Monji Koga

A case of discoid lupus erythematosus of the eyelid

A 39‐year‐old man was first evaluated 12 years ago for erosive erythema of the left lower eyelid. The response to topical therapy was poor. The eyelid lesion was excised on the patients demand 6 years ago and blepharoplasty was performed. However, recurrence of erythematous plaques at the same site prompted referral to our department. Examination clinically revealed atrophic erythematous plaques and several white papules on the left lower eyelid, in addition to an atrophic erythema of the upper back. The histopathological findings of both plaques and papules were typical of discoid lupus erythematosus. Immunoserological findings suggestive of systemic lupus erythematosus were absent. The lesions improved with administration of low‐dose corticosteroids. Discoid lupus erythematosus involving the eyelid is rare, and definitive diagnosis often takes considerable period of time. In patients with lesions of the eyelid such as erythema or papules that are refractory to treatment, skin biopsy and careful physical examination of the entire body for similar lesions are important.

Median Raphe Cyst with Ciliated Cells of the Penis

Median raphe cysts of the penis are uncommon benign lesions that affect young men. The cysts are lined with pseudostratified columnar or stratified squamous cell epithelium, mimicking the epithelial lining of the male urethra. Immunohistochemical study also indicates a histogenetic relationship with the urothelium. To our knowledge, there have been only 2 reports of ciliated epithelium in this kind of cyst published previously (1, 2). We describe here a rare case of median raphe cyst of the penis with ciliated cells, and discuss the histopatho-logical and immunohistochemical features.CASE REPORT

Proactive treatment with calcipotriol reduces recurrence of plaque psoriasis

Topical calcipotriol is a widely used treatment for plaque‐type psoriasis worldwide, and has been shown to improve psoriatic plaques as well as very potent corticosteroids. However, there remains the practical question of whether calcipotriol application should continue on healed pigmentation/depigmentation associated with psoriatic plaques. Therefore, we conducted a pilot clinical study to answer this question. Plaque‐type psoriatic patients not receiving systemic treatment were enrolled and treated with calcipotriol for 8 weeks (stage I) to achieve maximum effect. The patients were then divided into two groups: group A continued to apply calcipotriol to the entirety of the previous lesion (including pigmentation/depigmentation) regardless of whether skin was healed or not, while group B applied calcipotriol to the remaining lesion only. Patients were followed for 12 weeks (stage II) and dates of plaque recurrence were recorded. A total of 29 patients (13 men, 16 women) were enrolled. During stage I, reductions in scores for redness, induration and scale occurred in 40%, 47% and 55% of patients, respectively. After stage II was completed, group A (n = 19) showed a significantly better Kaplan–Meier curve of non‐recurrence than group B (n = 8, P < 0.01). The mean non‐recurrence duration was 76.8 ± 11.8 in group A and 35.0 ± 12.0 in group B. Our study showed that applying topical calcipotriol on seemingly healed psoriatic plaque lesions suppresses recurrence better than applying it only on remaining plaques. This finding may be important for instructing psoriatic patients on topical calcipotriol treatment.

Anthropometric characteristics and comorbidities in Japanese patients with neurofibromatosis type 1: A single institutional case–control study

Neurofibromatosis type 1 (NF1) is a well‐known genetic disorder characterized by café‐au‐lait spots and neurofibromas, but many other clinical characteristics and associated comorbidities also have been reported. This study aimed to characterize NF1 further by investigating its association with anthropometric characteristics and other diseases. We performed a case–control study of 227 NF1 patients (101 male, 126 female) and a randomly selected age‐ and sex‐matched control group of 681 non‐NF1 patients (303 male, 378 female) who visited our institution in Japan. We examined adult (≥20 years) height and body mass index (BMI), and, in the total sample, allergic diseases (bronchial asthma [BA], atopic dermatitis [AD] and allergic rhinitis) and other respiratory cardiovascular and psychiatric disorders. In adults, the mean BMI was lower in the NF1 group than in the control group, and was significantly statistically different among men (P = 0.0238). In the whole sample, the prevalences of BA (P = 0.0184), AD (P = 0.0144) and valvular heart disease (P = 0.0166) were significantly greater in the NF1 group than in the control group. To date, no similar research on the BMI or the prevalence of allergic disease in NF1 patients has been reported. Our results suggest that NF1 patients tend to have lower BMI and may have alterations in specific metabolic pathways and altered allergic immunity.

Development of miliary tuberculosis related to anti‐tumor necrosis factor‐alpha inhibitor therapy for pustular psoriasis

ing agents is not mediated by adiponectin, the alteration of serum adipokines would not be the specific action of TNF-a blocking agents but rather be the result of decreased serum TNF-a levels by the successful psoriasis treatments including ustekinumab and NB-UVB. In conclusion, we demonstrated that improvements of serum levels of adiponectin, leptin and resistin are linked to successful treatment of psoriasis. Because these adipokines are significantly involved in “psoriatic march”, adequate control of psoriasis may contribute to the decreased development of metabolic syndrome including cardiovascular disease.

A pediatric case of pityriasis rubra pilaris successfully treated with low-dose vitamin a.

Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory keratosis that is clinically characterized by gradually developing reddish or orange extending plaques and keratotic follicular papules. In pediatric patients, we frequently hesitate to administer certain medications for treatment of PRP, specifically etretinate, systemic corticosteroids, and biologics recommended by previous studies. Although administration of high-dose vitamin A was described in a previous textbook of dermatology, details about the lower limits and treatment periods were not provided. We presented a pediatric case of PRP that was successfully controlled with minimum dosage of systemic vitamin A in the literature. Before and 14 days after beginning the therapy, both vitamin A levels of peripheral blood were within the normal range. We considered that the clinical efficacy may not be due to a supplementary effect of vitamin A, but to a pharmacological action because serum vitamin A was within the normal limits during the therapy.

Effects of rotation of topical vitamin D3 in chronic plaque‐type psoriasis

the healthy control group. We concluded that untreated psoriatic patients are characterized by a quantitative deficit in their peripheral circulating DC. MDC and pDC subsets were also evaluated after infliximab therapy. Responders (patients who had 75% improvement or more in PASI after 6 months of therapy) showed a substantial increase in their number of circulating DC (Table 1). Specifically, mDC frequency increased from 3.2% ± 0.3% to 7.8% ± 1.8% (P < 0.05), whereas there was a blood pDC level increase, but was not statistically significant (from 1.3% ± 0.4% to 3.8% ± 2.7%, P = 0.16). Next, we set out to determine if numerical alterations of blood DC were associated also with phenotypic or functional abnormalities. Because the expression of co-stimulatory molecules on the surface of DC has been correlated to their immunostimulatory capacity, we evaluated the expression of CD86 in these patients, before and during infliximab therapy and we compared it to the controls. The expression of this antigen is shown in Table 1. Patients before and after treatment did not show a significant variation in the expression of CD86. Our preliminary results, although in a limited number of patients, suggest that the increased recruitment of DC into cutaneous plaques might be responsible for decreased peripheral DC and their relative lower percentage in untreated patients. If this is the case, it seemed likely that effective therapy might block this migration and increase the blood DC level. According to our results there is an alteration in circulating DC which could correlate with the persistence of the disease and the response to biologic therapy. Francesca PRIGNANO, Federica RICCERI, Matteo BECCATTI, Paola DI GENNARO, Claudia FIORILLO, Torello LOTTI, Niccolo TADDEI Division of Clinical, Preventive and Oncology Dermatology, Department of Critical Care Medicine and Surgery, and Department of Biochemical Sciences, University of Florence, Florence, Italy

Subcutaneous Metastasis due to Primary Central Nervous System Malignant Lymphoma

© 2009 The Authors. doi: 10.2340/00015555-0640 Journal Compilation

Nutritional, muscular and metabolic characteristics in patients with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) has many reported clinical characteristics. We previously found that NF1 patients (especially men) had lower body mass index (BMI) than controls, but the reason has not been elucidated. To address this issue, a retrospectively case–control study was conducted. Anthropometric and serum chemistry data that potentially relate to BMI were collected from medical records of NF1 patients and their age‐ and sex‐matched controls. Enrollment of 98 adult patients who underwent skin surgery with NF1 (41 men, 57 women) and 173 without NF1 (74 men, 99 women) were investigated. The median BMI in male NF1 patients was significantly lower than that of the controls. Triglycerides in male NF1 patients were significantly lower than male controls, creatine kinase and lactate dehydrogenase in NF1 patients were also lower than controls, aspartate aminotransferase and alanine aminotransferase showed a lower tendency in NF1 patients, but were significantly lower in female patients. With correlation analysis, lactate dehydrogenase was moderately correlated with BMI in male NF1 patients. Creatine kinase and creatinine showed no statistical correlation with BMI in either group. Triglycerides and alanine aminotransferase showed a positive correlation with BMI in both male and female controls, but not in NF1 patients. In conclusion, only lactate dehydrogenase was moderately correlated with BMI in male NF1 patients, although results of some nutritional and metabolic parameters suggest a specific metabolism in NF1.

Spontaneous regression of an epidermolysis bullosa nevus on the sole

Epidermolysis bullosa (EB) is a group of inherited rare diseases characterized by fragility and blistering of the skin and mucous membranes. An EB nevus (EBN) is a very rare acquired nevus that occurs only in patients with EB. An EBN usually arises in an area of previous blistering, and frequently has an atypical appearance that mimics malignant melanoma. We describe an older man with an EBN on the sole. Physical examination revealed irregularly pigmented band‐like black macules on his right sole, suggestive of malignant melanoma. Dermoscopic examination showed a parallel furrow pattern with a monotonous area. The histopathological findings were consistent with a benign acquired nevus. Interestingly, the lesion regressed spontaneously within a year. To the best of our knowledge, this is the first reported case of EBN on the sole. Dermoscopy may be a useful addition to histopathological examination to ensure correct diagnosis of EBN.