Yayoi Nagai

Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis

Background  Although digital ulcerations frequently occur in patients with systemic sclerosis (SSc), there have been few reports on the macrovascular involvement.

Lichenoid Drug Eruption with Palmoplantar Hyperkeratosis due to Imatinib Mesylate: A Case Report and a Review of the Literature

Cutaneous adverse effects of imatinib mesylate (Glivec) are common and various types of skin eruptions have been reported. We report here a 57-year-old man who presented with lichen planus-like lesions on his extremities and palmoplantar hyperkeratosis due to the use of imatinib mesylate for chronic myeloid leukaemia. The skin lesions improved after discontinuation of imatinib mesylate but re-administration of the drug at a lower dose provoked a mild recurrence. He could, however, continue to take the drug at the lower dose and his skin lesions were well-controlled by topical corticosteroid treatment. The literature on lichenoid drug eruption due to imatinib mesylate is reviewed.

Detection of human papillomavirus type 56 in Bowen’s disease involving the nail matrix

Background  As Bowen’s disease of the nail apparatus is quite rare, there have been only a few reports on the prevalence of human papillomavirus (HPV) infection in this condition.

Primary cutaneous nocardiosis due to Nocardia nova in a healthy woman.

We describe a 68‐year‐old healthy woman with an unusual skin manifestation of mycetoma. A dark reddish erythematous plaque was present on the dorsum of the left hand for over 10 years. A skin biopsy revealed sulphur granules in the abscess, and Nocardia nova was identified from the specimen. Treatment with dapsone resulted in significant improvement. We review the clinical features of cutaneous N. nova infections.

Pneumomediastinum and Subcutaneous Emphysema Associated with Fatal Interstitial Pneumonia in Dermatomyositis

We described a 65‐year‐old woman who died of acute interstitial pneumonia associated with dermatomyositis. Subcutaneous emphysema and pneumomediastinum simultaneously developed. The association of the pulmonary rupture with vasculitis has been assumed as the common cause in interstitial pneumonia. Diffuse alveolar damage, however, might have led to the pneumomediastinum and subcutaneous emphysema in our patient, who had no signs of cutaneous vasculitis.

Juvenile systemic sclerosis: Report of three cases and review of Japanese published work

Juvenile or childhood onset systemic sclerosis (SSc) is rare. In our department, we encountered three juvenile SSc patients among the overall 206 SSc patients. All three cases were diffuse cutaneous type SSc (dSSc) with positive antinuclear antibodies. In this report, we report the three patients and analyze clinical and serological features of 58 Japanese patients with juvenile SSc. Forty‐eight patients (92.3%) were classified as dSSc and 21 out of 35 patients (60%) had anti‐topoisomelase I antibodies, while anticentromere antibodies were not detected in any patients. Prevalence of lung fibrosis and scleroderma renal crisis was low, however, the rate of cardiac involvement was higher than that in adult patients.

Isolated cutaneous manifestation of IgG4-related disease

Aims The cutaneous manifestation of IgG4-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG4-positive plasma cell infiltration in the skin. Methods The authors investigated two cases of IgG4-related disease with solitary skin lesions and compared the immunohistochemical characteristics of infiltrating cells among IgG4-related disease, Kimuras disease and cutaneous pseudolymphoma. Results IgG4-related disease manifested as an indurated plaque on the anterior chest in one case and a nodule on the toe in the other case. Histopathologically, skin lesions of IgG4-related disease showed a dense, mixed-cell infiltrate containing lymphocytes, plasma cells and eosinophils along with fibrosis. Plasma cells stained positively with anti-IgG and anti-IgG4 antibodies, and the ratio of IgG4+/IgG+ cells was more than 50%. Serum levels of IgG and IgG4 were not elevated and no lesions were found in other organs. Skin samples taken from Kimuras disease showed histopathological features similar to those of IgG4-related disease. The proportion of IgG4+/IgG+ was high in Kimuras disease, but not in cutaneous pseudolymphoma. Conclusions The solitary skin lesions of IgG4-related disease were similar histologically and immunohistochemically to the skin lesions of Kimuras disease. The concept of IgG4-related disease may help clarify the pathomechanism of diseases of unknown aetiology that possess features of IgG4-related disease.

Nephrogenic Systemic Fibrosis with Multiple Calcification and Osseous Metaplasia

We describe here a 50-year-old Japanese man with nephrogenic systemic fibrosis. He had been suffering from chronic renal insufficiency and had been treated with haemodialysis. He had undergone magnetic resonance angiography using gadodiamide 7 years previously. One month after magnetic resonance angiography, he noted swelling, hotness, induration and pain in his left arm. The same symptoms gradually spread over his lower extremities, resulting in flexion contractures with limited range of motion. Physical examination revealed skin sclerosis on his extremities with a glossy brownish skin surface. Histologically, increased collagen fibres with high cellularity were seen in the dermis and subcutaneous septa. Thickened fascia was also noted, as well as osseous metaplasia under the fascia. Computed tomography of the whole body revealed multiple calcification of the fascia in many muscles. Treatment with intravenous sodium thiosulphate did not result in any clinical improvement.

Adult-onset Still's disease with prurigo pigmentosa-like skin eruption

A 34‐year‐old woman with adult‐onset Stills disease (AOSD) developed prurigo pigmentosa‐like lesions on her chest and upper back in addition to the typical rash of AOSD. A biopsy specimen taken from the upper back showed characteristic features of prurigo pigmentosa. The eruption and fever subsided immediately after the administration of 40 mg/day prednisolone, but arthralgia persisted even after intravenous pulse methylprednisolone therapy in combination with immunosuppressive drugs. Various atypical skin rashes, including prurigo pigmentosa‐like lesions, have been reported in association with AOSD. Therefore, one should carefully follow the clinical course of a patient in order not to overlook these atypical cutaneous manifestations of AOSD.

Expression of Autocrine Motility Factor Receptor in Cutaneous Malignant Melanoma

BACKGROUND The autocrine motility factor receptor (AMFR), a specific receptor for AME, is considered to be related to the metastatic potential of tumor cells. OBJECTIVE We investigated whether AMFR is expressed on cutaneous malignant melanoma cells and has any relationship to clinical stage and metastatic ability. METHODS We performed an immunohistochemical study on 20 cases of cutaneous malignant melanoma using anti-AMFR antibody. AMFR was expressed in 9 cases (45%), and its expression had a tendency to correlate with the clinical stage and the presence of lymph node metastasis. CONCLUSIONS The expression of AMFR may have a possible association with the metastatic potential of malignant melanoma.