A Case of Eccrine Spiradenoma in a Patient with Neurofibromatosis

Neurofibromatosis is a systemic hereditary disorder with varied manifestations in bone, soft tissue, the nervous system and skin. Cutaneous manifestations of neurofibromatosis are characterized by cafe-au-lait macules, multiple neurofibromas, Lisch nodules and intertriginous freckling. Some benign or malignant tumors such as juvenile xanthogranuloma, pheochromocytoma, and malignant melanoma can accompany neurofibromatosis. But, in the English literature, no case of eccrine spiradenoma associated with neurofibromatosis has been reported. Eccrine spiradenoma is a benign uncommon neoplasm of skin adnexa. It presents as a painful, slow-growing and solitary nodule on the head or upper trunk. Here, we report a rare case of eccrine spiradenoma in a patient with neurofibromatosis.

Congenital Zinc Deficiency from Mutations of the SLC39A4 Gene as the Genetic Background of Acrodermatitis Enteropathica

Acrodermatitis enteropathica (AE) is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. AE is known to be caused by mutations of the SLC39A4 gene on the chromosome band 8q24.3, encoding the zinc transporter in human. An 8-month-old Korean boy presented with eczematous changes on the inguinal area and knees and was diagnosed with AE. Blood tests revealed a markedly decreased level of plasma zinc, and his symptoms improved on oral zinc replacement. To confirm the diagnosis of AE from congenital zinc deficiency, direct sequencing analysis of SLC39A4 was performed and revealed that he was compound heterozygous for a known missense mutation (Arg95Cys) and a novel splicing mutation in the donor site of intron 7 (c.1287+2T>C). Family study showed that his parents were heterozygous carriers of the mutations. To the best of our knowledge, this is the first report of genetically confirmed AE in Korea.

A Case of Gardner's Syndrome Associated with Desmoid Tumor

Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner's syndrome. Herein, we report a 16-year-old woman with Gardner's syndrome complicated by desmoid tumors on the right subscapular area.

Therapeutic Effects of High Fluence Pulsed Dye Laser for Periungual Warts / 대한피부과학회지

BACKGROUND: Viral warts are benign epithelial proliferations induced by the human papillomavirus (HPV) and they affect 7~10% of the population. Some warts such as periungual warts are resistant to various treatment modalities, including cryotherapy, surgical excision, carbon dioxide laser therapy and immune therapy. Thus, some studies have reported that pulsed dye laser has been effectively used for treating recalcitrant warts. OBJECTIVE: The purpose of this study was to evaluate the therapeutic response and safety of pulsed dye laser using high fluence for the treatment of periungual warts. METHODS: 66 periungual warts on 28 patients were treated with pulsed dye laser. The treatment parameters were a spot size of 5.0 mm, a pulse duration of 0.45~1.5 ms and a fluence of 14.0~15.0 J/cm2. The warts were evaluated at 2~3 week intervals to assess the clearance rate and side effects. RESULTS: Complete clearance was achieved for 47 out of 66 periungual warts (71.2%). The average number of treatment sessions was 2 (range: 1~6). After the first session, complete clearance was achieved for 24 out of 66 warts (36.4%). No significant side effects were observed except for pain during and after the procedure. During a median follow up period of 7 months (range: 3~15 months), 10 periungual warts on 3 patients recurred. CONCLUSION: Pulsed dye laser using high fluence could be used as a safe and successful treatment for recalcitrant periungual warts.

A Case of Subcutaneous Benign Fibrous Histiocytoma / 대한피부과학회지

Benign fibrous histiocytoma is a common benign fibrohistiocytic tumor that's usually confined to the dermis. A 20-year-old female presented with a skin-colored, hard subcutaneous nodule of 1 cm diameter on her right flank. The histopathologic findings showed a well circumscribed, purely subcutaneous nodule composed of a fibrohistiocytic proliferation, and this was consistent with subcutaneous benign fibrous histiocytoma. We report here on a rare variant of benign fibrous histiocytoma that was confined to the subcutaneous layer without any epidermal and dermal changes.

A Case of Becker Nevus Syndrome with a Tooth Defect andFibrous Dysplasia of the Sphenoid Bone / 대한피부과학회지

Becker nevus syndrome is a phenotype characterized by the presence of a Becker nevus in association with unilateral hypoplasia of the breast or other cutaneous, muscular or skeletal defects. We report an interesting case of Becker's nevus syndrome associated with fibrous dysplasia of the sphenoid bone, tooth abnormalities and facial asymmetry.

A Case of Milker's Nodule / 대한피부과학회지

Milker's nodule is a harmless skin lesion that's caused by the paravaccinia virus (also called pseudocowpox virus), which is a member of the genus Parapoxviridae. It occurs worldwide, but it is prevalent only in individuals who are in close contact with cattle. We report here on a woman who presented with a dusky purpuric center surrounded by a white or gray ring and an outer red halo on the back of the right hand. Histologically, intracytoplasmic inclusion bodies were evident in the upper epidermis, and these revealed cylindrical virus particles on electron microscopy. Parapoxvirus was identified by polymerase chain reaction with using pan-parapoxvirus primer 1 (PPP-1) and PPP-4.

A Case of Generalized Granuloma Annulare That Clinically Presented as Erythema Annulare Centrifugum / 대한피부과학회지

Granuloma annulare (GA) is usually a benign, self-limiting, granulomatous, chronic dermatosis of an unknown cause, characterized by skin colored papules that often have an annular configuration. Generalized granuloma annulare (GGA) is an uncommon variant of GA, and this is characterized by disseminated papules on the trunk and limbs. We report here on an interesting case of GGA that clinically presented as erythema annulare centrifugum.

A Case of Cystic Clear Cell Hidradenoma / 대한피부과학회지

Clear cell hidradenoma (CCH) is a relatively rare benign tumor that shows differentiation toward the structure of an eccrine sweat gland. The tumor masses are composed of solid areas and tubular lamina. Cystic spaces are often present and on rare occasions they can be of considerable size. The cystic variant of CCH is referred as solid cystic hidradenoma. We present a case of cystic clear cell hidradenoma that occurred on the abdomen in a 42-year-old man. The tumor contained a solid tumor of CCH encircled by a large cystic space, and the cystic space was lined by two to four layers of epithelium.

A Study on CAG Repeat Polymorphisms of the Androgen Receptor in Korean Androgenetic Alopecia : Preliminary Report / 대한피부과학회지

BACKGROUND: The androgen receptor (AR) is a conserved member of the nuclear receptor superfamily. Differences in the AR gene sequence are characterized mostly by a highly polymorphic trinucleotide repeat (CAG) encoding a polyglutamine stretch in the N-terminal domain. The transactivational activity of the AR might be inversely associated with the numbers of this CAG repeat chain, and the smaller numbers of CAG repeats are believed to be associated with androgenetic alopecia? (AGA). OBJECTIVE: The purpose of this study was to investigate a possible etiologic association between Korean AGA and CAG repeat numbers in the AR gene. METHODS: We compared CAG repeat numbers within the AR gene of 64 male Korean AGA patients with those of 40 normal male controls. RESULTS: There was no significant difference in the number of CAG repeats between the Korean AGA patients and controls. There were no robust or significant correlations between (i) CAG repeat numbers and (ii) age of onset or severity of AGA in Korean AGA patients. CONCLUSION: This study suggests that AR receptor CAG polymorphisms in the Korean male population might not have a major role in susceptibility to AGA expression.

A Study on CAG Repeat Polymorphisms of the Androgen Receptor in Korean Androgenetic Alopecia : Preliminary Report / 대한피부과학회지

BACKGROUND: The androgen receptor (AR) is a conserved member of the nuclear receptor superfamily. Differences in the AR gene sequence are characterized mostly by a highly polymorphic trinucleotide repeat (CAG) encoding a polyglutamine stretch in the N-terminal domain. The transactivational activity of the AR might be inversely associated with the numbers of this CAG repeat chain, and the smaller numbers of CAG repeats are believed to be associated with androgenetic alopecia? (AGA). OBJECTIVE: The purpose of this study was to investigate a possible etiologic association between Korean AGA and CAG repeat numbers in the AR gene. METHODS: We compared CAG repeat numbers within the AR gene of 64 male Korean AGA patients with those of 40 normal male controls. RESULTS: There was no significant difference in the number of CAG repeats between the Korean AGA patients and controls. There were no robust or significant correlations between (i) CAG repeat numbers and (ii) age of onset or severity of AGA in Korean AGA patients. CONCLUSION: This study suggests that AR receptor CAG polymorphisms in the Korean male population might not have a major role in susceptibility to AGA expression.

A Case of Acquired Zinc Deficiency in a Patient with Ischemic Colitis / 대한피부과학회지

Zinc deficiency is characterized by acrodermatitis enteropathica like eruption such as periorificial dermatitis, diarrhea and mental irritability. This syndrome occurs due to decreased supplementation, increased consumption or decreased bowel absorption of zinc. We report here on a case of acquired zinc deficiency in a patient who was receiving total parenteral nutrition due to ischemic colitis. She showed denuded patches on the perinasal, perioral and perineal area and tense bullae on both hands. She was suffering from diarrhea and mental irritability. Her blood zinc level was 4.9microng/dl. Subsequent intravenous zinc supplementation cleared up her clinical manifestations.

Two Cases of Linear Alopecia on the Occipital Scalp

Alopecia of a scalp shows various shapes and extents of hair loss, from a small round patch to polymorphous patches or total global alopecia. But alopecia of a linear shape is very rare. Only a few such cases have currently been reported in the medical literature. We recently had the chance to observe and treat two cases of linear alopecia that developed on the occipital scalp. The lesions themselves were like alopecia areata that shows a smooth bald area without any abnormality except the hair loss, but histopathologically, the lesions were compatible with lupus erythematosus profundus.

Two Cases of Linear Alopecia on the Occipital Scalp

Alopecia of a scalp shows various shapes and extents of hair loss, from a small round patch to polymorphous patches or total global alopecia. But alopecia of a linear shape is very rare. Only a few such cases have currently been reported in the medical literature. We recently had the chance to observe and treat two cases of linear alopecia that developed on the occipital scalp. The lesions themselves were like alopecia areata that shows a smooth bald area without any abnormality except the hair loss, but histopathologically, the lesions were compatible with lupus erythematosus profundus.

A Case of Trichloroethylene Hypersensitivity Syndrome / 대한피부과학회지

Trichloroethylene (TCE) is a chlorinated hydrocarbon that is widely used as a degreasing agent in industry. Systemic absorption of this chemical can result in neurological, hepatic and cutaneous toxicity. Several cutaneous manifestations of TCE-exposed patients have been reported, including Stevens Johnson syndrome, subcorneal pustular eruption and generalized pustular eruption. We report here on an unusual case of TCE hypersensitivity syndrome in a 38 year old man after occupational exposure to TCE.

Therapeutic Effect of Oral Itraconazole for Verruca Plana / 대한피부과학회지

BACKGROUND: Despite numerous therapeutic methods (destructive surgery, topical application or systemic administration of various medicaments), the treatment of verruca plana still remains unsatisfactory and some novel remedies have been required. The authors noticed incidentally the clearance of verruca plana lesions in a patient who received itraconazole for tinea pedis. OBJECTIVE: The purpose of this study was to investigate the therapeutic effects of itraconazole for verruca plana. METHODS: The patients with verruca plana were treated with itraconazole (200 mg/day for adults, and 100 mg/day for children below 10 years of age) for 2 weeks. Assessment of clinical response and occurrence of the side effects were examined at weekly intervals. RESULTS: Of the 20 patients, 11 patients (55.0%) had either complete (40.0%) or partial clearance (15.0%) of verruca plana within 2 weeks of itraconazole therapy without severe adverse effects. No significant differences in therapeutic responses were found with regard to ages, sexes and duration of the diseases. CONCLUSION: This study demonstrates that oral itraconazole could be an effective and safe candidate in the treatment of verruca plana.

Two Cases of Trichorrhexis Nodosa / 대한피부과학회지

Trichorrhexis nodosa is the most common hair shaft anomaly in which there is a chemical or physical traumatic injury, characterized by the development of node-like swelling, fragility of the shaft and paint brush effect on the side of the fracture. We report two cases of trichorrhexis nodosa in 15-year-old girl and 16-year-old-boy, occurring as results of repetitive hot-comb straightener and excessive scratching. Scanning electroscopic findings are typical to trichorrhexis nodosa.

A Case of White Fibrous Papulosis of the Neck / 대한피부과학회지

White fibrous papulosis of the neck is a rare disorder, characterized by asymptomatic white papules that appear around neck in elderly people. The histopathologic findings showed thickened collagen bundles in the papillary to mid dermis. We report a case of white fibrous papulosis of the neck in a 53-year-old man.

A Case of Grouped Premature Sebaceous Hyperplasia / 대한피부과학회지

Sebaceous hyperplasia is not a rare disorder and commonly develops on the face over the age of forty. The typical clinical manifestation is 2~3 mm sized, single or multiple, soft, protuberant, yellowish nodules with central umbilication. Histological examination reveals a group of mature sebaceous lobules and central sebaceous ducts. We describe an unusual case of a 28-year old female patient with grouped premature sebaceous hyperplasia. The lesions presented clinically as grouped, yellowish, umbilicated papules on the left cheek, and had developed at 10 years of age.

A Case of Taxane-induced Onycholysis and Subungual Abscesses

A 35-year-old woman with advanced lung cancer, treated with intravenous docetaxel and paclitaxel, developed subungual abscesses and secondary onycholysis involving all the finger nails. Bacterial culture demonstrated the growth of Pseudomonas aeruginosa. We report a case of onycholysis and subungual abscesses in a patient treated with docetaxel and paclitaxel.