on her left sole which was growing slowly for 2 years. Palmoplantar keratoderma and premature loss of teeth had been observed since infancy and 13 years old, respectively, thus she was diagnosed with Papillon-Lefevre syndrome 16 years ago. A biopsy was performed on pigmented patch, and histopathologic examination showed masses of spindle-shaped cells, with nuclear polymorphism, and numerous mitotic figures. A diagnosis of acral lentiginous melanoma was established with consideration for clinicopathologic features and the patient was transferred to the plastic surgery department for wide excision. To our knowledge, this is the third report of a patient with Papillon-Lefevre syndrome in whom acral lentiginous melanoma developed in the literature. Because malignant melanoma is the cutaneous neoplasia with the greatest mortality rates, dermatologists should examine acral lesions carefully in Papillon-Lefevre syndrome patients.

Epidermolytic ichthyosis: Hot spot mutation in KRT10 gene mutation (Arg156Cys)

( Dongyoung Roh ),( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Gun-wook Kim ),( Hoon-soo Kim ),( Byung-soo Kim ),( Moon-bum Kim ),( Hyun-chang Ko ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Epidermolytic ichthyosis (EI) is a hereditary keratinization disorder caused by mutations in the keratin 1 (KRT1) or keratin 10 (KRT10) genes. It is characterized by generalized blister formation and erosion at birth and generalized epidermolytic hyperkeratosis in adulthood. According to the genotype-phenotype correlation, R156C mutation in KRT10 gene is a hot spot mutation which presents severe and typical form of EI. A 47-day-old girl presented with erythematous scaly patches on whole body with focal erosions. Whole body skin exfoliation with erythroderma was appeared at birth. As growing up, cobble stone pattern on the extensor area and corrugated hyperkeratosis on the flexural area were shown. Both palms and sole were sparing in hyperkeratosis. Histopathological findings showed orthohyperkeratosis with intraepidermal blister. Epidermolytic hyperkeratosis and granular degeneration were observed in epidermis. Genetic analysis revealed a mutation in the KRT10 gene which was a heterozygous missense mutation of c.466C>T leading to an amino acid substitution from Arginine to Cysteine (p.Arg82Cys). We diagnosed this patient as epidermolytic ichthyosis. To date, only 1 report of epidermolytic ichthyosis were confirmed genetically in Korean literature. This case supports the genotype-phenotype correlation that R156C mutation in KRT10 gene is a hot spot mutation.

A case of reflex sympathetic dystrophy (RSD) following prosthetic infection

( Yeona Kim ),( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Gun-wook Kim ),( Hoon-soo Kim ),( Byung-soo Kim ),( Moon-bum Kim ),( Hyun-chang Ko ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Reflex sympathetic dystrophy (RSD), known as complex regional pain syndrome type 1, is usually arises after trauma . RSD is characterized by hyperalgesia, sudomotor change and impaired motor function. Patients complain of changing in skin temperature, color, or swelling of lesion. It can often be misdiagnosed as soft tissue infection, vascular disease, and peripheral neuropathy. A 74-year-old female consulted to our clinic for evaluation for cutaneous infection of the right knee region. She presented with shiny erythematous patch accompany with hyperalgesia, swelling and heating sensation for 8 months. She also suffered motor weakness and knee stiffness. She had past history of repeated prosthetic infection after knee surgery several years ago. However, her symptom didn’t improve after treatment of antibiotics for 8 months. Bacterial culture from blood and skin tissue were both negative for pathogen. Radiologic and additional laboratory findings also showed no evidence of infection. Digital infrared thermal imaging showed localized hyperthermia on the lesion. She was finally diagnosed as RSD based on the above findings, and treated with oral pregabalin. RSD is rarely reported in the dermatologic literature, but it is not uncommon in practice. Therefore, dermatologists should consider the possibility of RSD when symptoms mimic skin infections not responding to antibiotics persist.

Eruptive xanthogranuloma in a healthy adult male

( Dongyoung Roh ),( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Gun-wook Kim ),( Hoon-soo Kim ),( Byung-soo Kim ),( Moon-bum Kim ),( Hyun-chang Ko ),( Won-ku Lee ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although rarely occurs in adults. Lesions in the adult form are typically solitary. Multifocal eruptive presentation of xanthogranuloma in adult is very rare. We report a rare case of eruptive xanthogranuloma in a healthy man. A 31-year-old male presented with multiple erythematous to yellowish papules on trunk for 2 months. The lesions were asymptomatic, but the number of the lesion gradually increased. The patient had no other general symptom. On physical examination, 20 to 30 discrete, reddish to yellow, dome-shaped and flat-topped, 0.5 to 1 cm papules and nodules were observed on the chest, abdomen and extremities. Serum lipid studies were normal. Histopathological findings showed dermal infiltrate of histiocytes with foamy macrophages and touton giant cells. We diagnosed this patient as adult onset eruptive xanthogranuloma. Our case is an exceptionally rare presentation of eruptive multiple xanthogranuloma in an adult with no associated disease. To our knowledge, this is the 3rd reported case of eruptive adult xathogranuloma with more than 20 lesions in Korean literature.

Clinical study of Morbihan disease: analysis of 24 cases

( Yeona Kim ),( Kihyuk Shin ),( Woo-il Kim ),( Min-young Yang ),( Won-ku Lee ),( Hoon-soo Kim ),( Byung-soo Kim ),( Tae-wook Kim ),( Moon-bum Kim ),( Hyun-chang Ko ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2

Background: Morbihan disease (MD), also known as edematous rosacea, is a rare variant of rosacea that involves chronic erythema and persistent upper-facial swelling. MD is not extremely rare disease, but there is little articles regarding its clinical features and course. Objectives: The aim of this study is to examine the clinical characteristics of MD. Methods: We reviewed clinical photographs, medical records of 24 cases diagnosed as MD visiting Pusan national university hospital (Pusan and Yangsan) since 2010. Results: The mean age of patients was 56 (range: 36-75) years old, and 19 (79%) patients were male. 10 patients (42%) showed unilateral upper-facial swelling. The most common subtype was erythematotelangiectatic rosacea (15, 62.5%), followed by ocular rosacea. No significant correlation was found among the severities of cutaneous finding, eyelid swelling and feature of rosacea. Patients were treated by various modalities (including systemic antibiotics, corticosteroids, isotretinoin etc.). However, only one quarter of patients noted partial and transient improvement. Conclusion: We have analyzed various clinical features of MD, and showed that upper-facial edema of MD and cutaneous rosacea features had no relationships. Our results support that although MD is described as a variant of rosacea, pathomechanism between typical rosacea and MD may be different.