Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “PEMPHIGUS ERYTHEMATOSUS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

560 records · Page 32Linked to original sources
PubMed2002

Immune-mediated complications during interferon therapy in hematological patients.

Interferon (IFN), a leukocyte-derived cytokine, has been used to treat several hematological malignancies. The most common adverse effects of IFN are flu-like symptoms. Autoimmune side effects are infrequent but may be hazardous and irreversible. These may occur in several ways: autoantibodies may either appear during the treatment or existing titers may rise, subclinical autoimmune phenomena may become clinically manifest or autoimmune diseases may appear de novo. The main categories of IFN immune-mediated side effects are: thyroid, hematological, connective tissue, renal and miscellaneous disorders. The most common ones are thyroid disorders, which manifest either as hypo- or hyperthyroidism. Patients with pre-existing autoantibodies are more susceptible to the exacerbation of thyroid autoimmunity, probably since IFN enhances the levels of autoimmunity. Hematological disorders include autoimmune anemia and thrombocytopenia and thrombotic thrombocytopenic purpura. The immunological derangement of autoimmune hemolytic anemia manifests as enhanced destruction of antibody-coated red blood cells and induction of autoreactive B cells secreting these antibodies. Although autoimmune thrombocytopenia is rare, a sharp reduction in the platelet counts, beyond that expected from the antiproliferative effects of IFN, should raise this possibility. Thrombotic thrombocytopenic purpura has recently been included among the autoimmune disorders. Sporadic cases have been reported in association with IFN treatment. The clinical spectrum of IFN-induced connective tissue disorders ranges from typical systemic lupus erythematosus to seropositive or seronegative rheumatoid arthritis. Some authors also reported on the development of Behçet's disease in chronic myeloid leukemia patients treated with IFN. The underlying reason for the skin hyperreactivity in Behçet's disease and the effect of IFN treatment in these patients may be altered neutrophil activity in both disorders. Several series evaluated the incidence of Raynaud's phenomenon in patients treated with IFN for hematological disorders. Some of them reported on a rather high incidence of nailfold capillary microscopy abnormalities with or without Raynaud's phenomenon. Whether IFN-induced Raynaud's phenomenon is immune-mediated or directly caused vasospasm, is still unknown although the occurrence of several autoantibodies suggests an immune mechanism. Adverse effects of IFN therapy on the kidney include proteinuria and rarely nephrotic syndrome or acute and chronic renal failure. The mechanism of renal injury is unclear although an immune mechanism is suggested. Sporadic cases of other immune-mediated side effects have been published. These include dermatological adverse effects manifesting as psoriasis, pemphigus and vitiligo, and also rare cases of sarcoidosis, hepatitis, colitis or cryoglobulinemia. In conclusion, patients treated with IFN should be monitored for symptoms of autoimmunity. Patients with previous autoimmune phenomena should be treated, if possible, with alternative drugs since there is risk of exacerbation of these manifestations in these patients.

Autoimmune Diseases↗
PubMedDate not supplied

Chronic granulomatous disease: two members of a single family with different dermatologic manifestations.

Case 1: A 33-year-old man with a 14-year history of localized skin disease on the face and scalp was evaluated at the department of dermatology. The physical examination revealed plaques with papules, pustules, and a golden yellow crusting on the forehead, cheeks, upper lip, and chin (Figure 1). The scalp presented fine, whitish scales. At the beginning of his disease, the patient presented large red and painful purulent boils. The 14-year clinical course of these lesions was characterized by partial remissions and recurrences, but he did not specify any treatment related to improvement. The clinical diagnosis given for the scalp lesions was seborrheic dermatitis. For the facial lesions, many differential diagnoses were considered, among them: seborrheic dermatitis, acneiform dermatitis, impetigo, folliculitis, seborrheic pemphigus, and demodicidosis. The histopathologic study of a biopsy taken from the cheek (Figure 2) showed superficial spongiform dermatitis with neutrophils and folliculitis that are compatible with the diagnosis of seborrheic dermatitis. Both Gram and periodic acid-Schiff stains were negative. Follow-up of the patient was not possible since he did not come back. The disease in this patient initially manifested at age five by the presence of recurrent ganglionic abscesses. At age 15, he presented a pulmonary abscess of a left lobule that was surgically removed; at this point the diagnosis of chronic granulomatous disease was established. At age 28, an exploratory laparotomy was performed due to peritonitis and multiple hepatic abscesses. At that time, he was treated with antibiotics (mainly trimethoprim-sulfamethoxazole) and interferon-g. The patient had two brothers who died due to complications of chronic granulomatous disease. In addition, both his mother and sister presented a history of discoid lupus-like lesions. Case 2: Coincidentally, his 27-year-old sister was seen in our department of dermatology 5 years before, presenting infiltrated and erythematous plaques with fine scales (Figure 3) on the right side of the nose and the left annular finger. No other cutaneous or mucous lesions were seen. She referred onset in childhood with similar lesions on sun-exposed areas that disappeared without scarring. A biopsy was performed and the results were compatible with the diagnosis of discoid lupus erythematosus (Figure 4). Direct immunofluorescence was not available. At that time, she did not mention the family history of chronic granulomatous disease. Clinical follow-up was not possible, but his brother referred that she afforded complete remission only with sun protection.

Adult↗