A 69-year-old female had appetite loss, dry cough, slight fever and multiple joint pains in June 2005 and subsequently dark red erythema developed on her body in August. She had heliotrope eyelids, Gottron?s sign and interstitial pneumonia. She did not complain of muscular weakness, and skeletal muscle enzymes were not elevated, although a high serum KL-6 level was observed. Her skin lesions disappeared after 40 mg of prednisolone per day was administered. In late August, the interstitial pulmonary disease worsened. At the same time she developed multiple skin ulcers on her tongue, right second finger, right shoulder, and left buttock in addition to pustules on the upper back and lumbar area. Pulmonary symptoms responded to methylprednisolone pulse therapy, and the KL-6 level decreased but the skin ulcers on her buttock and shoulder were resistant to this therapy. After high dose intravenous immunoglobulin pulse therapy and tacrolimus with oral prednisolone, ulcers stopped enlarging and surgical closure was performed. In December 2005 when tacrolimus was stopped for 2 months on suspicion of drug-induced liver dysfunction, the KL-6 level became elevated and erythema of her trunk again worsened. After the readministration of tacrolimus, the erythema almost completely resolved and the KL-6 level slowly decreased without any side effects. With this treatment we were able to taper the dose of oral prednisolone and concluded that tacrolimus was effective in our patient.

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We report an 80-year-old female with dermatomyositis. When she visited our hospital, she had experienced general malaise with muscle weakness, poikiloderma of the upper chest, and edematous erythema with itching on the neck and waist. Dermatomyositis was diagnosed according to skin biopsy of the neck lesion, thigh MRI, and blood examinations. After daily administration of 40 mg of oral corticosteroid, symptoms and laboratory data improved. However, exacerbation of the erythema and an increased level of aldolase were observed to be caused by cytomegalovirus infection that was revealed by cytomegalovirus antigenemia assay. Intravenous infusion of Ganciclovir 5 mg/kg for 14 days improved skin lesions and laboratory data, and dosage of corticosteroid was reduced. Cytomegalovirus infection is suggested to be involved in the exacerbation of dermatomyositis in this case.

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We report a 47-year old male patient who had dermatomyositis with skin ulcers. He had swollen fingers with erythema and arthralgia 9 months before visiting our hospital. Having suspected collagen disease at a former clinic, daily oral prednisolone of 12 mg/day was administered without any symptom improvement. In addition, skin ulcers of dorsal manus and back, muscle weakness, and dyspnea on exertion gradually developed in the patient. In December, 2005, he visited our hospital, and dermatomyositis was diagnosed according to the presence of the heliotrope rashes, Gottron’s signs, and his histological findings of skin lesions. Chest computed tomography and echocardiography revealed interstitial pneumonia and mild pulmonary hypertension. After a steroid pulse therapy, oral predonisolone of 60 mg/day was administered in combination with oral cyclosporine A 200mg/day and beraprost sodium. Skin ulcers were managed by topical basic fibroblast growth factor and prostaglandin E1 ointments. Pulmonary hypertension was also managed by beraprost sodium. Although therapeutic responses of interstitial pneumonia and pulmonary hypertension were relatively good and rapid, the complete healing of skin ulcers required about 7 month’s treatment. No malignant tumors could be found.

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A 42-year-old woman noticed itchy papules in the occipital region 3 months ago. Since the papules increased gradually in number, she visited a nearby hospital, where she received treatment under the diagnosis of folliculitis. However, since she saw no satisfactory response to the treatment, she visited our department. Histopathological examination of biopsy specimens obtained from the lesions revealed the presence of spongiosis in part of the epidermis and perifollicular dense inflammatory cell infiltration with destruction of the follicular wall. The inflammatory cell infiltrate consisted mainly of lymphocytes, histiocytes and eosinophils. Treatment with indomethacin at a daily dose of 75 mg was started; however, since no improvement of itchy papules was noted in response to this treatment, the drug was switched to DDS at the dose of 75 mg daily. One month later, the eruption had almost completely disappeared. Only a limited number of cases of eruption on the head have been reported from Japan, and our present case was the first one presenting with lesions localized to the head that were covered with hair. When encountering cases of papules and pustules on the head covered with hair associated with pruritus, the possibility of the diagnosis in the present case should be taken into consideration.

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A 57-year-old male had noticed a pruritic eruption with a linear arrangement on the left forearm from the age of 50 years. He had been treated with topical steroids and vitamin D₃, but treatment was not effective. At the first visit to our division, from the left forearm to the left shoulder the nodules followed the distribution of the lines of Blaschko. Histopathologically, acanthosis and thickening of the epidermis with elongation of rete ridges with a psoriasiform appearance were revealed. Perivascular infiltration of lymphocytes was seen in the upper dermis. A sharply demarcated alternation of orthokeratotic hyperkeratosis with hypergranulosis and parakeratosis lacking a granular layer was seen. We assigned the diagnosis of inflammatory linear verrucous epidermal nevus (ILVEN) from the viewpoint of clinical and histopathological findings. Of ILVEN patients, 75% are recognized before the age of 5 years, but our case was noticed at the age of 50 years. Adult onset of ILVEN is rare, and we have reviewed cases reported in the literature.

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We report a case of microcystic adnexal carcinoma (MAC) with basal cell carcinoma (BCC). This 66-year-old male had small black papules on his head beginning 10 years previously. When he came to our hospital in September 2005, he had an erythematous plaque about 4 cm in size, next to which were the black papules. We resected the tumor, and histopathological examination revealed that the erythematous plaque was MAC and the black papules were BCC. This is the only case in which MAC is coexistent with BCC in our country. We also report points that differentiate MAC from BCC.

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The patient was a 34-year-old man with a high fever and muscular pain in all four extremities. High serum concentrations of soluble IL-2 receptor and ferritin were observed. Positron emission tomographic images showed nodular accumulations in multiple subcutaneous lesions across the entire body. Erythematous nodules with tenderness and erythema with induration appeared repeatedly. Histological findings of skin biopsy specimen revealed lobular panniculitis with infiltration of histiocytes, atypical lymphocytes and histiocytes containing nuclear dust in the cytoplasm. Since atypical lymphocytes were positive for CD3, CD8, TIA-1, perforin, granzyme B and TCRβ, but negative for CD4, CD56 and EBER, it is considered that they were derived from cytotoxic T cells. CHOP therapy was administered on the basis of the diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Subsequently, the skin eruptions and fever disappeared. SPTCL is a skin-origin T cell lymphoma that causes high fever, liver dysfunction, and pancytopenia. Furthermore, it induces a hemophagocytotic syndrome with a poor prognosis. SPTCL should be considered in the case of refractory, cytopenic and recurrent erythema nodosum-like eruptions.

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A 72-year-old Japanese man noticed eruptions with pain on the right upper back in January 2004. The following day, the patient consulted a nearby dermatologist and was administered an analgesic and topical ointment. The same kind of eruptions appeared on the right subcostal and upper abdomen two days later and small vesicles began to form. He was referred to the outpatient section of the dermatology department of our hospital and was immediately hospitalized. He had a history of chronic rheumatoid arthritis over a period of 15 years and had been treated with oral administration of prednisolone and methotrexate. Five years previously he had stomach ulcer. It was not clear whether he had ever had chicken pox, but he had never had herpes zoster. Initial physical examination revealed grouped red papules and crusted vesicles on the right shoulder and right upper arm (C4∼5 dermatomes). The same eruptions were detected on the right lower back, right flank, and subcostal abdomen (Th8∼9 dermatomes). Those findings suggested the diagnosis of unilateral double herpes zoster involving two dermatomes. Administration of anti-viral agent (aciclovir) resulted in marked improvement in symptoms. However, one week after the treatment, he experienced difficulty in lifting his right upper extremity. This condition was diagnosed as motor paralysis as a complication of unilateral double herpes zoster.

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We have diagnosed 4 cases of early syphilis resens at Kyushu University Hospital (Fukuoka, Japan) in a relatively short period (from November 2005 to June 2006), although syphilis is rarely seen in a university clinic these days. The first 2 of the 4 cases were males with refractory genital ulcers and were diagnosed as having typical primary syphilis. The other 2 cases were females with secondary syphilis who developed erythematous papules. One of the females had a high fever, inguinal lymph node swelling and liver dysfunction and previously had been misdiagnosed as having a viral infection in many other clinics, including a dermatology clinic. The skin eruptions and systemic symptoms rapidly improved after the administration of amoxicillin (750-1000 mg/day) for 4 to 7 weeks. During treatment, the syphilis markers, STS and TPHA, were also improved along with the symptoms of secondary syphilis. However, STS, but not necessarily TPHA, reflected the clinical course of primary syphilis in accordance with previous reports. After the historical finding of amoxicillin in 1942, the disease became curable and the diagnosis of syphilis might not be thought of as significant, as in previous times. However, there have been many reports of concomitant syphilitic cases with HIV infection, and Japan is one of the few developed countries where AIDS patients and HIV carriers have been increasing in number. It is thought that the existence of genital ulcerations may allow easy HIV entry through sexual activities. Furthermore, moderate outbreaks of syphilis have occurred in a roughly 20-year cycle in Japan since the end of World War II (1945) for some unknown reasons. According to statistics, such outbreak-peaks seem to come several years after in smaller cities such as Fukuoka. Thus, we would like to point out that diagnostic capabilities for the diagnosis of syphilis are again significant today.

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We experienced a 2-year-old boy with cranial fasciitis. A subcutaneous tumor had appeared in his right temporal region one month before presentation. The tumor was not painful or tender but rapidly increased in size. Total resection of the tumor was performed. The border of the tumor was clear, and the underlying skull bone was not involved. Histopathological examination revealed spindle cells and multinucleated giant cells. Therefore, the lesion was diagnosed as cranial fasciitis. We reviewed characteristics of cranial fasciitis that have been reported previously.

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We examined Candida species on the surface of the tongue in a total of 109 cases of Sjögren syndrome (Sjs: 34 cases), suspected Sjs (42 cases)and non-Sjs (33 cases). There were 17 males and 92 females aged 61.1±11.7 years. We divided these patients into two groups according to the average salivary fluid volume secreted while chewing gum for 10 minutes before and after breakfast and before and after dinner for 3 days. Groups were the dry mouth group (less than 10 ml) and non-dry mouth group (more than 10 ml). The surface of the tongue was rubbed 10 times with cotton swabs before a sample for culture was obtained, placed on CHROMagar-Candida slants and checked by API C oxanogram within 3 inoculation days. Five or more colonies were obtained in 160 of 188 samples (85.1%), only one species of Candida was found in 39 samples and 2 to 4 colonies were found in 44 samples. The former consisted of Candida albicans (C. albicans) in 28 (71.8%) samples, C. tropicalis in 3, C. famata in 2, C. glabrata in 1, Cryprococcus neoformans (C. neoformans)in 1, and unknown in 4 samples. C. albicans and another species were found in 33 samples, and in 11 samples other combinations that did not include C. albicans were found. Overall, C. albicans was counted in 63, C. glabrata in 40, C. tropicalis in 14, C. dubliniensis in 6, C. parapsilosis in 6, etc. Many colonies were obtained in the Sjs group in contrast to the suspected Sjs and non-Sjs groups. Especially, C. albicans was predominantly obtained in the suspected Sjs group. In addition, other species, with the exception of C. albicans, were predominantly obtained in the dry mouth group (p<0.05). There was no statistical significance between other species except for C. albicans and C. glabrata. These results can be speculated to be caused by a decrease in salivary secretion and an abnormal immunological response.

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We evaluated the clinical usefulness of loratadine rapidly disintegrating tablets (Claritin^®, RediTabs^®) in 102 patients with a variety of pruritic skin diseases including atopic dermatitis and urticaria, who presented at the outpatient clinic of the Department of Dermatology of Kagawa University Hospital and affiliated hospitals. A questionnaire survey of these patients was also conducted to assess the ease of oral administration and usefulness of this drug. The overall patients evaluation of the “ease of ingestion,” including related factors such as taste and rapid dissolution, was “good” in 72 patients (70.6%), “neither good nor bad” in 25 (24.5%), and “bad” in 1 (1.0%). As for previously forgetting to take oral medicines, 81 patients (79.4%) responded “yes” and 21 (20.6%) responded “no.” As for taking loratadine rapidly disintegrating tablets in this study, 69 patients (67.6%) responded that they “rarely forgot to take,” 24 (23.5%) that they “sometimes forgot to take,” and 6 (5.9%) that they ” often forgot to take.” The percentage of patients who forgot to take loratadine rapidly disintegrating tablets was lower than that for conventional oral medicines (20.6% vs. 67.6%). As for the effect of loratadine rapidly disintegrating tablets on itching, 9 of 47 patients (19.1%) responded that loratadine rapidly disintegrating tablets was “better” and 35 of 47 (74.5%) responded that loratadine was “neither better nor worse” than previous treatment with second-generation non-sedative antihistamines. This result indicated that 93.6% of the patients evaluated loratadine rapidly disintegrating tablets to be about as effective as previous treatment with second-generation antihistamines. Among the 47 patients who previously received second-generation antihistamines, 23 patients (48.9%) reported “no drowsiness,” as compared with 74.5% of the patients who received loratadine rapidly disintegrating tablets. The incidence of drowsiness was significantly lower with loratadine rapidly disintegrating tablets (p=0.0027 by the McNemar test). These results showed that loratadine rapidly disintegrating tablets have excellent ease of administration when used to treat pruritic skin diseases. The clinical usefulness and efficacy of loratadine rapidly disintegrating tablets was clearly confirmed on the basis of surveys of patients as well as physicians.

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The efficacy of low concentration tacalcitol with concomitant bath-PUVA therapy in maintaining improvement achieved by treatment with high concentration tacalcitol was studied in 10 psoriasis vulgaris patients. In an inpatient facility, patients received both applications of high-concentration tacalcitol ointment and bath-PUVA therapy once daily. Tacalcitol was switched from a high concentration to low concentration once a 60% remission rate was achieved and was applied once daily. As a result, the mean PASI score was decreased to 9.5 after 8 weeks compared to 22.3 at baseline with significant improvement in skin findings. The mean duration from initiation of treatment with high concentration tacalcitol to switch to low concentration was 21 days. Of the 8 patients who finally started maintenance therapy, in 5 patients the outcome remained the same or improved compared to the condition at start of maintenance therapy. Once-daily topical tacalcitol application with concomitant bath-PUVA therapy was shown to be sufficiently efficacious even after switching to low concentrations of tacalcitol.

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We administered cyclosporine A (NEORAL^®)at a daily dose of 2.5 mg/kg before breakfast to seven patients with psoriasis, and measured the drug level at 1 hour after dosing (C1). Relatively rapid improvement of skin manifestations was achieved. Especially in patients with moderate to severe psoriasis and a PASI score of more than twelve points, there was 74.8% improvement of the PASI score at four weeks and 81.6% improvement of the score after eight weeks. As adverse effects, three out of seven patients developed an increase of blood pressure and complained of feeling dull and of flushing. These symptoms were more likely to occur in the patients with relatively high C1 levels (that is more than 1000 ng/ml). In conclusion, administration of NEORAL^®once daily before breakfast seems to be a useful method to induce the remission of psoriasis, but further assessment should be done.

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We studied low doses of Ciclosporin for short-term treatment in 26 patients with psoriasis vulgaris. The microemulsion form of Ciclosporin was given once under fasting conditions every morning at a dose of 2.0∼2.5mg/body weight daily. This dose was maintained for a maximum of 12 weeks, and it was reduced gradually. PASI (Psoriasis Area and Severity Index) was assessed for its efficacy. Within the 12 weeks, 75% or more improvement in PASI was achieved in 62.5% of the patients, with more than 50% improvement in 91.7%. Hypertension occurred in 2 cases during the study, but the serum creatinine concentration and liver function did not show any clinically significant changes. Our results show that low dose, short term therapy with Ciclosporin provides effective control of psoriasis without severe toxicity. In addition, although tapering off Ciclosporin induces psoriasis relapse in 9 cases among 14 cases, topical therapy maintained the improvement even after the cessation of Ciclosporin in other 5 cases. Such a different effect after the administration is clinically interesting, and therapies are still necessary for suppressing the relapse.

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