ABSTRACT
We report two patients with severe amicrobial pustular dermatosis with immunological abnormalities: a 63-year-old woman with a 30-year-history of discoid lupus erythematosus and sicca syndrome, and a 35-year-old woman with high levels of gamma-globulinemia and positive antinuclear antibodies. Both patients presented with crusty and eroded erythematous plaques studded with aseptic pustules on the back, face, and scalp. Histological examination showed acanthosis, neutrophilic exocytosis to the epidermis, and neutrophilic and lymphocytic infiltration with nuclear dust in the dermis. These patients were diagnosed as having "amicrobial pustulosis associated with autoimmune diseases". The eruptions improved with combination treatment of oral prednisolone with cyclosporin A or diaminodiphenylsulphone. Although the pathogenesis remains unclear, amicrobial pustular dermatosis might be one of the cutaneous complications in autoimmune diseases.
Subject(s)
Autoimmune Diseases/pathology , Skin Diseases, Vesiculobullous/pathology , Administration, Oral , Adult , Aged , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Autoimmune Diseases/complications , Autoimmune Diseases/drug therapy , Cyclosporine/therapeutic use , Dapsone/therapeutic use , Dermatologic Agents/therapeutic use , Drug Therapy, Combination , Female , Humans , Prednisolone/therapeutic use , Skin Diseases, Vesiculobullous/complications , Skin Diseases, Vesiculobullous/drug therapyABSTRACT
Nasal and nasal-type natural killer (NK)/T-cell lymphomas follow an aggressive course and have a poor prognosis. Recent pathologic studies suggest that the disease is a malignant proliferation of NK cells, which often express CD56. An association with the Epstein-Barr virus has also been reported. Skin involvement occurred in each of the 3 patients studied. Radiation therapy provided some benefit to the patients in the early stages. Conventional chemotherapies were not effective. To overcome this multiple-drug resistance of the tumor cells, cyclosporine and high-dose chemotherapy was combined with peripheral-blood stem-cell transplantation. The average life span from the onset of the disease for our patients was 9.6 months. Further improvement in the management of nasal and nasal-type NK/T-cell lymphomas is necessary.
Subject(s)
Killer Cells, Natural/pathology , Lymphoma, T-Cell, Cutaneous/pathology , Nose Neoplasms/pathology , Skin Neoplasms/pathology , Adult , Aged , Fatal Outcome , Female , Humans , Immunohistochemistry , Killer Cells, Natural/immunology , Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/therapy , Male , Nose Neoplasms/immunology , Nose Neoplasms/therapy , Skin Neoplasms/immunology , Skin Neoplasms/therapyABSTRACT
A 68-year-old woman had a peculiar clinical course of cutaneous lymphoma. She first developed nonpuritic erythematous macules with fine scales followed by development of erythematous infiltrated plaques. The clinical course could be interpreted as that of mycosis fungoides. Histologically, the lesions showed pagetoid infiltration of atypical lymphoid cells. Suddenly, with high fever, numerous purpuric, ulcerated, or crusted plaques with underlying infiltration or nodules began to appear on most areas of the patient's body. Biopsy specimens of the lesions revealed angiocentric and angiodestructive infiltration by neoplastic T cells and marked epidermotropism of these cells. These atypical cells immunohistochemically had CD8+ surface phenotype. The patient died of respiratory insufficiency due to acute pulmonary infiltration. Autopsy demonstrated angiocentric and angiodestructive lymphomatous infiltration in the lung.
Subject(s)
Lymphoma/pathology , Skin Neoplasms/pathology , Aged , Blood Vessels/pathology , Epidermis/pathology , Erythrocytes/pathology , Female , Humans , Necrosis , Paget Disease, Extramammary/blood supply , Paget Disease, Extramammary/pathology , Skin Neoplasms/blood supply , T-Lymphocytes , T-Lymphocytes, Cytotoxic , T-Lymphocytes, RegulatorySubject(s)
Infant Mortality , Autopsy , Birth Injuries/mortality , Congenital Abnormalities/mortality , Female , Fetal Death/etiology , Fetal Hypoxia/mortality , Fetal Organ Maturity , Humans , Hyaline Membrane Disease/mortality , Infant, Newborn , Infections/mortality , Japan , Lung/embryology , Male , PregnancyABSTRACT
Twelve infantile autopsy cases up to 205 days using a long-term respirator care were examined in this study. These cases could be divided into three subgroups. The first group included the cases treated with long-term respirator care for hyaline membrane disease. In this subgroup, bronchopulmonary dysplasia was found during the course. The second group included the cases treated with long-term respirator care for other causes and were very premature infants. In this group, some cases showed bronchopulmonary dysplasia-like lesions. The third group was similar to the second group except for not being very premature. All groups had similar histological changes including alveolar cell desquamation, regeneration of alveolar lining cells, fibrosis, and smooth muscle-like tissue formation of alveolar walls. However, the premature lung tissues remained characteristically in some cases of the second group. In this group, the prematurity of lung was thought responsible for the development of bronchopulmonary dysplasia-like lesion. In addition to bronchopulmonary dysplasia or bronchopulmonary dysplasia-like lesion, other changes including pneumonia, persistent ductus arteriosus, cytomegalic inclusion disease and so forth were discussed.
