Back issues No.1 - 2010  
  Paraneoplastic pemphigus
Sang Eun Lee, Soo-Chan Kim

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune mucocutaneous blistering disease associated with malignancy, particularly lymphoproliferative neoplasms. Clinically, it is characterized by severe and intractable mucositis and polymorphous cutaneous eruptions, ranging from blisters to lichenoid lesions. The histologic features are also diverse according to the morphology of the clinical lesions, ranging from suprabasal acantholysis to interface changes with necrotic keratinocy ..................More
  Original Study
  Performance of the SCORTEN in Taiwanese patients with Stevens-Johnson syndrome and toxic epidermal necrolysis
Yu-Ling Ho, Yun-Ting Chang, Yu-Tseng Chu, Shiao-Chi Wu

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare drug-related skin conditions that are potentially life-threatening with a 20–30% mortality rate. A severity-of-illness score specified for SJS and TEN, SCORTEN, was developed in 2000. Until now, no study of SCORTEN has been conducted in Asian with SJS or TEN. The goal of the present study is to evaluate the performance of SCORTEN in predicting in-hospital mortality in Taiwanese patient with SJS or TEN. ..................More
  Linear IgA bullous dermatosis: a clinical study of 16 cases at National Taiwan University Hospital
I-Chen Tsai, Chia-Yu Chu, Hsiang-Jung Chen, Li-Fang Wang, Hsien-Ching Chiu

Background Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease. It is defined by continuous linear deposition of IgA in the basement membrane zone on direct immunofluorescence microscopy. The clinical presentations of LABD may mimic other diseases, and data in Taiwanese populations are still lacking. The current study aims to examine LABD status in Taiwan. Methods We reviewed the database at our institute from 1995 to 2008. The gold standard ..................More
  Case Report
  Naproxen-induced pseudolymphoma syndrome: a case report
Shang-Hong Lin, Cheng-Yu Wang, Ji-Chen Ho, Wei-Ming Wu

Drug-induced pseudolymphoma syndrome is a rare form of adverse cutaneous drug reaction. Its clinical and histological presentation may mimic mycosis fungoides, which sometimes leads to misdiagnosis and unnecessary treatments. We describe the case of a 65-year-old man with a generalized pruritic and confluent maculopapular eruption. His history of skin lesions was concordant with medication and resolved after discontinuation. However, microscopic examination showed characteristic features of ..................More
  Deep dermatofibrosarcoma protuberans: a pitfall in the ultrasonographic diagnosis of lipoma-like subcutaneous lesions
Po-Yu Shih, Chien-Hsun Chen, Tseng-Tong Kuo, Chin-Yi Yang, Yu-Huei Huang, Chih-Hsun Yang

Dermatofibrosarcoma protuberans (DFSP) is an indolent sarcoma known for its propensity for local invasive growth and recurrence. It typically presents as a protuberant tumor mass. Rare nonprotuberant presentations have recently been described; these invariably present as pigmented or depressed plaques. Lesions arising in the subcutaneous compartment and without cutaneous manifestations have rarely been reported or emphasized in the literature. Here we report a case of deep DFSP that lacked ..................More
  Localized pemphigus herpetiformis: two case reports
Cheng-Chieh Huang, Yu-Hung Wu, Chi-Fu Kao

Pemphigus herpetiformis (PHF) is a rare variant of pemphigus that combines the clinical features of dermatitis herpetiformis and immunopathological features of pemphigus. In previous case reports, distribution of lesions is usually generalized, involving trunk and limbs. We report two cases of localized PHF presenting with grouped pustules on bilateral dorsal feet and left preauricular area, respectively. Microscopic examination showed neutrophilic spongiosis, subcorneal pustules, and focal ..................More
  Atrichia with papular lesions in a Taiwanese patient without hairless (HR) gene mutation
Hung-Chien Lin, Julia Yu-Yun Lee, Jeng-Hsien Lin, Sheau-Chiou Chao

Atrichia with papular lesions is a rare form of hair loss with an autosomal recessive mode of inheritance characterized by the absence of normal hair follicles and formation of intradermal cystic structures. Mutations in the hairless (HR) gene in both mice and human have been implicated in the development of this phenotype. HR codes for a putative transcription factor containing a single zinc-finger DNA binding domain, with restricted expression in the brain and the skin. Here, we report th ..................More
  Unilateral nevoid telangiectasia—response to long-pulsed Nd:YAG laser and pulsed dye laser: a case report
Lu-An Chen, Tsung-Hua Tsai, Ying-Jui Chang, Lin-Hui Su, Yu-Ling Hsu, Jung-Yi Chan

Unilateral nevoid telangiectasia (UNT) is a rare disease characterized by punctuate and stellate telangiectasias that follow dermatomal distribution.1 Its etiology remains elusive; no standard treatment currently exists. We describe a 14-year-old Asian female (skin type IV) with UNT, who underwent treatment with long-pulsed 1064-nm Nd:YAG laser and 595-nm pulsed dye laser for her disease. The patient came to our clinic with linearly arranged, punctate telangiectasias over right side of b ..................More
  Juvenile pityriasis rubra pilaris: a case report with immunohistochemical and electromicroscopic studies
Jeng-Feng Chen, Hong-Wei Gao, Wei-Ming Wang

A 5-year-old male child was brought to our clinic with itchy hyperkeratotic scaly eruptions on the palms and soles which appeared 2 months ago. He was otherwise healthy and born full term by an uneventful vaginal delivery. Subsequently, similar skin lesions developed on his face, ears and scalp. The cephalocaudal spreading of hyperkeratotic lesions to his elbows, sacral area, lower abdomen, groins and knees was noted in the following 2 months. In addition, he was also found to have palmo ..................More
  Resident Forum
  An erythematous plaque in the left thumb of a 58-year-old woman
Chun-Hung Hsu, Wei-Yu Chen, Chi-Long Chen, Tsung-Hsien Tsai

A 58-year-old woman presented with an ill-defined erythematous plaque on volar aspect of the promixal interphalangeal joint of her left thumb for 3 months. The plaque (1.0 cm × 0.7 cm) was mildly elastic and tender on palpation (Figure 1). There was no scaling, vesiculation or ulceration. She denied previous trauma in this area. Upon physical examination, no regional lymphadenopathy or systemic finding was noted. An incisional biopsy of the plaque was performed. Pathological sections showed ..................More
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