Dermatomyositis is an idiopathic inflammatory myopathy with cutaneous manifestations including a heliotrope rash, Gottron’s papules, cuticular changes and poikiloderma. Some cutaneous features are thought to be pathognomonic, and others are thought to be only characteristic. There is an association of dermatomyositis with cancer, and the prognosis of such patients is very poor. Management of patients complicated by progressive interstitial pneumonia is very difficult. Various characteristic cutaneous features of the disease should be recognized to detect the disease in its initial stages and provide early treatment.
TIA-1 and granzyme B are cytotoxic granule proteins in T cells and natural killer cells. They have been found to be involved in the induction of apoptosis and are manifested in some type of cutaneous T cell lymphoma. We examined immunohistopathological studies of component subsets of the lymphocytic infiltrates in inflammatory skin disorders, mainly histopathologically characterized lichenoid tissue reaction. A total of 73 skin biopsies were obtained from 13 cases of lichen planus, 5 cases of lichenoid drug eruptions, 14 cases of discoid lupus erythematodes, 4 cases of lupus erythematodes profundus, 12 cases of systemic lupus erythematosus, 11 cases of toxicoderma, 5 cases of psoriasis vulgaris, 7 cases of chronic eczema, and 2 cases of lymphadenitis benigna cutis. The formalin-fixed paraffin-embedded tissues were stained using the labeled streptavidinbiotinylated antibodies method with antisera to UCHL-1, CD 4, CD 8, CD 79 a, CD 30, CD 56, CD 68, S-100, HLA-DR, TIA-1, and Granzyme B. Results : Most cells in the dermal infiltrates stained with UCHL-1 (T cells) antibodies and with CD 8 (suppressor cytotoxic T cell). These cells particularly expressed TIA-1 in lichen planus and lichenoid drug eruption. A double-staining method using antibodies for CD 8 and TIA-1 was performed ; concomitant TIA-1-positive and CD 8-positive cells were found in lesions with severe basal damage.
Chronic hepatitis C virus (HCV) infection has recently been reported in association with various skin disorders. To determine the incidences of those disorders in HCV positive patients, we examined 220 patients for skin symptoms. One hundred and fifty-one of them (69%) had at least one skin disorder. HCV positive patients with skin diseases had a statistically higher mean age, a longer duration of HCV infection, and a higher serum ZTT level compared to those without them. Twenty patients with immunity-related skin diseases, including urticaria, lichen planus and cryogroblinemic purpura, were examined, and it was found that statistically more time had elapsed since blood transfusion for the patients with those diseases than those without. From these results, we speculate that the long duration of HCV infection encourages antibody production against HCV and/or immune complexes, which may cause the development of these disorders. The reported incidences of palmar erythema, vascular spider, and paper money skin were higher in patients with chronic alcoholic hepatopathy than in our 220 HCV positive patients. To determine the localization of HCV in those diseases, we immunohistochemically exaamined skin leasions of lichen planus and cryogroblinemic purpura using monoclonal and polyclonal antibodies against HCV. However, we could not obtain any definitive results.
We report a 62-year-old woman with paraneoplastic pemphigus who developed severe mucosal ulceration and widespread keratotic papules. Skin biopsies revealed epidermal vacuolization, keratinocyte necrosis, and a band-like mononuclear cell infiltration, showing a lichenoid reaction. IgG and C 3 deposition was observed in intercellular spaces of the epidermis and along the basement membrane zone on direct immunofluorescence. An indirect immunofluorescence study showed intracellular IgG staining in the normal human epidermis, and IgG deposition in the basement membrane zone and intercellular spaces of rat bladder epithelium. By immunoblotting, polypeptides with molecular weights of 250-, 190- and 130-kD, were recognized in the patient’s serum. Antibodies against desmoglein 1 and 3 were also found in the serum by ELISA. B cell lymphoma was diagnosed by a lymph node biopsy. Acute respiratory failure developed and caused the patient’s death. In our patient, lichenoid tissue reactions were prominent and no bulla formation was seen. This allows us to hypothesize that both cell-mediated and humoral immune reactions are important in the pathogenesis of paraneoplastic pemphigus. We consider that the diagnosis of the disease should be made by multiple immunological examinations including indirect immunofluorescence using rat bladder, immunoblotting and ELISA.
We report here a case of solitary malignant peripheral nerve sheath tumor (MPNST) not associated with neurofibromatosis. A 67-year-old-man presented with an elastic-hard, subcutaneous tumor on his nuchal area. Microscopic examination of the center of the tumor revealed a storiform pattern composed of spindle and oval shaped tumor cells. The tumor cells had large atypical or bizarre nuclei. These cells were positive for S-100 protein on immunohistochemistry. The patient was treated by a surgically wide excision. There was no evidence of recurrence or metastasis during a follow-up period of one year and ten months. We reviewed 133 cases of MPNST from 1990 to 1999 in Japan. Compared with the patients with neurofibromatosis, the patients without neurofibromatosis were older, and the interval between diagnosis and metastasis or recurrence were longer.
We directly examined Candida and isolated it from the tongues of bedridden patients. When a few hyphal fungal elements were detected by KOH method, the direct examination was determined to be positive. Twenty-five out of 56 (44.6%) patients were positive by the KOH method. Many of the positive patients had red or atrophic tongues with white plaques. Positive rates of patients with tuberculosis and shorter periods of being bedridden were much higher than others. However, there was little difference in positive rates when compared by patients’ laboratory findings, oral medicine, way of feeding, score of activities of daily living, braden scales, presence of decubitus, or survival rate after one month. Candida spp. were isolated from 43 of the 56 (76.8%) patients by cotton swab. The most commonly isolated species was Candida albicans.
A 56-year-old male presented with a subcutaneous tumor (6×6.5×3.5 cm) on the lumber area of about 8 years duarations. The tumor was elastic hard and showed no adherence to the underlying muscles in the CT scan examination. The whole tumor was excised with a 3 cm surgical margin. On histologic evaluation, the tumor was consisted of areas of ordinary dermatofibrosarcoma protuberans (DFSP) composed of uniform spindle-shaped tumor cells with storiform or cart-wheel growth patterns. In other histologic areas, a dense proliferation of atypical spindle cells arranged in fascicular and herring-bone patterns was also observed. Immunohistochemical staining for CD34 was negative in fibrosarcomatous areas (DFSP-FS) but positive in DFSP areas.
A 40-year-old Japanese man presented with purpura and subcutaneous nodules, which caused severe skin ulcers with multiple mononeuropathy. He also had marked neutrophilia and eosinophilia. His WBC was 27,300/l. (Neut. 40.5%, Lym. 3.5%, Eos. 54.0%) Antineutrophil cytoplasmic autoantibodies were negative. He had no past history of asthma. A biopsy of the left foot revealed necrotizing vasculitis with severe infiltration of eosinophils in the vessels of mid dermis to subcutaneous fat, and a diagnosis of polyarteritis nodosa (PAN) was made. He was treated with steroid pulse therapy (methylpredonisolone 1,000 mg/day for 3 days) followed by oral prednisolone 60 mg/day and cyclophosphamide 100 mg/day, with good results. We had some difficulties in making the definite diagnosis. We finally diagnosed this case as PAN, but clinical and serological findings suggested allergic granulomatous angiitis or microscopic PAN. We discuss whether this case would fit the criteria for polyangiitis overlap syndrome.