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Multiple autoimmune syndrome (MAS) is a rare condition, in which at least three well-defined autoimmune diseases co-occur in a single patient. Although the pathogenesis of MAS remains poorly understood, genetic, immunological, hormonal, and environmental factors may all play a role. Although three classic subtypes of MAS are well known, several cases that could not be placed into any of these three categories have recently been reported. To the best of our knowledge, the co-existence of psoriasis, vitiligo, and Crohn's disease has been reported in only one patient, who had a total of five autoimmune diseases (alopecia areata and oral lichen planus in addition to the three conditions mentioned). We herein describe a rare case of MAS presenting with psoriasis, vitiligo, and Crohn's disease, and suggest that tumor necrosis factor-α may be associated with the pathogenesis of all three conditions.

Folliculocystic and collagen hamartoma is a newly described complex hamartoma characterized by abundant collagen deposition, concentric perifollicular fibrosis, and keratin- filled infundibular cysts that are visible on histopathological examination. Here, we report the case of a 19-year-old Korean man who had large brownish infiltrated plaques with numerous follicular comedo-like openings and subcutaneous cystic masses on his right temporal scalp and ear since birth. Histopathological examination showed abundant collagen deposition in the dermis that extended up to the subcutaneous fat layer, multifocal infundibular cysts packed with keratin, and perifollicular inflammation and fibrosis. Hence, we describe a new type of hamartoma with folliculocystic and collagen components but without tuberous sclerosis.

Dear Editor: Unilateral nevoid telangiectasia (UNT) syndrome is a rare vascular dermatosis, characterized by areas of superficial telangiectases distributed in a dermatomal, unilateral pattern usually on the upper trunk and extremities. The etiopathogenesis is not completely understood, but the most widely accepted theory is its association with hyperestrogenic condition1. Herein, we report a rare case of UNT accompanied by liver metastasis of colon cancer. A 26-year-old male presented with progressive asymptomatic erythematous telangiectactic patches localized to the left chest and abdomen of 5-month duration. Three months after from when he had noticed skin lesions, he was diagnosed with colon cancer with multiple metastases at lumbar spine, lung, intra-abdominal lymph nodes including the liver and started chemotherapy following the FOLFIRI (fluorouracil, leucovorin, and irinotecan) regimen. He had no history of hepatitis or alcoholism. No family members were known to have any similar condition. On physical examination, there were no palmar erythema, ascites, or hepatosplenomegaly suggestive of chronic liver disease. On examination of the skin, erythematous telangiectactic patches on the left chest and abdomen involving dermatomes C3-4 and T4-10 were observed (Fig. 1). Biopsy specimen of the skin revealed intact epidermis with thin-walled capillaries in the superficial dermis (Fig. 2). Immunohistochemical staining was negative for estrogen, progesterone, and vascular endothelial growth factor (VEGF) receptors. Laboratory investigations were within normal range including liver function test. However, circulating estrogen level (155.55 pg/ml; normal range, 40~115 pg/ml) and serum carcinoembryonic antigen level (329.50 ng/ml; normal range, 0~4.7 ng/ml), which may be due to colon cancer and multiple metastases. Based on clinical and histopathologic findings, a diagnosis of UNT syndrome accompanied by liver metastasis of colon cancer was made. Reported cases of UNT syndrome mostly occur in women during pregnancy or puberty and in men with alcoholic cirrhosis2. Rarely, it has also been described in patients with carcinoid tumor of stomach with liver metastasis3. Several cases reported in recent years have cast doubt on the view that estrogen is the causal agent of telangiectasia development because of normal estrogen levels or negative estrogen and progesterone receptors1,4. Turan et al.4 suggested that VEGF may play a role in the pathogenesis of UNT syndrome in patients with underlying hepatic disease. On the other hand, Smith et al.5 reported a case of UNT syndrome accompanied by chronic hepatitis C virus infection, with a normal serum estrogen profile and VEGF level but strong positive immunohistochemical staining of lesional skin with VEGF. They suggested this result supports the hypothesis that angiogenic factors play a role in the pathogenesis of UNT syndrome. VEGF is already known to be a commonly expressed angiogenic factor in human colorectal cancer metastases and high serum VEGF level is correlated with colon cancer liver metastasis. Despite being unable to check serum VEGF level, elevated estrogen and VEGF levels could be related to the pathogenesis of UNT syndrome in our patient who had colon cancer with multiple metastases including the liver. To our knowledge, this is the first report of UNT syndrome accompanied by liver metastasis of colon cancer. Fig. 1 (A) Multiple erythematous telangiectactic patches localized to left chest (12×8 cm sized). (B) abdomen (27×21 cm sized). Fig. 2 Normal epidermis with dilated thin-walled capillaries in the superficial dermis (H&E, ×100).

Dear Editor: Erythrodermic psoriasis is a severe form of psoriasis, presenting as prominent erythema rather than thick scales, affecting the entirebody including the face, trunk, extremities, and nails. The disease poses a therapeutic challenge, and numerous treatment choices have been examined1. Ustekinumab (Stelara; Jannssen Biotech Inc., Horsham, PA, USA) is a human monoclonal antibody targeting the p40 subunit shared by interleukin (IL)-12 and IL-23. This biological agent has shown good efficacy against plaque-type psoriasis and emerging effectiveness in a variety of inflammatory skin diseases such as atopic dermatitis2. Erythrodermic psoriasis has been an exclusion criterion in studies evaluating the efficacy of ustekinumab for psoriasis, but recently, a few reports have proven its efficacy in treating erythrodermic psoriasis3,4,5. Herein, we report 2 cases of erythrodermic psoriasis that successfully improved following ustekinumab treatment. A 42-year-old man presented with a 7-year history of erythrodermic psoriasis and a 5-year history of psoriatic arthritis. He was previously treated with cyclosporine A, methotrexate, and narrow band ultraviolet B phototherapy. The treatments were interrupted due to liver enzyme elevation and low efficacy. The patient was treated with ustekinumab 45 mg subcutaneously at 0, week 4, and every 12 weeks thereafter. The patient was also diagnosed with latent pulmonary tuberculosis by the QuantiFERON-TB Gold In-Tube test (Cellestis Limited, Carnegie, VIC, Australia). He was concurrently treated with isoniazid 300 mg/day for 6 months and has not experienced a recurrence of tuberculosis to date. At week 28, his Psoriasis Area and Severity Index (PASI) score decreased from 64.8 (baseline) to 9.6 (Fig. 1). The psoriasis aggravated temporarily during discontinuation of the treatment for 5 months and he promptly recovered upon restarting ustekinumab injections. His arthritis symptoms improved slightly, but the improvement was not sufficient for him to stop taking oral sulfasalazine and steroid for the arthralgia. Fig. 1 A 42-year-old man presented with whole body erythema with scanty scales. He responded well to ustekinumab (A: baseline, B: 28 weeks after the treatment, C: 76 weeks after the treatment). The second case is a 26-year-old man suffering from erythrodermic psoriasis for 10 years. Therapies with conventional systemic agents and anti-tumornecrosis factor were discontinued due to lack of efficacy. Four months after the first administration of ustekinumab (45 mg), the patient achieved 75% improvement of the PASI score from 50.9 (baseline) to 4.4 (Fig. 2A, B). Currently, the patient has received treatment for 112 weeks, and is maintaining a PASI 90 improvement (Fig. 2C). Fig. 2 A 26-year-old man. (A) Baseline: normal skin islands are noticeable within the diffuse erythroderma. Clinical improvement was significant and sustained (B) 28 weeks after the treatment and (C) 112 weeks after the treatment, respectively. Both patients are currently on treatment, and show sustained responses without any adverse reactions or exacerbations. Although further studies are needed to evaluate the efficacy and safety of ustekinumab, it is likely that ustekinumab can be an effective therapeutic option for patients affected by erythrodermic psoriasis where other treatments have failed.