CD4-/CD56+/CD123+ Hematodermic Neoplasm Showing Early Liver Metastasis

Hematodermic neoplasm (HN) is a clinically aggressive neoplasm with a high incidence of cutaneous involvement and a risk of leukemic dissemination. In the recent WHO-EORTC classification, the term blastic natural killer cell lymphoma has been replaced with CD4+/CD56+ HN because of its derivation from a plasmacytoid dendritic cell precursor. Cases of HN that completely lack CD4 or CD56 expression, therefore represents a diagnostic problem. A 68-year-old Korean male was diagnosed with CD4-/CD56+ HN and treated with hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) at initial treatment, and then switched to high dose methotrexate/cytarabine. His disease relapsed and resulted in death from bone and brain disease 6 months after complete clinical remission, despite diagnostic workups, including a radioisotope liver scan and ultrasound-guided fine needle aspiration biopsy. Further cytogenetic studies such as comparative genomic hybridization could elucidate the genetic mechanisms in the development and progression of lymphomas. We report an unusual case of 'CD4-/CD56+/CD123+ HN' showing early liver metastasis.

Comparative Study of the Gross Interpretation of Phototesting and Objective Measurement with Using a Spectrophotometer for Patients with Psoriasis and Vitiligo Treated with Narrow-band UVB

BACKGROUND:Determination of the minimal erythema dose (MED) is important for developing a phototherapy protocol and to diagnosis photosensitivity disorders. But obtaining a precise and reproducible MED is quite difficult because a phototest for erythema is based on subjective assessment. OBJECTIVE: The objective of our study was to compare the gross interpretation of a phototest and the objective measurement using a spectrophotometer for determining the parameters of cutaneous narrow-band UVB (NBUVB) therapy. METHODS: A total of 14 psoriasis and 10 vitiligo patients who receiving NBUVB phototherapy with skin types III and IV were selected for this study. To perform phototesting, ten sites on the skin of the back were vertically exposed to a series of 10 NBUVB doses among 14 doses between 340 and 1,400 mJ/cm2. We interpreted the gross findings of erythema and measured the L*a*b* values with using a spectrophotometer at each phototest spot and at the control skin. Also, we evaluate the relationship between the gross presentation and the spectrophotometric analysis by delta E for the assessment of the minimal perceptible erythema (MPE) and MED. RESULTS: For all the subjects, the MEDs were measured in the 490~1,000 mJ/cm2 range. The average of the colorimetric values for the control skin were L*: 64.8, a*: 7.9 and b*: 19.8. Among them, the L* value and MED value were shown to be inversely correlated, and as the L* value was decreased, the MED was increased. For the MPE, the delta E, which was the color difference of the normal skin and the phototest area, was within the range of 1.5~3.0 in 17 of the 21 patients, and 4 patients were within the range of 1.0~1.5. For the MED, among the 21 patients, the delta E of 17 patients was within the range of 3.0~6.0, and 4 patients were within the range of 6.0~12.0. CONCLUSION: A spectrophotometer enables UV erythema to be assessed objectively and quantitatively, and this can compensate for the disadvantages of subjective gross interpretation when determining the MED. Delta E is a good novel and objective indicator for determining the MPE and MED. So, a spectrophotometer is a very useful instrument for developing a phototherapy protocol for psoriasis and other dermatoses and for making the diagnosis of photosensitivity disorders.

Comparative Study of the Gross Interpretation of Phototesting and Objective Measurement with Using a Spectrophotometer for Patients with Psoriasis and Vitiligo Treated with Narrow-band UVB

BACKGROUND:Determination of the minimal erythema dose (MED) is important for developing a phototherapy protocol and to diagnosis photosensitivity disorders. But obtaining a precise and reproducible MED is quite difficult because a phototest for erythema is based on subjective assessment. OBJECTIVE: The objective of our study was to compare the gross interpretation of a phototest and the objective measurement using a spectrophotometer for determining the parameters of cutaneous narrow-band UVB (NBUVB) therapy. METHODS: A total of 14 psoriasis and 10 vitiligo patients who receiving NBUVB phototherapy with skin types III and IV were selected for this study. To perform phototesting, ten sites on the skin of the back were vertically exposed to a series of 10 NBUVB doses among 14 doses between 340 and 1,400 mJ/cm2. We interpreted the gross findings of erythema and measured the L*a*b* values with using a spectrophotometer at each phototest spot and at the control skin. Also, we evaluate the relationship between the gross presentation and the spectrophotometric analysis by delta E for the assessment of the minimal perceptible erythema (MPE) and MED. RESULTS: For all the subjects, the MEDs were measured in the 490~1,000 mJ/cm2 range. The average of the colorimetric values for the control skin were L*: 64.8, a*: 7.9 and b*: 19.8. Among them, the L* value and MED value were shown to be inversely correlated, and as the L* value was decreased, the MED was increased. For the MPE, the delta E, which was the color difference of the normal skin and the phototest area, was within the range of 1.5~3.0 in 17 of the 21 patients, and 4 patients were within the range of 1.0~1.5. For the MED, among the 21 patients, the delta E of 17 patients was within the range of 3.0~6.0, and 4 patients were within the range of 6.0~12.0. CONCLUSION: A spectrophotometer enables UV erythema to be assessed objectively and quantitatively, and this can compensate for the disadvantages of subjective gross interpretation when determining the MED. Delta E is a good novel and objective indicator for determining the MPE and MED. So, a spectrophotometer is a very useful instrument for developing a phototherapy protocol for psoriasis and other dermatoses and for making the diagnosis of photosensitivity disorders.

The Photosensitivity Localized in a Vitiliginous Lesion Was Associated with the Intramuscular Injections of Synthetic Progesterone during an In Vitro Fertilization-embryo Transfer

The cutaneous diseases associated with progesterone are autoimmune progesterone dermatitis, erythema multiforme- like eruption, drug-induced progesterone dermatitis and solar urticaria. Estrogen and progesterone are widely used in oral contraceptives and hormone replacement therapies, and they are rarely known to cause a photosensitive reaction. The mechanism of contraceptive-induced photosensitivity is uncertain. Estrogen, rather than progesterone, in the combined oral contraceptive pill has been most frequently implicated in the induction of photosensitivity. A 32-year-old woman presented with an erythematous patch with an itching sensation on the centrofacial area of a residual vitiligious lesion. She had a history of being previously treated with narrow band UVB for 1 year. Her skin lesions had mostly subsided, but some lesions continued. She underwent an in vitro fertilization-embryo transfer 3 months previously, and she then took synthetic progesterone for 3 weeks starting at the 4th week of pregnancy. She was in good health with neither a family history of photosensitivity nor a personal history of any other drug ingestion or topical agent such as sunscreen in association with the beginning of her lesions. Phototesting revealed her to be markedly photosensitive in the UVB and UVA ranges. The intradermal skin reactions to progesterone combined with irradiation with UVA or UVB were positive. We report here on an unusual case of photosensitivity that was localized in a vitiliginous lesion, and this was associated with the intramuscular injections of synthetic progesterone that she had received during an in vitro fertilization-embryo transfer.

A Case of Milium-like Syringoma on Vulva / 대한피부과학회지

Milium-like syringoma is a rare variant of syringoma first described by Friedman and Butler in 1987. Clinically, it presents as milium, but histologically reveals syringoma with overlying keratin filled epidermoid cysts. It occurs predominantly on the periorbital area of middle-aged women. Syringomas on the genital area have rarely been reported. The lower portion of the cysts and keratin materials show positivity for CEA and negativity for CK7. The pathogenesis is not well defined, but there are some hypotheses, which include pruritus and increasing hormonal receptors (estrogen and progesterone receptors). We report a case of milium-like syringoma on the vulva.

Surgical Tip: The Double-Bladed Scalpel in Mohs Micrographic Surgery

Mohs micrographic surgery is applied as the primary method of treatment for various cutaneous neoplasms. Many other methods that are modified applications of Mohs micrographic surgery have also been suggested. We introduce a technique, which is a modified vertical method of Mohs micrographic surgery using the double-bladed scalpel.

A Case of Xanthoma Disseminatum with Diabetes Insipidus / 대한피부과학회지

Xanthoma disseminatum (XD) is a rare, potentially progressive non-Langerhans cell histiocytosis, which preferentially affects males in their childhood or young adulthood. It is characterized by the insidious onset of small, yellowish-red to brown papules and nodules that are discrete and disseminated. They characteristically involve the eyelids and flexural areas of the axillary and inguinal folds, and the antecubital and popliteal fossae. Systemic disease frequently occurs, the most common manifestation being diabetes insipidus (DI), which occurs in about 40% of cases and is due to deposition of xanthoma cells in the hypothalamic-pituitary axis. We present a case of XD with DI, which was treated with the combination therapy of CO2 laser vaporization, cyclophosphamide, electrocauterization and synthetic anti-diuretic hormone.

Two Cases of Acneiform Eruption Caused by Epidermal Growth Factor Receptor Inhibitors / 대한피부과학회지

Epidermal growth factor receptor (EGFR) inhibitors are associated with unique and dramatical dermatological side effects. The most commonly encountered adverse effect is a mild skin toxicity characterized by a sterile follicular and pustular rash that may be treated empirically and usually does not require treatment modification. Secondary adverse reactions seen with anti-EGFR therapy include xerosis, pruritus, paronychia, hair abnormality, and mucositis. According to the Korean literature, there are no reports of acneiform eruption induced by erlotinib (Tarceva(R)). We report a case of acneiform eruption induced by cetuximab (Erbitux(R)) and a case of acneiform eruption induced by erlotinib (Tarceva(R)).

Primary Sjogren's Syndrome Manifested asAnnular Erythema and Fever of an Unknown Origin / 대한피부과학회지

Sjogren's syndrome is an autoimmune disorder that's characterized by dry eyes and dry mouth, and this is due to lymphocytic infiltration of the lacrimal and salivary glands. The skin lesions in Sjogren's syndrome usually manifest with xeroderma, pruritus and pigmentation, but sometimes the lesions appear as annular erythema. Patients with Sjogren's syndrome rarely display fever, with the exception of the cases that show complicated illness such as lymphoma. We report here on a case of a 27-year-old woman who was diagnosed with Sjogren's syndrome, and she showed signs of annular erythema and a fever of an unknown origin.

Primary Sjogren's Syndrome Manifested asAnnular Erythema and Fever of an Unknown Origin / 대한피부과학회지

Sjogren's syndrome is an autoimmune disorder that's characterized by dry eyes and dry mouth, and this is due to lymphocytic infiltration of the lacrimal and salivary glands. The skin lesions in Sjogren's syndrome usually manifest with xeroderma, pruritus and pigmentation, but sometimes the lesions appear as annular erythema. Patients with Sjogren's syndrome rarely display fever, with the exception of the cases that show complicated illness such as lymphoma. We report here on a case of a 27-year-old woman who was diagnosed with Sjogren's syndrome, and she showed signs of annular erythema and a fever of an unknown origin.

A Case of Churg-Strauss Syndrome Associated with Small Bowel Perforationfollowing High Dose Systemic Steroid Intravenous Injection / 대한피부과학회지

Churg-Strauss syndrome is an uncommon disorder characterized by bronchial asthma or allergic rhinitis, peripheral eosinophilia, systemic necrotizing vasculitis, and extravascular granuloma formation. It commonly affects the lungs, skin, gastrointestinal tract, peripheral nerve, kidneys and the heart. Gastrointestinal symptoms of Churg-Strauss syndrome are common, mainly shown in abdominal pain, diarrhea, or bleeding. Ulceration, perforation, stenosis can occurr with ischemia induced by vasculitis. Gastrointestinal perforation by Churg-Strauss syndrome has been reported rarely, and was recently discovered to have a close correlation to systemic steroid treatment. Nearly one third of patients infected with a gastrointestinal perforation die. This report covers the interesting case of a 54 year-old woman who was being treated for asthma, and was diagnosed as Churg-Strauss syndrome by multiple polyneuropathy and ulcerative macules. Small intestine perforation was generated 4 days after high dose systemic steroid intravenous injection.

Combination Nasolabial Transposition Flap and Island Pedicle Flap Following Mohs Surgery of Simultaneous Basal Cell Carcinomas Involving Both Nasal Alae

The nasal ala is a challenging area for surgical reconstruction, with thick sebaceous skin, the lack of an ample tissue reservoir, and an adjacent free margin. Numerous flaps have been reported for the repair of alae defects. A 71-year-old woman with simultaneous basal cell carcinomas involving both nasal alae was treated by Mohs micrographic surgery. The surgical defects measured 1.5x1.5 cm on the center of the right nasal ala and 1.0x1.0 cm on the left nasal ala, including the alar crease and rim. The right nasal ala was used as a nasolabial transposition flap and the left nasal ala was reconstructed by an island pedicle flap. The final shape and texture were satisfactory. The flaps survived and nasal symmetry was preserved. Combined nasolabial transposition and island pedicle flaps thus offer a superior esthetic and functional result owing to minimized tension. This may be a valuable reconstructive option in the repair of bilateral nasal alae defects.

A Case of Trichilemmal Carcinoma Treated with Mohs Micrographic Surgery

Trichilemmal carcinoma is a cutaneous adnexal tumor originating from the outer root sheath of hair follicle, and it was first described by Headington in 1976. Clinically, it usually occurs as an asymptomatic solitary papule, nodule or mass on the face or scalp. This neoplasm is a malignant counterpart of trichilemmoma, and it has been reported in the literature as trichilemmal carcinoma, tricholemmal carcinoma, malignant trichilemmoma, and tricholemmocarcinoma. Although histologically, trichilemmal carcinoma frequently has maliganant features, it has a relatively benign clinical behavior. We think Mohs micrographic surgery is a useful treatment modality in trichilemmal carcinoma because the final skin defect is smaller than a wide excision. We report a case of primary trichilemmal carcinoma which had developed on the face, treated with Mohs micrographic surgery.

A Case of Atypical Vascular Lesion of the Skin following Radiotherapy for Breast Carcinoma / 대한피부과학회지

"Atypical vascular lesions" is the term which was first used in 1994 by Fineberg and Rosen for vascular lesions after radiation therapy except for cutaneous angiosarcoma. Vascular lesions mostly occur after radiation therapy in breast cancer patients who have undergone mastectomy. The lesions are 5 mm or less, red or red-brown papules, histologically dilated vascular channels extended as the superficial and mid-dermis are observed. Post-radiation vascular lesions tend to follow a benign course with metastasis or death not having been reported yet. The lesions look histologically similar to well differentiated cutaneous angiosarcoma occurring after radiation therapy and breast reconstruction, indeed it is possible for post-radiation vascular lesions to become cutaneous angiosarcoma. Atypical vascular lesions have recently become a new issue for concern in some countries. Within Korea however, until now, a case it had not yet been reported. The patient was 50 year-old female who had chemotherapy after Modified Radical Mastectomy and salpingoophorectomy for breast cancer and endometriosis, atypical vascular lesions in the field of radiation therapy occurred. We report an interesting case of atypical vascular lesion.

A Case of Warty Dyskeratoma Presenting as a Plaque Form / 대한피부과학회지

Warty dyskeratoma (WD) is a benign solitary tumor characterized clinically as a crateriform keratotic nodule and histologically by focal acantholytic dyskeratosis. The lesion of WD presents as a whitish or grayish hyperkeratotic papule with an umbilicated center, often involving the face, the scalp, or the back. Lesions are usually less than 1~2 cm in size. In 2007 a new plaque form of WD was first described by Omulecki. We report a case WD on the forehead presenting as a plaque form in a 9-year-old boy.

Treatment Outcome and Satisfaction after Micrographic Surgery Using Margin Strip Method / 대한피부과학회지

BACKGROUND: Mohs micrographic surgery (MMS) was known as an ideal treatment for non-melanocytic skin cancers. Recently, various modified MMS methods have been introduced. Margin strip method is a modified micrographic surgery technique that makes operation and reconstruction more easy. OBJECTIVE: The aim of this study was to evaluate the treatment results and patients' satisfaction of micrographic surgery using margin strip method. METHODS: We reviewed 72 cases of basal cell carcinomas (BCCs) and 36 cases of squamous cell carcinomas (SCCs) treated with micrographic surgery using margin strip method from January 2001 to December 2004. We analyzed them in aspects of the sex, age, duration, location, size and histological types of tumor. They were also evaluated in aspects of surgical margin, depth, stage, defect size and reconstruction methods. We also analyzed follow-up data concerning recurrence rate, complications and patients' satisfaction. RESULTS: SCCs were more common in older age groups, and BCCs were showing centrofacial distribution. Mean numbers of stage was 1.5 in BCC and 1.3 in SCC, and safety excised margins were 0.72 cm in BCC and 1.03 cm in SCC. Infiltrative type of BCC, the numbers of stage and size of safety excised margin were increased. The transposition and rotation flap were most commonly used in BCC and SCC, respectively. Patients' satisfaction were 8.06 in BCC and 7.76 in SCC. 6. Recurrence rate were 0% in BCC and 5.6% in SCC. CONCLUSION: Micrographic surgery using margin strip method may be a useful and satisfactory method for the treatment of non-melanocytic skin cancers.

A Case of Generalized Lichen Nitidus Treated with Low Dose Cyclosporine / 대한피부과학회지

Lichen nitidus is a rare and chronic inflammatory dermatosis first described by Pinkus in 1907. The etiology of it is still unknown and the majority of instances appear in children and young adults. The lesions are usually asymptomatic but may be mildly pruritic and the Koebner phenomenon may be observed. It is characterized by flesh-colored, minute, shiny papules occurring on the genitalia, abdomen, chest, and extremities. This disorder is most often localized, but there are rare reports of patients having a more generalized distribution of lesions. Although lichen nitidus is self-limited in most cases without any sequelae, the course of generalized lichen nitidus is unpredictable. We report a case of a 16-year-old girl diagnosed by generalized lichen nitidus refractory to general treatment and successfully treated with low dose cyclosporine (2 mg/kg/day).

The Effect of Controlled Elastic Dynamization on Regenerate Bone Healing in Distraction Osteogenesis of Canine Tibia / 대한정형외과학회잡지

In order to investigate wheter or not elastic dynamization by axial micromotion enhanced regenerate bone healing in distraction osteogenesis(callotasis), a unilateral external fixator, with which the magnitude of dynamization could be controlled, was applied to the tibiae of 12 Korean adult mongrel dogs, weighting approximately 35kg. Tibiae and fibulae of both sides were osteotomized at the proximal metaphysio-diaphyseal junction and fixed with unilateral external fixators. Distraction was started from the third postoperative day until 2 cm (10% of tibial length) of length gian was achived, at the rate of 0. mm twice a day. When 1 cm (5%) of lengthening was achieved, controlled elastic dynamization of 1 mm (group I), 2 mm (group II), and 3 mm (group III) in magnitude was allowed on left tibia. Right tibia was not dynamized until sacrifice, and served as a control for each group. Plain anteroposterior radiographs and measurements of bone mineral density(BMD) by dual x-ray absorptiometry(DXA) were obtained pre- and post-operatively. Relative ratio of BMD was calculated, dividing the BMD value measured in each period by the BMD value measured at the beginning of dynamization. Mechanical test was performed in uniaxial compression on an Instron machine. Following observations were made: 1. Plain anteroposterior radiography showed that at mid-consolidation phase (postoperative 50th day) the width of callus in the distraction gap increased significantly in all dynamization groups, as compared to their control groups(p 0.05). 3. Axial Compression test revealed that the dynamization group had significantly increased stiffness, maximal load, elastic modulus, and strength, as compared to those of the control group(p < 0.05). In conclusion, controlled elastic dynamization effectively enhances regenerate bone healing in distraction osteogenesis of canine tibia, at 1 mm to 3 mm of magnitude.