Giant acquired digital fibrokeratoma occurring on the left great toe.

Acquired digital fibrokeratoma is an uncommon, benign fibrous tumor which usually occurs in adults as a solitary lesion. The most frequent locations are fingers and toes and the size of the tumor is generally small, around 3~5 mm. An 18-year-old female presented with a solitary, skin-colored, round and protruded nodule of the left great toe. The size of nodule was 2.5×1.6×1.4 cm. Histopathologic examination revealed typical findings of acquired digital fibrokeratoma. Herein, we report a giant acquired digital fibrokeratoma.

Detection of a novel missense mutations in atrichia with papular lesions.

BACKGROUND: Atrichia with papular lesions (APL) is a rare inherited disease characterized by early onset of total hair loss, followed by papular lesions over the extensor areas of the body. Recently, mutations in the human hairless (HR) gene have been implicated in its pathogenesis. The identification of mutations in the HR gene is important for differentiating between APL and alopecia universalis (AU). OBJECTIVE: We compared the HR genes of patients with presumed AU who showed minimal or no response to treatment with the HR genes of healthy controls. METHODS: The subjects were 11 patients with presumed AU who had not responded to treatments. Fifty healthy people were included as controls for molecular analysis. To screen for mutations, polymerase chain reaction was performed. RESULTS: DNA analysis identified a novel heterozygous G-to-A transition at nucleotide position 191 in exon 5. The mutation was not found in the controls, other AU patients, or any unaffected family members except for the patients' mother and maternal grandfather, who were heterozygous HR gene carriers. CONCLUSION: Our study identifies a novel missense mutation in exon 5 of the HR gene in a Korean APL patient previously diagnosed as AU.

Analysis of familial factors using the basic and specific (BASP) classification in Korean patients with androgenetic alopecia.

BACKGROUND: The mechanisms and inheritance of androgenetic alopecia (AGA) have yet to be elucidated. Several clinical studies suggest that a predisposition to AGA is affected by a variety of paternal and/or maternal hereditary factors. No previous study has addressed the association of AGA with family history based on the pattern of hair loss. OBJECTIVES: The purpose of this study was to investigate paternal and/or maternal genetic influences in each type of hair loss pattern using the basic and specific (BASP) classification and to explore whether the morphology of AGA tends to be inherited in family members. METHODS: Between October 2007 and September 2008, 1220 Korean participants, 998 male and 222 female, were classified according to the BASP classification at 13 university dermatologic centers. Information was collected using a standard questionnaire and BASP classification sheet. RESULTS: Parental influences on anterior hairline shape in men were predominantly from the paternal side, whereas these effects were less notable in women. In the absence of a family history, statistical analysis showed a higher frequency for early-onset AGA than late-onset AGA (Pearson χ(2)P < .05). Basic types of hair loss had a higher degree of inheritance from the paternal side of the family, regardless of specific type. LIMITATIONS: The evaluation of hair loss pattern and family history was done by the patients. CONCLUSIONS: Familial factors affecting the morphology of AGA differ between male and female individuals, and for each type of BASP classification.

Changes in murine hair with dietary selenium excess or deficiency.

It is known that an excess or deficiency of selenium (Se) causes abnormalities in hair. We evaluated changes in the hair follicles associated with Se imbalance in a C57BL/6 mouse model to better understand the role of Se in hair growth. Fifteen C57BL/6 mice were assigned to diets providing excessive, adequate, or deficient amounts of Se. Alopecia with poliosis was observed in the groups receiving either excessive or deficient selenium. Skin biopsy from alopecia patches showed increased telogen hair follicles with epidermal atrophy. There was a significant decrease of anti-apoptotic Bcl-2 and an increase of pro-apoptotic Bax in the excessive-Se group compared with the adequate group. We suggest that alopecia with poliosis is caused by changes in the hair follicle cycle due to the imbalance of Se and partially influenced by the decrease of the ratio of Bcl-2/Bax, which is associated with induction of apoptosis of keratinocytes.

Vitiligo Coexistent with Nevus Depigmentosus: This Was Treated with Narrow-Band UVB and These Lesions Were Followed Using the Mexameter®, a Pigment-Measuring Device.

Nevus depigmentosus (ND) is a congenital, non-progressive, hypopigmented lesion that is usually stable throughout an affected individual's lifetime. The clinical features of vitiligo are similar to those of ND, but the two diseases have different treatment responses and prognoses. We report here on a rare case of vitiligo that was coexistent with ND. Both conditions were treated with narrow-band UVB. An 11-year-old boy presented with two distinct types of hypopigmented lesions, one on the forehead and the other on his back. The first was a hypopigmented patch with leukotrichia, and it was incidentally discovered 3 months before the child was examined at our clinic and it had rapidly increased in size. The second hypopigmented patch was detected at birth and it had slowly been increasing in size. The hypopigmented lesion on the child's forehead was diagnosed as vitiligo, and the one on his back as ND. Once- or twice-weekly narrow-band UVB treatment was initiated. Improvements in the two lesions were assessed with clinical photography and using a Mexameter® (Courage-Khazaka Electronic, Germany), which is a pigment-measuring device.

Successful treatment of lichen spinulosus with topical tacalcitol cream.

Lichen spinulosus (LS) is a rare idiopathic cutaneous eruption characterized by follicular keratotic spiny papules that are grouped in large patches. Here, we report two cases of LS in the submental area, an uncommon site, which were treated effectively and safely with topical tacalcitol cream.

Reactive vascular lesions treated using ethanolamine oleate sclerotherapy.

BACKGROUND: Ethanolamine oleate (EO) is used infrequently in dermatology, but is used to treat vascular lesions such as esophageal varices, varicose veins, and congenital vascular malformations. OBJECTIVE: To evaluate the efficacy and safety of EO for treating reactive vascular lesions. MATERIALS AND METHODS: Patients with reactive vascular lesions, such as pyogenic granulomas or venous lakes, were enrolled. EO was used as a sclerosing agent in a 1:1 dilution with normal saline. According to the response, treatment was repeated with EO with less or no dilution. The treatment response was scored as complete remission (CR), moderate improvement (MI), or no change according to the clinical results; any side effects were recorded. RESULTS: The efficacy of EO was evaluated in 21 patients (16 pyogenic granulomas and 5 venous lakes). The diameters of the lesions ranged from 0.3 to 1.0 cm. The mean number of EO injections was 1.6 (range 1-4). A CR was achieved in 95% of the reactive vascular lesions (20 CR, 1 MI). Two episodes of transient pain occurred. CONCLUSION: EO is an excellent sclerosing agent for treating reactive vascular lesions, and it may be an alternative therapy for vascular lesions in dermatology.

A case of adams-oliver syndrome.

Adams-Oliver syndrome (AOS) is a congenital condition characterized by aplasia cutis congenita, transverse limb defects, and cutis marmorata telangiectatica. AOS can also be associated with extensive lethal anomalies of internal organs, including the central nervous, cardiopulmonary, gastrourointestinal, and genitourinary systems. Generally, the more severe these interrelated anomalies are, the poorer the prognosis becomes. In the relevant literature on this topic, it is somewhat unclear as to whether the prognosis of AOS without lethal anomalies alters the lifespan. We report a case of AOS with typical skin defects only, and no internal organ anomalies.

A case of linear focal elastosis with a family history.

Linear focal elastosis is an uncommon disorder typically occurring in the back region, which clinically presents as band-like striae, having a histological focal increase in abnormal elastic fibers. Until now, linear focal elastosis occurring in patients with a family history have been rarely reported. Here, we present one such case, of linear focal elastosis which occurred in a brother and sister.

Serum levels of IL-18 and sIL-2R in patients with alopecia areata receiving combined therapy with oral cyclosporine and steroids.

This study was to determine which immunologic factors contribute to the prognosis of patients with alopecia areata (AA) who were receiving oral cyclosporine A and methylprednisolone. Patients with > 25% hair regrowth were defined as responders, and patients exhibiting < or = 25% regrowth were poor-responders. The serum levels of IL-18 and soluble IL-2 receptor (sIL-2R) were measured at baseline in 21 patients with AA and 22 control subjects. The mean serum level of IL-18 in the patients with extensive AA was significantly higher than that in the control subjects. The mean serum concentration of sIL-2R in the AA patients significantly decreased after 1 month of treatment. The mean basal serum level of IL-18 was highest in the responder, whereas the baseline level of sIL-2R was significantly higher in the poor-responder group than other groups. In conclusion, increased serum sIL-2R level and lower IL-18 level at baseline was associated with a poor prognosis in patients with AA.

A case of hypermelanosis in a phylloid pattern.

Pigmentary anomalies display a variety of different patterns. The phylloid pattern is characterized by a leaf-shaped arrangement reminiscent of floral ornaments. We describe a 20-year-old Korean man with hyperpigmented oblong patches and atypically short, thick hairs in a phylloid pattern on the face, the left shoulder, and the left side of the trunk. Associated musculoskeletal anomalies included equinovarus deformities, bilateral club foot, lumbar lordosis, spina bifida, and relative hypertrophy of the right thigh muscles.

Clinical efficacy of 25% L-ascorbic acid (C'ensil) in the treatment of melasma.

BACKGROUND: L-Ascorbic acid is used to treat melasma; however, it is quickly oxidized in aqueous solutions. Thus, C'ensil, a formulation containing 25% l-ascorbic acid and a chemical penetration enhancer, was created to promote the penetration of l-ascorbic acid into the skin. OBJECTIVE: To evaluate the efficacy of C'ensil in patients with melasma. METHODS: Forty subjects with melasma were treated with C'ensil during an open-label trial over a period of 16 weeks. Each subject's skin pigmentation was assessed every 4 weeks using the Melasma Area and Severity Index (MASI) and mexameter score. In addition, transepidermal water loss, skin dryness and irritation, and quality of life (Melasma Quality of Life Scale [MelasQoL]) were evaluated. RESULTS: After 16 weeks, a significant decrease was noted in the degree of pigmentation based on the patients' MASI and mexameter scores. MelasQoL scores also decreased, indicating an increase in the subjects' quality of life. CONCLUSION: Our data indicate that C'ensil is an effective treatment modality for melasma.

Progressive macular hypomelanosis in korean patients: a clinicopathologic study.

BACKGROUND: Progressive macular hypomelanosis is characterized by ill-defined, non-scaly, hypopigmented macules primarily on the trunk of the body. Although numerous cases of progressive macular hypomelanosis have been reported, there have been no clinicopathologic studies of progressive macular hypomelanosis in Korean patients. OBJECTIVE: In this study we examined the clinical characteristics, histologic findings, and treatment methods for progressive macular hypomelanosis in a Korean population. METHODS: Between 1996 and 2005, 20 patients presented to the Department of Dermatology at Busan Paik Hospital with acquired, non-scaly, confluent, hypopigmented macules on the trunk, and with no history of inflammation or infection. The medical records, clinical photographs, and pathologic findings for each patient were examined. RESULTS: The patients included 5 men and 15 women. The mean age of onset was 21.05+/-3.47 years. The back was the most common site of involvement. All KOH examinations were negative. A Wood's lamp examination showed hypopigmented lesions compared with the adjacent normal skin. A microscopic examination showed a reduction in the number of melanin granules in the lesions compared with the adjacent normal skin, although S-100 immunohistochemical staining did not reveal significant differences in the number of melanocytes. Among the 20 patients, 7 received topical drug therapy, 6 were treated with narrow-band ultraviolet B phototherapy, 4 received oral minocycline, and 3 did not receive any treatment. CONCLUSION: Most of the patients with progressive macular hypomelanosis had asymptomatic ill-defined, non-scaly, and symmetric hypopigmented macules, especially on the back and abdomen. Histologically, the number of melanocytes did not differ significantly between the hypopigmented macules and the normal perilesional skin. No effective treatment is known for progressive macular hypomelanosis; however, narrow-band ultraviolet B phototherapy may be a useful treatment modality.

A case of syphilitic keratoderma concurrent with syphilitic uveitis.

Syphilitic keratoderma is a rare cutaneous manifestation of secondary syphilis, characterized by symmetrical and diffuse hyperkeratosis of the palms and soles. In addition, no cases of syphilitic keratoderma and uveitis have been reported in the dermatologic literature. A 69-year-old woman presented with steroid-resistant hyperkeratotic patches on the palms and soles and uveitis for 4 months. As steroid-resistant uveitis must be evaluated for syphilis, viral infections, and autoimmune diseases, we ran several laboratory tests and the serologic test for VDRL was reactive (titer; 1:128). After treatment with penicillin G (4 MU, IV every 4 hours for 2 weeks), her skin lesions and visual disturbance were completely resolved. Therefore she was diagnosed as having syphilitic keratoderma and uveitis. Here, we report a rare case of syphilitic keratoderma concurrent with syphilitic uveitis and suggest that evaluation for syphilis may be required when skin lesions and ocular disturbance are resistant to long-term steroid therapy.

Dermatofibrosarcoma protuberans arising from a burn scar.

Malignant neoplasms arising in burn scars are well known. In previous literature, 25 cases of burn scar sarcomas were reported. However, dermatofibrosarcoma protuberans is very rare and only two cases have been reported. A 43-year-old Korean man presented with multiple erythematous clustered plaques and nodules and a skin-colored subcutaneous mass on the chest after a severe burn injury at the age of 8 years. A biopsy specimen revealed dermatofibrosarcoma protuberans. The tumor was excised widely to include the surrounding burn scar. Herein, we report this third case of dermatofibrosarcoma protuberans arising from a burn scar.

A case of unilateral ashy dermatosis.

Ashy dermatosis, also known as erythema dyschromicum perstans, is a peculiar, slowly progressive, idiopathic dermal melanosis. In most cases, slate gray- to lead-colored patches are symmetrically distributed over the body. Ashy dermatosis with a unilateral distribution is rare. We report a case of unilateral ashy dermatosis in a 27-year-old Korean man.

A Hydroxyurea-induced Leg Ulcer.

Hydroxyurea is a cytostatic agent that has recently become the drug of choice in the treatment of various myeloproliferative diseases. The cutaneous side effects of hydroxyurea include xerosis, hyperpigmentation, nail discoloration, and scaling. Leg ulcers have only rarely been reported in association with hydroxyurea treatment. A 75-year-old woman presented with leg ulcers, nail discoloration, and xerosis. The leg ulcers were refractory to conventional treatment. She had been taking oral hydroxyurea since being diagnosed with essential thrombocytosis in 2002. Hence, we suspected hydroxyurea-induced leg ulcers and discontinued her hydroxyurea treatment; the ulcers gradually healed thereafter. We present a rare case of hydroxyurea-induced leg ulcers in Korea.

A case of acquired smooth muscle hamartoma on the sole.

A smooth muscle hamartoma is a benign proliferation of smooth muscle bundles within the dermis. It arises from smooth muscle cells that are located in arrector pili muscles, dartos muscles, vascular smooth muscles, muscularis mammillae and the areolae. Acquired smooth muscle hamartoma (ASMH) is rare, with only 10 such cases having been reported in the English medical literature to date. Most of these cases of ASMH were shown to have originated from arrector pili and dartos muscles. Only one case was reported to have originated from vascular smooth muscle cells. A 21 year-old woman presented with a tender pigmented nodule, with numbness, on the sole of her foot, and this lesion had developed over the previous 18 months. The lesion showed no hyperpigmentation or hypertrichosis, and the biopsies demonstrated increased smooth muscle bundles in the dermis, and especially around the blood vessels. Moreover, the specimens stained positive with Masson trichrome stain and alpha-smooth-muscle actin antibodies, thus supporting our diagnosis of ASMH of the foot sole. Herein, we report on a rare case of ASMH on the foot sole, and this lesion originated from vascular smooth muscle cells. This type of case has not been previously described in the English medical literature.