Autosomal recessive type 2 pseudoxanthoma elasticum presenting with generalized skin laxity.

Herein, we describe a sporadic case of recessive type 2 pseudoxanthoma elasticum. A 26-year-old woman without family history presented with cutis laxa-like marked wrinkling involving the whole-body and a serpiginous streak on the upper left arm. She denied any other systemic problems related to difficulty with visual acuity or vascular disease. A skin biopsy specimen from the loose skin showed the accumulation of calcified degenerated elastic fibers and foci of ossification in the dermis. Histopathological study from a serpiginous streak revealed mineralized debris that was eliminated through the epidermis, the finding consistent with elastosis perforans serpiginosa. Recessive type 2 pseudoxanthoma elasticum is very rare and the presenting case is interesting in that this patient presented with lesions of secondary ossification and elastosis perforans serpiginosa in association with pseudoxanthoma elasticum.

Cutaneous metastasis from large-cell neuroendocrine carcinoma of the urinary bladder expressing CK20 and TTF-1.

Large-cell neuroendocrine carcinoma (LCNEC) of the urinary bladder is very rare. Immunohistochemical and biochemical examinations have shown that neuroendocrine carcinomas (NECs) have features of neuroendocrine and epithelial differentiation. We describe the first case of cutaneous metastasis from LCNEC of the urinary bladder. The patient had been treated with partial cystectomy and chemotherapy for LCNEC of the urinary bladder, but a year later, he visited our clinic with a reddish mass on his scalp that was diagnosed as a cutaneous metastasis from LCNEC. The tumor cells were positive for the neuroendocrine markers, cytokeratin (CK) 20 and thyroid transcription factor-1. Most NECs, except for Merkel cell carcinomas, do not express CK20, whereas most urothelial carcinomas do express CK20. These results suggest that a histogenetic link may exist between NEC of the urinary bladder and urothelial carcinoma.

A case of tinea manuum caused by Trichophyton mentagrophytes var. erinacei: the first isolation in Korea.

Hedgehogs have recently become popular exotic pets in Korea. Several diseases may be acquired from these animals including dermatophytosis, Salmonella infections, contact urticaria and possibly Mycobacterium infections. We describe here a 15-year-old girl who developed tinea manuum after keeping a hedgehog for 4 months. Diagnosis was confirmed by histopathologic and mycological examination. The causative agent was identified as Trichophyton mentagrophytes var. erinacei, a zoophilic dermatophyte.

Q-Switched Nd:YAG Laser Therapy of Acquired Bilateral Nevus of Ota-like Macules.

BACKGROUND: Acquired bilateral nevus of Ota-like macules (ABNOM) is a dermal pigmented lesion common in individuals of Oriental origin. The Q-switched Nd:YAG laser (QSNYL) has been used successfully to treat a variety of benign, dermal, pigmented lesions, including nevus of Ota lesions. The similarity between ABNOM and nevus of Ota suggested that QSNYL may also be effective in the former. OBJECTIVE: To determine the efficacy and side-effect profiles of QSNYL treatment of ABNOM in Korean patients. METHODS: Of 42 Korean patients with ABNOM, 29 were treated with QSNYL (1,064 nm, 3 mm spot size, fluence 8~9.5 J/cm(2)), for up to 10 sessions each. Clinical photographs were taken before and after treatment. Lesion clearance was graded and complications such as hyperpigmentation, scarring, hypopigmentation, and erythema were assessed. RESULTS: Of the 29 treated patients, 19 (66%) showed excellent or good results. Of the patients who were treated more than 3 times, 76% showed good to excellent results. Two patients experienced post-laser hyperpigmentation (PLH), which persisted for more than one month, but no patient experienced persistent erythema or hypertrophic scarring. CONCLUSION: QSNYL is safe and effective in the treatment of ABNOM in Korean patients. Short-interval repetitive treatment is especially useful in improving therapeutic results and reducing PLH.

Cutaneous metastasis of extraskeletal osteosarcoma arising in the mediastinum.

Cutaneous metastases have been reported in 5%-10% of patients with internal malignancy. The most common clinical features are the sudden appearance of discrete, firm, and nontender nodules in a particular area of the body. The most frequent metastatic site of extraskeletal osteosarcoma (ESOS) is the lungs. Cutaneous metastasis of ESOS is rare. To our knowledge, only one case of ESOS has been reported with skin metastasis, histopathologically classified as the osteoblastic subtype. Here, we describe a case of chondroblastic ESOS arising in the mediastinum with metastasis to the skin. Our patient presented with a scalp nodule after diagnosis of primary ESOS at the mediastinum.

Extramammary Paget's disease in Korea: its association with gastrointestinal neoplasms.

BACKGROUND: Extramammary Paget's disease (EMPD) most commonly occurs in the perineal and genital areas of elderly people. The current treatment of choice is adequate surgical excision. MATERIALS AND METHODS: Between 1990 and 2007, 28 patients (27 men and one woman) with EMPD were treated at Asan Medical Center, Seoul, Korea, by wide local excision (WLE) with a 2- to 3-cm normal skin margin. "Carcinoma in situ" (CIS) was defined as confinement of Paget cells to the epithelium, whereas "invasive carcinoma" was defined as infiltration of Paget cells into more than dermal connective tissue. RESULTS: Of the 28 lesions, 21 (75%) were located in the penoscrotal area. Six (21.4%) patients had associated gastrointestinal neoplasms, three with associated gastrointestinal malignancies and four with synchronous colorectal adenomas, including one with both. Surgical procedures included WLE with primary repair in 13 patients (46.4%), WLE with skin graft in 12 (42.9%), WLE with skin graft and Gracilis muscle transposition in two (7.1%), and combined abdominoperineal resection and distal gastrectomy in one (3.6%). Four patients (14.3%) also underwent inguinal lymph node dissection, with three found to have lymph node metastases. Of 11 patients with invasive carcinoma, three (27.3%) had lymph node metastases, compared with none of 17 patients with CIS. Patients with lymph node metastases showed a significantly lower disease-specific survival rate (P = 0.008). Patients with invasive carcinoma tended to have a lower disease-specific survival rate (P = 0.087). CONCLUSIONS: EMPD in Korea showed an absolute male predominance and an association with gastrointestinal neoplasms. Lymph node metastasis significantly affected, and depth of invasion tended to affect, disease-specific survival rate.

Papular Mucinosis Associated with Systemic Lupus Erythematosus.

Papulonodular mucinosis (PNM) is a rare variant of lupus erythematosus (LE) eruptions, and PNM is characterized histologically by diffuse dermal mucin without any typical epidermal inflammatory changes. We herein describe a case of papular mucinosis that was characterized by several erythematous papules on the lower back of a 32-year-old man with systemic LE. It is interesting that he didn't display any other skin manifestations of LE such as malar rash, discoid rash and photosensitivity during the previous 2 years. He achieved remission of his PNM without recurrence after 5 months treatment with topical steroids, in addition to receiving systemic antimalarials and steroids.