Bronchopulmonary manifestations of Horton's disease. Apropos of a case

A 78 years old woman developed a pulmonary recurrence four years after the diagnosis of temporal arteritis. The lung biopsy showed the same giant-cells granulomatous lesions with central vessels as the initial biopsy. The recurrence in giant cells-arteritis has already been published but the pulmonary involvement had never been illustrated before by histological data. The difficulties of diagnosis in case of granulomatous angiitis on lung samples is emphasized.     

Cardiac manifestations of Lyme disease. Apropos of a case

A new case of heart involvement in Lyme's disease is reported. Its originality lies in its strictly isolated nature, therefore revealing the disease; in its typical picture of myocardopericarditis combining, in various stages, infra-hissian conduction disorders, bouts of left ventricular insufficiency, pseudoischemic repolarisation disorders and finally a moderate pericardial shift; in the demonstration, during two successive heart explorations by NMR (Nuclear Magnetic Resonance), of a diffuse myocardial hypertrophy, subsiding in time, as the other symptoms of this patient.     

Cardiovascular manifestations of Cogan syndrome. Apropos of a case]

The authors describe a case of Cogan's syndrome in a patient with ulcerative colitis complicated by several cardiovascular manifestations including bilateral coronary ostial stenosis, rapidly progressive aortic regurgitation and aneurysm of the thoracic aorta, thrombosis of the common iliac artery and pericardial symphysis. This rare form of inflammatory arteritis, the diagnosis of which is usually made on the finding of associated ocular and auditory involvement, is distinct from other types of angiitis by the predisposition to severe cardiovascular complications which influence the vital prognosis. The differential diagnosis with more common collagen diseases with cardiovascular complications is discussed.     

Methoxsalen photochemotherapy for mycosis fungoides.

Methoxsalen photochemotherapy (PUVA; psoralen plus ultraviolet light) is effective in the treatment of mycosis fungoides (MF). The mechanism of this beneficial effect is unknown but probably involves covalent photo-binding of methoxsalen molecules to pyrimidine bases in DNA at the cellular level and impaired T-cell function or survival at the tissue level. Eleven patients (seven with plaques and four with erythroderma) were referred for PUVA therapy because of poor response to conventional therapy. Seven patients had complete clearing of skin lesions and three improved markedly. All ten of these patients experienced good to excellent control of the disease while receiving maintenance therapy, resulting in a virtual remission lasting for greater than 2 years in four patients. Of the six patients who discontinued PUVA during the followup period, three died within 1 year and three experienced progression of MF despite conventional therapy. The very promising results in this small patient group should encourage further studies of PUVA for cutaneous T-cell lymphomas.     

Paget's骨病伴皮肤蕈样霉菌病一例报告

Paget's骨病(Paget's disease of bone,PDB),又称畸形性骨炎,系原因不明骨吸收增加的缓慢、局限性骨骼病变.可致骨再建异常,新形成不正常骨质增加,局部骨骼膨大、疏松、血管增多,易畸形和骨折.     

Sclerodactyly in a patient with mycosis fungoides

A 44-year-old man had mycosis fungoides and generalized plaque disease involving 80% of his skin surface with diffuse lymphadenopathy and alopecia of the scalp and groin. In addition, distal to the wrist, there were sclerodermatous changes involving the skin of the hands with associated sclerodactyly of all digits with loss of normal palmar creases. There were no subungual telangiectasis or digital ulcers. The changes in the hand that occurred in this case, no doubt arose as a result of the patient's neoplasm. Abnormalities of collagen biosynthesis and degradation probably occur with mycosis fungoides as a result of the extensive infiltration of the epidermis and dermis with malignant cells. To our knowledge, the association of sclerodactyly with mycosis fungoides has not been previously reported.     

Combined therapy for patients with mycosis fungoides.

In a comprehensive mycosis fungoides program, 60 patients have been seen with a pathologic diagnosis of this disease. Forty-four patients with advanced disease were referred for radiation therapy. Three treatment techniques were identified in which 14 patients were treated with localized fields using electrons or whole-body electron-beam therapy with doses of less than 3000 rads, 21 patients were treated using the Stanford technique with tissue doses of between 3000 and 4000 rads, and nine patients were treated with six cycles of mechlorethamine, vincristine, prednisone, and procarbazine or cyclophosphamide, vincristine, prednisone, and procarbazine following the electron-beam therapy. The actuarial survival rate was 45% at 1 year for the 14 patients with localized electron-beam therapy, whereas the actuarial survival rates were 83% for patients treated with whole-body electron-beam therapy and 100% for patients treated with whole-body electron-beam therapy followed by four-drug chemotherapy. The recurrence-free interval for these three groups correlates with these observations. A central nervous system recurrence has been observed in the combined-therapy group.     

Meningeal mycosis fungoides: clinical and cellular characteristics.

A patient with mycosis fungoides developed meningeal disease while his skin disease was in remission with systemic chemotherapy. His central nervous system involvement with mycosis fungoides was controlled with intrathecal methotrexate for 7 months. The proliferating cells recovered from the spinal fluid showed similarities to the Sézary cell by light and electron microscopy. Surface receptor sutudies suggested that these cells were lymphoid cells of thymic derivation. Although mycosis fungoides has been shown to spread to the central nervous system in autopsied cases, reports of clinical neurologic disease are rare, and in only one earlier report have malignant cells have been found in the spinal fluid. Thus, as in other lymphoproliferative disorders, prompt consideration of meningeal involvement in a patient exhibiting neurologic symptoms while in peripheral remission may allow earlier treatment of this complication.     

Heart valvular and coronary manifestations of Takayasu disease. Apropos of a surgically-treated case]

Cardiac involvement in Takayasu's disease is well documented. This is often the result of severe hypertension. However, severe clinical manifestations of aortic regurgitation and coronary insufficiency are much less common. The authors report a case in which post-infarction angina and severe left ventricular failure led to a double valve replacement and an aorto-right coronary bypass graft procedure. The diagnosis of Takayasu's disease was suspected before surgery and was confirmed by histological examination.     

Photochemotherapy for mycosis fungoides: long-term followup study.

A 3-year followup on ten of 12 patients with mycosis fungoides (MF) treated with photochemotherapy (PUVA; psoralens + ultraviolet light) reveals eight patients whose diseases have remained clear while receiving maintenance PUVA. An additional 35 patients with MF have been successfully treated with PUVA. Eight cases of parapsoriasis en plaque have cleared with PUVA. PUVA is indicated in early eczematous and plaque stages of MF. It may be used as adjunctive therapy in tumor-stage MF treated with X-ray therapy or chemotherapy.     

Combination chemotherapy for mycosis fungoides: a Southwest Oncology Group study.

Between 1972 and 1977, the Southwest Oncology Group studied the following three chemotherapy programs for the treatment of patients with advanced forms of mycosis fungoides: (a) cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) (seven patients); (b) adriamycin, vincristine, and prednisone (HOP) (five patients); and (c) cyclophosphamide, vincristine, prednisone, and bleomycin (COP plus bleomycin) (12 patients). Among the 24 evaluable patients there was an overall objective response rate of 95% with seven (29%) achieving a complete remission. With the adriamycin-containing chemotherapy, five (42%) of 12 patients achieved a complete remission compared to two (17%) of 12 patients treated with COP plus bleomycin. The median duration of remission (partial plus complete) was longer with the COP plus bleomycin combination (median, 47 weeks) than with the adriamycin-containing combinations (median, 22 weeks; P = 0.03). The median survival for all 24 evaluable patients was 95 weeks and was similar regardless of remission-induction therapy. In summary, combination chemotherapy proved to be effective palliative therapy for advanced mycosis fungoides.     

Atypical mycosis fungoides with cerebral involvement

Summary This report concerns a 17-year-old male patient with atypical mycosis fungoides (m.f.). Initial examination revealed generalized lymphoma and uncharacteristic livid skin efflorescence. The patient developed bone marrow involvement and meningeal leukaemia 6 months later. Diagnosis was confirmed by immunohistochemistry and electron microscopy. Aggressive chemotherapy yielded no response.