A 63-year-old female noticed a brown pigmented spot on her left hip at the end of 2008 and an erythematous eruption on her hands and extremities in February 2009. Interstitial pneumonia was noted on her chest X-ray and the patient was admitted to our hospital at the middle of May 2009. The diagnosis of dermatomyositis (DM) was made based on the presence of a typical Gottron-sign on her hands and extremities. An electromyogram showed slight myopathy, but muscle enzymes such as CK, aldolase and myoglobin were within normal limits and she had no obvious sign of muscle weakness. Chest high-resolution computed tomography revealed NSIP pattern interstitial pneumonia. Consequently, we diagnosed her with clinically amyopathic dermatomyositis with interstitial pneumonia. Although treatment with 1mg/kg/day of prednisolone was started and additional cyclosporin A with steroid pulse therapy was administered, intractable skin ulcer and pneumomedastinum developed during the treatment. After the introduction of tacrolimus in place of cyclosporin A, the patient's breathing state appeared to stabilize. However, the patient subsequently developed aspiration pneumonia 5 month after her admission. Although aspiration pneumonia was temporarily improved by antibiotic therapy, interstitial pneumonia caused the patient's condition to deteriorate rapidly and she died due to respiratory failure. It is well known that interstitial pneumonia associated with CADM is resistant to various treatments and, as a result, presents a poor prognosis. Although there is no sufficient evidence of therapy for DM associated interstitial pneumonia, some reports suggest early intervention with immunosuppressive agents result in a favorable prognosis. Precise evaluation of interstitial pneumonia and intensive therapy with immunosuppressive agents at early stage is very important for this life-threatening disorder.

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A 52-year-old woman had suffered from Raynaud's symptom for 10 years. A diagnosis of systemic sclerosis (limited type) was made on the basis of clinical and laboratory findings and histopathology of a skin biopsy. The year after diagnosis, she presented with pruritis. Oral administration of antihistamines is temporarily effective against pruritis, but the pruritis recurred 4 months later. Moreover, she noticed erythema on her extremities and swelling on her left upper limb. Laboratory investigation revealed hypereosinophilia, and histological examination showed infiltration of numerous eosinophils in the dermis with prominent flame figure. Based on these findings, a diagnosis of Wells' syndrome with systemic sclerosis was made. Oral corticosteroid was effective, but the erythema and pruritus recurred when administration was stopped or the dose was tapered.

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Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant vascular dysplasia associated with frequent episodes of nosebleeds. The majority of HHT patients have mutations in either endoglin (ENG) or activin receptor-like kinase 1 gene (ACVRL 1), which encode proteins that modulate transforming growth factor (TGF)-β superfamily signaling in vascular endothelial cells. ENG mutations are most common among Japanese HHT patients. We report a 39-year-old man who had often had nosebleeds since childhood. He had multiple tiny red papules on the oral mucosa, tongue and lip. A biopsy from the lip showed multiple dilated vessels in the upper dermis. Systemic examinations revealed arteriovenous malformations (AVMs) in the left lung, cerebellum and small intestine. Somatic mutation in the ENG gene was also found, and we therefore diagnosed him as having HHT type 1.

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A 72-year-old woman presenting for routine medical examination was found to have occult blood in the stool three months before the first admission. She received sigmoidoscopy by a nearby doctor, and was found to have a black, ulcerated tumor between anus and rectum. A biopsy of the lesion confirmed the diagnosis of malignant melanoma, and therefore, she was admitted to Kumamoto University hospital. At the first admission, endoscopic investigations showed a 10 × 10 mm black tumor with ulceration the anal lesion and another depigmented tumor measuring 15 × 15 mm on the rectum. There were no metastatic lesions found after a general examination including PET-CT, therefore we excised the rectum and anus by laparoscopy. The tumor thickness was 9 mm and we diagnosed the patient as pT4bN0M0, Stage IIc. We are now performing postoperative chemotherapy (DAV-feron).

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We herein report a 54 years old female patient suffering with general arthralgia, erythema on the face and multiple elastic hard reddish cutaneous nodules on the head, back, waist and dorsum of fingers for eight months. The diagnosis as multicentric reticulohistiocytosis (MR) was confirmed by biopsy from cutaneous nodule of finger, including an infiltrate composed of histiocytes and multinucleated giant cells with eosinophilic cytoplasm. In addition, study of Ga scintigraphy showed many abnormal uptakes in not only lesional skin but also normal skin, suggesting extended widely. Furthermore, biopsy from synovial membrane of knee also indicated similar histological features of skin. With this result, this case was diagnosed as polyarthritis due to MR. In this report, we presented some points of differential diagnosis about polyarthritis by MR and rheumatoid arthritis because those diffential diagnosis is hard to distinguish.

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A 57-year-old woman presented with appetite loss, malaise and dyspnea in August 2007. Chest radiograph showed left pleural effusion and thickening of the left pleura, and thoracic drainage was performed. Positron emission tomography (PET) showed multiple sites of uptake within the left pleura indicating malignant tumors. No uptake was seen in other parts of her body. An open lung biopsy disclosed multiple black-colored masses widely disseminated in the left thoracic cavity, and partial resection was performed. Pathological diagnosis was malignant melanoma. She was admitted to our hospital in September 2007. Repeated physical examinations failed to reveal any site for the primary melanoma in the skin, eyes, oral cavity, or genital or anal regions, and neither computed tomography (CT) nor PET showed masses in other parts of her body. Thus, a diagnosis of primary malignant melanoma of the pleura was made. Beginning in October 2007, she received 2 courses of DACTam (DTIC, ACNU, CDDP and Tamoxifen) therapy, a single course of CDV (CDDP, DTIC, VDS) therapy, and then 6 courses of triweekly paclitaxel therapy. But metastasis to the liver and abdominal lymph nodes appeared. Chemotherapy was discontinued and beginning in August 2008, she was treated with subcutaneous injection of IFN-β every other week. However, she died from multiple metastases in May 2009, a year and 9 months after the diagnosis.

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A 60-year-old male presented with a melanotic macule on the right heel for 20 years. The pigmented lesion was histopathologically examined by punch biopsy and diagnosed as lentigo simplex at another clinic 3 years previously. Therefore, the lesion was untreated after diagnosis, but it began to enlarge and to exhibit enhanced shading. Clinical examination revealed a 70 × 40 mm pigmented lesion on the right heel. The lesion showed a parallel ridge pattern and depigmentation on dermoscopy. Malignant melanoma was suspected and excisional biopsy was performed. Based on these clinical and histopathological findings, the lesion was diagnosed as acral lentiginous melanoma in situ. It was unclear whether the malignant melanoma had developed before or after the biopsy at the previous clinic. As incisional biopsy may not always reflect the state of the whole lesion, excisional biopsy should be recommended if possible in cases suspicious for malignant melanoma.

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A 77-year-old man was referred to our department with a red plaque on the right hand. He had been prone to frequent injury because of his farm work, and he had been treated with oral prednisolone (10 mg/day) for 20 years due to emphysema. Histopathological examination revealed granulomatous changes and brown fungal elements with hyphal forms in the dermis. We diagnosed this case as having phaeohyphomycosis. Complete surgical excision and postoperative oral itraconazole 100 mg/day led to a clinical resolution. Based on the morphological findings in the mycological cultures from the tissue and restriction fragment length polymorphism analysis of the internal transcribed space regions of ribosomal RNA genes, the isolate was identified as E. jeanselmei, type 5.

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Food-dependent salicylate-induced anaphylaxis (FDSIA) is a type of anaphylaxis caused by the simultaneous ingestion of a substance that contains salicylic acid and a causative food. We report on an FDSIA patient (male, 58 years old) who suffered anaphylaxis as a result of simultaneous ingestion of wheat and acetylsalicylic acid. This patient experienced wheals 3 or 4 times a year after ingestion of bread, Chinese noodles, hamburger, etc. The patient visited this department because of systemic wheals and dyspnea that occurred after ingestion of buckwheat noodles on April 2008. According to the laboratory test findings, the IgE RAST for wheat and gluten were assessed as class 2 and class 3, respectively. After treatment comprising a drip infusion of steroid and oral administration of an antiallergy drug, the symptoms remitted. When a loading test was conducted on another day, no anaphylaxis was induced by wheat ingestion + exercise loading and aspirin ingestion alone. However, when he ingested wheat after aspirin intake, systemic wheals and erythema occurred after 1 hour. Since IgE that is specific to ω-5 gliadin was found in the serum, our patient was diagnosed as having FDSIA caused by wheat.

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The modified Rodnan total skin thickness score (m-Rodnan TSS) is generally used for quantitative evaluation of dermal sclerosis in patients with systemic scleroderma (scleroderma). Physicians with experience in treating scleroderma patients believe that this scoring method is excellent in terms of the reproducibility, although involvement of a subjective element in the examination is undeniable. Tissue strain imaging (TSI) with an ultrasonographic diagnostics system (Toshiba Medical Systems Inc.) has been introduced recently for quantitative analysis of the skin stiffness. The degree of stiffness of the dermis and subcutaneous tissue evaluated by this in vivo method, commonly known as strain measurement, at sites of dermal sclerosis in 11 scleroderma patients visiting the Department of Dermatology, Dokkyo Medical University Hospital, was compared with that measured at the corresponding sites in 18 healthy volunteers matched for gender and age. The strain measurement allows the elasticity of the dermal and deeper layers of the skin to be objectively and quantitatively expressed as the strain value. Strain values as measured by this method decrease with increasing degree of dermal sclerosis. On the side of the extended forearm, the strain value tended to be low in relation to the skin scores for the dermis and fat layers. In particular, there were significant differences in the scores for the dermis and fat layers between the scleroderma patients with high skin scores (2-3) and the healthy volunteers. These results indicate that the strain measurement is useful as an objective measurement technique, that can aid in the conventional evaluation by m-Rodnan TSS, of dermal sclerosis in scleroderma patients. It is considered that the strain measurement, the results of which are not influenced by the examiner's experience, may become useful in clinical practice for making the diagnosis of scleroderma and for judging the therapeutic efficacy for this disease in the future.

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In patients in whom treatment using anti-allergic agents is not effective for the itching associated with atopic dermatitis and other conditions, a change in medication is often attempted. However there have been a limited number of reported studies investigating the effectiveness of this approach. Therefore, we studied the efficacy of loratadine in patients whose treatment with other antihistamines was ineffective using SCORAD index, Visual Analogue Scale (VAS), and Dermatology Life Quality Index (DLQI). Twenty patients (17 patients with atopic dermatitis, 3 patients with other pruritic skin diseases) were treated with loratadine 5∼10 mg, once daily for 4 weeks and skin symptoms (SCORAD, VAS) and QOL (DLQI) were evaluated. At the end of the study, skin symptoms significantly improved from 31.6 at baseline to 15.2 after 4 weeks of treatment. Itching improved from 60.8 at baseline to 27.0 after 4 weeks (1st week : 40.1, 2nd week : 33.2, 4th week : 27.2), and sleeplessness due to itching improved from 31.1 at baseline to 9.2 (1st week : 18.2, 2nd week : 13.2, 4th week : 8.0). Both measurements showed significant improvement. The improvements were observed less than a week after administration. At baseline, the total QOL score was 4.2 ; subscale scores were 2.4 for “symptoms and feelings”, 0.9 for “daily living”, and 0.7 for “work and school”. At the end of the study, the total score was 2.2, showing a significant improvement ; other scales also showed improvement. Taken together, improvements were observed not only in assessments by physicians but also in the patient self-assessments (VAS and DLQI). Thus, if treatment with other antihistamines is ineffective, a change in medication may be helpful, and loratadine seems to be very useful for those cases.

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