Drug-induced pemphigus (DIP) is a special type of pemphigus, and its pathogenesis, characteristics of treatment, and prognosis are closely related to the inducing drugs. This article reports the diagnosis and treatment of DIP (pemphigus vulgaris) caused by the administration of rifampin to a patient with tuberculosis. Combined with the literature, we discussed the types, pathogenesis, differential diagnosis, and treatment principles of DIP. We propose that in the oral clinical practice for patients with pemphigus vulgaris, the importance of investigating suspected drugs that induce DIP should be emphasized.
Diagnosis, Differential ; Humans ; Pemphigus/drug therapy* ; Pharmaceutical Preparations ; Prognosis

Female ; Humans ; Middle Aged ; Pemphigus ; diagnosis ; drug therapy ; Pemphigus, Benign Familial ; diagnosis ; drug therapy ; Tacrolimus ; therapeutic use

Objective To investigate the clinical manifestations,diagnosis,treatment,and laboratory examination characteristics of 8 pemphigus patients with high titers of anti-desmoglein antibodies in remission. Methods A retrospective study was conducted for the pemphigus patients diagnosed and treated in the department of dermatology from January 2013 to September 2020.The patients should have the serum anti-desmoglein antibodies ≥150 U/ml in remission or the antibody levels dropped less than 20%(calculated based on the maximum detection limit of 150 U/ml)of their initial ones detected before treatment,and the clinical and laboratory data of patients eligible for the inclusion criteria were collected. Results Among the 134 pemphigus patients with available follow-up data during this period,a total of 8 patients met the criteria,with the follow-up period of 21-85 months and the remission duration of 18-70 months.They all received less than or equal to 10 mg/d prednisone and had high titers of anti-desmoglein antibodies.At their first visit,the number of patients with positive anti-desmoglein 1/desmoglein 3 antibodies was 7.Two patients still had high titers of anti-desmoglein 1 antibodies 19 months and 21 months after they achieved remission,and 5 patients had high titers of anti-desmoglein 3 antibodies in 18-70 months.There was one patient showing high titers of both antibodies,especially for anti-desmoglein 1 antibodies.This patient relapsed after 19 months' remission while other patients were still in clinical remission. Conclusions Some pemphigus patients showed persistent high titers of anti-desmoglein antibodies in remission.Anti-desmoglein 3 antibodies were more common to keep positive,while high titer of anti-desmoglein 1 antibodies was less observed.The high titer of anti-desmoglein 1 antibodies had a correlation with recurrence.For the pemphigus patients with long-term clinical remission but high antibody titer,the dosages of corticosteroids should be adjusted carefully according to their actual clinical manifestations and the positive antibody type.For the patients with high titer of anti-desmoglein 1 antibodies,the dosage reduction of corticosteroids should be appropriately slower.
Autoantibodies ; Drug Therapy, Combination ; Enzyme-Linked Immunosorbent Assay ; Humans ; Pemphigus/drug therapy* ; Recurrence ; Retrospective Studies

Adult ; Dapsone ; adverse effects ; Humans ; Infectious Mononucleosis ; chemically induced ; Male ; Pemphigus ; drug therapy ; Syndrome

Pemphigus is a severe chronic autoimmune mucocutaneous bullous disease. Glucocorticoids are considered as the first line of treatment for this disease. Dysfunctional uterine bleeding is also observed as a result of hypothalamic-pituitary-ovary axis dysfunction. This study reported one female patient with pemphigus vulgaris complicated with dysfunctional uterine bleeding upon systemic glucocorticoid usage. Before this disease was diagnosed, the patient experienced normal menstruation. The mechanism of dysfunctional uterine bleeding triggered by glucocorticoids is elucidated on the basis of case studies and literature review.
Female ; Glucocorticoids ; adverse effects ; Humans ; Metrorrhagia ; chemically induced ; Pemphigus ; drug therapy

Candidiasis ; drug therapy ; immunology ; metabolism ; Diabetes Mellitus, Type 1 ; complications ; drug therapy ; immunology ; therapy ; Humans ; Lupus Erythematosus, Systemic ; drug therapy ; immunology ; metabolism ; Mycoses ; complications ; immunology ; therapy ; Paraneoplastic Syndromes ; drug therapy ; immunology ; metabolism ; Pemphigus ; drug therapy ; immunology ; metabolism

Aged ; Anti-Bacterial Agents ; therapeutic use ; Dermatitis Herpetiformis ; drug therapy ; Female ; Humans ; Minocycline ; therapeutic use ; Niacinamide ; therapeutic use ; Pemphigus ; drug therapy

OBJECTIVE: Critically ill patients with solid tumors complicated with paraneoplastic pemphigus are usually treated in intensive care units (ICU) for perioperative management after surgical treatment. In this study, the clinical characteristics and predictors of long-term prognosis of these critically ill patients were analyzed. METHODS: the clinical and laboratory data of 63 patients with solid tumors complicated with paraneoplastic pemphigus admitted to ICU from 2005 to 2020 were retrospectively analyzed, and the survival status of the patients were followed up. RESULTS: Among the 63 patients, 79.4% had Castleman disease as the primary tumor, and 20.6% with other pathological types; 69.8% had severe-extensive skin lesions, and 30.2% had other skin lesions; the patients with bronchiolitis obliterans accounted for 44.4%, and 55.6% were not merged. Postoperative fungal infection occurred in 23.8% of the patients, and 76.2% without fungal infection. The median follow-up time was 95 months, and 25 patients died during the study period. The 1-year, 3-year and 5-year survival rates were 74.6% (95%CI 63.8%-85.4%), 67.4% (95%CI 55.6%-79.2%) and 55.1% (95%CI 47.9%-62.3%), respectively. The log-rank univariate analysis showed that the patients had age>40 years (P=0.042), preoperative weight loss>5 kg (P=0.002), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.002), and perioperative fungal infection (P < 0.001) had increased mortality. Cox univariate analysis showed that preoperative weight loss >5 kg (P=0.005), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.009), preoperative bacterial pulmonary infection (P=0.007), prolonged surgical time (P=0.048), postoperative oxygenation index (P=0.012) and low albumin (P=0.010) and hemoglobin concentration (P=0.035) in ICU, acute physiology and chronic health evaluation (APACHE Ⅱ) score (P=0.001); sequential organ failure assessment (SOFA) score (P=0.010), and postoperative fungal infection (P < 0.001) were risk factors for long-term survival. Cox regression model for multivariate analysis showed that preoperative weight loss > 5 kg (HR 4.44; 95%CI 1.47-13.38; P=0.008), and preoperative albumin < 30 g/L (HR 4.38; 95%CI 1.72-11.12; P=0.002), bronchiolitis obliterans (HR 2.69; 95%CI 1.12-6.50; P=0.027), and postoperative fungal infection (HR 4.85; 95%CI 2.01-11.72; P < 0.001) were independent risk factors for postoperative mortality. CONCLUSION The 5-year survival rate of critically ill patients undergoing surgery for paraneoplastic pemphigus combined with solid tumors is approximately 55.1%, with preoperative weight loss > 5 kg, albumin < 30 g/L, bronchiolitis obliterans and postoperative fungal infection were associated with an increased risk of near- and long-term postoperative mortality.
Adult ; Albumins/therapeutic use* ; Bronchiolitis Obliterans/pathology* ; Critical Illness ; Hemoglobins ; Humans ; Neoplasms/complications* ; Paraneoplastic Syndromes/pathology* ; Pemphigus/drug therapy* ; Retrospective Studies ; Weight Loss

Radiation induced pemphigus, as an example of induced pemphigus, is a rare disease which occurs in patients receiving radiotherapy. A 56-year-old male patient with nasopharyngeal cancer received 3 cycles of chemotherapy and radiotherapy on the neck and anterior chest areas. One month after completion of radio-therapy, eroded and crusted areas developed within the irradiation site. In spite of systemic antibiotic treatment, the skin lesions persisted and spread further to the trunk and upper extremities with new crops of bullae. He was treated with a high dose of prednisolone and the skin lesions cleared. We discuss the suggestive pathogenesis of radiation-induced pemphigus with the comparative results of immunoblotting in various variants of pemphigus.
Cytochrome P-450 CYP1A1 ; Drug Therapy ; Humans ; Immunoblotting ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; Neck ; Pemphigus* ; Prednisolone ; Radiotherapy ; Rare Diseases ; Skin ; Thorax ; Upper Extremity

Bucillamine is a disease modifying anti-rheumatic drug, structurally similar to D-penicillamine. Although D-penicillamine-induced pemphigus has been not infrequently demonstrated, pemphigus associated with bucillamine was rarely reported. We describe a patient complicating pemphigus vulgaris after bucillamine treatment in rheumatoid arthritis (RA) and polymyositis (PM) overlap syndrome. PM and RA overlap syndrome was diagnosed three years ago and bucillamine was administrated for 20 months. Skin lesions including erythematous flaccid blisters on her chest, axillae, and back were occurred and were compatible with pemphigus vulgaris by typical pathology. Withdrawal from bucillamine and prednisolone treatment made rapid improvement of pemphigus lesions.
Syndrome ; Skin/pathology ; Polymyositis/*complications/*drug therapy ; Pemphigus/*chemically induced/*pathology ; Middle Aged ; Humans ; Female ; Cysteine/adverse effects/*analogs & derivatives ; Biopsy ; Arthritis, Rheumatoid/*complications/*drug therapy ; Arthritis ; Antioxidants/*adverse effects