Seon Wook Hwang

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.

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.

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 Woods 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.

Changes in murine hair with dietary selenium excess or deficiency

Abstract:  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.

Primary non-essential cutis verticis gyrata revealed with 3-D magnetic resonance imaging.

Sir, Cutis vertices gyrata (CVG) is characterized by thickening of the scalp, which becomes raised to form ridges and furrows resembling the cerebral gyri, which cannot be flattened by traction or pressure. The diagnosis is based on clinical features, and further investigations are needed to demonstrate the precise typical features. We describe how three-dimensional magnetic resonance imaging (3D MRI) can be used to visualize the extent of the lesions in primary non-essential CVG.

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.

Two Pilosebaceous Cysts with Apocrine Hidrocystoma in One Biopsy Site: A Spectrum of the Same Disease Process?

A 28-year-old woman presented with multiple, asymptomatic, erythematous to bluish papules located on the chest. Histopathologically, three round, well defined cystic structures were seen on the upper and lower dermis. The first cyst was milia, the second was apocrine hidrocystoma and the other, largest cyst was an eruptive vellus hair cyst (EVHC). A diagnosis of multiple pilosebaceous cysts combined with apocrine hidrocystoma was made. Since the milia and EVHC originate from the pilosebaceous unit, and the apocrine duct opens to the pilosebaceous orifice, we suggest that they can occur simultaneously in the same unit.

Treatment of Severe Alopecia Areata: Combination Therapy Using Systemic Cyclosporine A with Low Dose Corticosteroids

BACKGROUND Combination therapy using cyclosporine A (CsA) together with low-dose corticosteroids has adequate efficacy with little toxicity for the treatment of severe alopecia areata (AA). OBJECTIVE We wanted to evaluate the clinical efficacy of combination therapy using CsA with low-dose corticosteroid for the treatment of severe AA and we also wanted to determine the safe therapeutic concentration of CsA in the peripheral blood. METHODS We treated 34 cases of severe AA with combination therapy for 24 weeks and we evaluated the efficacy at 12 and 24 weeks. We monitored the peripheral blood concentration of CsA to determine the therapeutic range of CsA that has the fewest side effects. RESULTS Of the patients, 77.4% (n=24) and 22.6% (n=10) were classified in the responder and poor-responder groups, respectively. The mean trough concentration of CsA was 95.1 and 101.2 ng/ml in the responder and poor-responder groups, respectively. For the patients with side effects associated with CsA, the mean CsA concentration was 195.8 ng/ml. CONCLUSION We found that combination therapy with systemic CsA and low-dose corticosteroids effectively treats severe AA and this therapy results in a safe, therapeutic concentration of CsA in the peripheral blood.