ABSTRACT
Chronic granulomatous disease (CGD) is characterized by frequent uncontrollable infections which often lead to death in early childhood. The first clinical signs may be confined to the skin and manifest themselves as abscesses, pyoderma, eczema or draining sinuses. Frequently, lymph nodes, spleen, lungs or liver are also involved. The basic defect is a failure of leukocytes to kill certain bacteria or fungi. The exact biochemical defect is however not yet known. The diagnosis of CGD is based on the clinical picture and on a defect of the granulocytes, as proven by bactericidal, NBT-reduction or chemiluminescence tests. In most and possibly in all of the cases, the disease is X-linked, and the CGD-gene has been regionally assigned to the X-chromosome. The existence of a second type of CGD with autosomal recessive inheritance has been assumed by several authors. In order to improve the prognosis of CGD, it is essential that the disease is diagnosed as early as possible so that prompt treatment can be given.
Subject(s)
Granulomatous Disease, Chronic/physiopathology , Adult , Age Factors , Anti-Bacterial Agents/therapeutic use , Child , Child, Preschool , Diagnosis, Differential , Female , Granulomatous Disease, Chronic/diagnosis , Granulomatous Disease, Chronic/drug therapy , Humans , Infant , Leukocytes/immunology , Male , Nitroblue TetrazoliumABSTRACT
In four medical centers, 40 patients with keratinizing dermatoses were treated with topical tretinoin (vitamin A acid) 0.1% cream and salicylic acid 2% cream in a short-term, double-blind study. Tretinoin was the more effective treatment for several of the keratinizing dermatoses with the exception of palmar-plantar hyperkeratosis, for which it was not effective in the concentration and method of application used. The most striking clinical responses occurred in patients with lamellar ichthyosis and ichthyosis vulgaris. Local adverse reactions-chiefly pruritus, erythema, burning, excoriation, and irritation-were not severe and could be controlled by modification of the treatment regimen.
Subject(s)
Keratosis/drug therapy , Tretinoin/therapeutic use , Vitamin A/analogs & derivatives , Administration, Topical , Adolescent , Adult , Aged , Child , Child, Preschool , Double-Blind Method , Drug Evaluation , Female , Humans , Ichthyosis/drug therapy , Male , Middle Aged , Salicylates/therapeutic use , Tretinoin/adverse effectsABSTRACT
Macrophage migration inhibitor factor (MIF) activity in the sera of patients with mycosis fungoides, Sézary syndrome, and cutaneous lymphoma was observed in the sera of eight of the ten patients with stage II (infiltrative) mycosis fungoides, but in only one of the eight patients with stage I and in neither of the two patients with stage III mycosis fungoides. Two of the three patients with Sézary syndrome had MIF in the serum. No MIF was observed in cutaneous lymphoma. These data support the concept that Sézary syndrome and mycosis fungoides are T-cell diseases, and transitional, prelymphomatous diseases.
Subject(s)
Macrophage Migration-Inhibitory Factors/blood , Macrophages/immunology , Skin Diseases/immunology , Cell Migration Inhibition , Dermatitis, Exfoliative/immunology , Humans , Hypersensitivity, Delayed , Keratoderma, Palmoplantar/immunology , Lymphatic Diseases/immunology , Lymphoma/immunology , Mycosis Fungoides/immunology , Skin Neoplasms/immunology , Skin Tests , Syndrome , T-LymphocytesABSTRACT
Serum MIF activity was studied in ten patients with sarcoidosis, fourteen with granuloma annulare, four with necrobiosis lipoidica, and nine with various dermatological diseases. Positive MIF activity was found in the sera of nine of the ten patients with sarcoidosis and eleven of the fourteen patients with granuloma annulare. The delayed hypersensitivity tests were negative in all nine of the patients with sarcoidosis who had serum MIF activity and were positive in only three patients with cutaneous sarcoid lesions. One of four patients with necrobiosis lipoidica demonstrated minimal serum MIF activity. Data on serum lymphokine activity in sarcoidosis and granuloma annulare suggest that these two diseases are related to delayed hypersensitivity mechanisms.
Subject(s)
Granuloma/immunology , Macrophage Migration-Inhibitory Factors/blood , Sarcoidosis/immunology , Skin Diseases/immunology , Humans , Hypersensitivity, Delayed/diagnosis , Macrophages/immunology , Necrobiosis Lipoidica/immunologyABSTRACT
Heparin causes enhanced nitroblue tetrazolium (NBT) reduction by polymorphonuclear leukocytes (PMN's). To determine the mechanism of this stimulation, samples of 1 to 3 x 10(7) PMN's were incubated with various concentrations of heparin, chondroitin sulfate A (CSA), and chondroitin sulfate B (CSB), with and without NBT. The effect of the polyanions (PA) on PMN hexose monophosphate shunt (HMPS) activity was determined by the production of 14CO2 from glucose-1-14C by the leukocytes. NBT reduction was evaluated histochemically and spectrophotometrically at 515 mmu. Samples of PMN's in heparin and heparin-NBT mixtures were examined by electron microscopy after various incubation periods. Increased NBT reductions by PMN's was found when leukocytes were incubated with heparin, CSA, and CSB, but these compounds had no effect on the HMPS activity of PMN's unless NBT was added. Electron microscopy of samples that contained heparin-NBT revealed an insoluble complex that was phagocytosed by the leukocytes. The stimulation of PMN oxidative metabolism and NBT reduction that follows incubation with PA-NBT appears to be directly related to ingestion of this particulate complex by the leukocytes.
Subject(s)
Chondroitin/pharmacology , Glucose/metabolism , Heparin/pharmacology , Hexosephosphates/metabolism , Leukocytes/ultrastructure , Nitroblue Tetrazolium/metabolism , Pentosephosphates/metabolism , Tetrazolium Salts/metabolism , Carbon Dioxide/analysis , Humans , Leukocytes/drug effects , Leukocytes/metabolism , Microscopy, Electron , Phagocytosis , SpectrophotometryABSTRACT
Immunologic studies were performed in two patients with sarcoidosis, who developed cryptococcosis. Polymorphonuclear leukocyte function, complement, and serum antibodies were normal. Both patients had depressed cell-mediated immunity (cmi) to Cryptococcus neoformans and other antigens that persisted after therapy for their infection. These findings suggest that the patients' impaired CMI predisposed them to cryptococcal infection, which complicated their sarcoidosis. Evaluation of sarcoidosis cases should include studies of immune function, and the possibility of a secondary infection should be considered in patients with long-standing sarcoidosis, who develop unexpected changes in their clinical status.
Subject(s)
Cryptococcosis/immunology , Sarcoidosis/immunology , Amphotericin B/therapeutic use , Candida , Cell Count , Cryptococcosis/etiology , Cryptococcus neoformans/immunology , Dinitrochlorobenzene , Female , Fibula/diagnostic imaging , Flucytosine/therapeutic use , Humans , Immunity, Cellular , Immunoglobulin A , Immunoglobulin G , Immunoglobulin M , In Vitro Techniques , Middle Aged , Radiography , Sarcoidosis/complications , Sarcoidosis/drug therapy , Skull/diagnostic imaging , T-LymphocytesABSTRACT
Microscopical examination of biopsy specimens from cat-scratch skin test sites, performed in patients with cat-scratch disease (CSD), sarcoidosis, tuberculosis, and other granulomatous diseases, showed noncaseating granulomatous dermal inflammation in 11 of 12 patients with CSD and in some patients with sarcoidosis or tuberculosis. Biopsy of cat-scratch skin test sites may be a useful ancillary procedure in diagnosing CSD if other granulomatous diseases are excluded.
Subject(s)
Cat-Scratch Disease/diagnosis , Skin Tests , Adolescent , Adult , Biopsy , Cat-Scratch Disease/immunology , Child , Child, Preschool , Clinical Trials as Topic , Coccidioides/isolation & purification , Coccidioidomycosis/diagnosis , Diagnosis, Differential , False Positive Reactions , Female , Humans , Lymphadenitis/diagnosis , Male , Sarcoidosis/diagnosis , Skin/pathology , Skin Tests/methods , Tuberculosis, Cutaneous/diagnosisABSTRACT
Homogenates of lymph node and skin biopsy specimens and lymphocyte suspensions from patients with sarcoidosis and control subjects were injected into the footpads of CBA/J mice; histologic response was examined at intervals of 15 to 360 days. Kveim tests and complete autopsies were performed on animals of each group. Granulomas or equivocal granulomas were observed in 14.8% of biopsy specimens from animals that received sarcoid tissue homogenates and in 14.8% of control animals. No granulomas were observed after injection of lymphocytes from patients with sarcoidosis or from control subjects. Kveim tests were negative in all animals and autopsies were unremarkable. These studies fail to confirm previous reports of a "transmissible agent" in sarcoidosis and demonstrate that by the use of these techniques, such an agent is not invariably present in sarcoid granulomas.
Subject(s)
Lymph Nodes/microbiology , Lymphocytes/microbiology , Sarcoidosis/transmission , Skin/microbiology , Animals , Autopsy , Biopsy , Female , Hindlimb , Inflammation , Kveim Test , Mice , Mice, Inbred CBA , Sarcoidosis/microbiology , Sarcoidosis/pathologyABSTRACT
Bundle-shaped tubular (BST) inclusions were identified by electron microscopy in 1 to 6 percent of peripheral blood mononuclear cells obtained from 10 to 11 patients with sarcoidosis. The frequency of the inclusions within mononuclear cells did not correlate with the clinical status of the patients. The relationship, if any, of BST inclusions to sarcoidosis and the immunologic defects in sarcoid patients remains to be determined.