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• W161915915 abstract "Dapsone syndrome is characterized by high fever, skin rash, methemoglobinemia, liver toxicity, and generalized lymphadenopathy. The incidence of the dapsone syndrome is increasing with the extensive application of dapsone. However it is rarely reported in pemphigus. We present a case of “infectious mononucleosis-like” dapsone syndrome in a patient with pemphigus vulgaris. The syndrome appeared when he was being treated methylprednisolone, which was commonly used as a treatment for dapsone syndrome. CASE REPORT A 43-year-old man was admitted to our hospital for pemphigus vulgaris. He has been diagnosed with pemphigus vulgaris for 1 year. Pustule and mucosal erosions relapsed 1 month ago after he stopped the treatment with prednisone. Results of blood routine, liver function test on admission were normal. After giving his consent for immunosuppressive therapy, the patient was treated with methylprednisolone (40 mg daily), cyclophosphamide (600 mg weekly) and dapsone (100 mg daily). Within a month a complete remission was achieved and the patient was given 30 mg methylprednisolone daily as maintenance therapy. Thirty-four days after initiation of therapy, he began to have fever (37.8°C), weakness, and sore throat, adenoids. Other general examinations were unremarkable. Upper respiratory tract viral infection was initially suspected and thus antivirus therapy was started. However, the patient developed facial edema and pruritus (Fig. 1). Over the next 3 days, he was febrile up to 39.5 °C. The skin rash worsened to blanching erythema on face and his trunk (Fig. 2). Lymph node enlargement developed in the cervical, axillary and inguinal regions. The white blood cell count increased to 28.9×109/L with a differential of 21% neutrophils, 57% lymphocytes, and 7% monocytes; peripheral atypical lymphocytes 10% (Fig. 3); hemoglobin 114 g/L, hematocrit 33.1%, and a platelet count of 14.1×109/L. Aspartate aminotransferase raised to 136 IU/L, lactate dehydrogenase 414 IU/L. The following laboratory tests showed negative results: serologies for Epstein-Barr virus, herpes simplex virus, Hepatitis A, B and C, human immunodeficiency virus and syphilis; bacterial cultures of blood; Paul-Bunnell test and ELISA results from a series of antinuclear antibodies. Furthermore, chest X-ray was normal. Abdominal ultrasound showed a mildly enlarged lymph node of mesentery at right low abdomen.Fig. 1.: The patient developed facial edema and erythema.Fig. 2.: The skin rash worsened to blanching erythema as well as pigmentation on his back.Fig. 3.: There is 10% atypical lymphocytes (arrow) in peripheral blood of the patient.Given what mentioned above and the following symptoms which resemble “infectious mononucleosis” such as fever, rash, adenoids, lymphadenopathy and mononucleosis with remarkable atypical lymphocytes, “infectious mononucleosis-like” dapsone syndrome was considered. Dapsone was discontinued, while intravenous methylprednisolone 30 mg daily was continued. Seven days later, fever and skin rashes had subsided. His liver function also returned to normal range, and lymphadenopathy resolved before discharge. He will be carefully instructed to avoid dapsone and sulfone-containing medications in the future. DISCUSSION Traditionally, dapsone is used for the treatment of leprosy and dermatitis herpetiformis. Nevertheless, it has been used for a number of dermatologic diseases including Hailey-Hailey disease,1 bullous pemphigoid,2 acne,3 linear IgA dermatosis, urticarial vasculitis, leuko-cytoclastic and other form of vasculitis.4 As a gluco-corticoid-sparing agent in maintenance-phase pemphigus vulgaris, dapsone could reduce steroid dependence.5 The common side-effects of dapsone include lethargy, headache, methaemoglobinaemia and hemolysis. Dapsone syndrome is a more severe reaction, first noted by Lowe and Smith in 1949.6 The syndrome is characterized by high fever, malaise, dermatitis and methemoglobinemia, which will progress to liver toxicity (jaundice, hepatitis and hepatomegaly) and to generalized lymphadenopathy. It may masquerade as a virus-like illness, especially with its delayed pattern of drug-induced rash, as observed in our patient. Acute renal failure,7,8 hypersensitivity pneumonitis,9 cholangitis,10 sensory peripheral neuropathy,11,12 pancreatitis and pleural effusion13 have been reported as a part of the syndrome but rarely as well. Richardus and Smith14 suggested the following criteria for the diagnosis of dapsone syndrome: symptoms appear between the 2nd and 8th week of the commencement of dapsone and disappearing upon discontinuation of the drug; symptoms could not be ascribed to any other drug given simultaneously; symptoms not attributable to lepra reactions; no other diseases liable to cause similar symptoms. At least 2 signs or more symptoms occur in dapsone syndrome: fever, skin eruption, lymphadenopathy and liver abnormalities. Our patient has the following features in common with other patients reported with the dapsone syndrome: onset of illness within 5 weeks of initiating treatment, fever, malaise, hepatitis, delayed drug rash, generalized lymphadenopathy, and remarkably increased peripheral atypical lymphocytosis (leukemoid reaction). He did not exhibit methemoglobinemia. Hemolysis secondary to dapsone hypersensitivity tends to be worse in patients with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency.15 We do not know the G-6-PD status of this patient, but there was no reason to expect him to be deficient. The lack of hemolysis does not rule out the sulfone syndrome, and dapsone could also induce hemolytic anemia and agranulocytosis in the patient with normal G-6-PD.16 The pathogenesis of the dapsone syndrome is not well-known. In the late 1940s and early 1950s, dapsone syndrome was common, which were thought to be due to the large dose usage (300 mg daily). A second rise in cases was reported after 1982 during the implementation of multi-drug therapy for leprosy.14 Whereas, the syndrome is not dose-related with regards to occurrence and severity, and it occurs at various doses, ranging from 50 mg/d to 300 mg/d. Small dose may be dangerous in a previously sensitized person. This is somehow further influenced by individuals with a probable genetic predisposition to the syndrome. Discontinuation of dapsone and early commencement of systemic corticosteroid are vital to decrease mortality and morbidity. Our patient recovered soon after daspone was stopped. Based on anecdotal experience, dapsone treatment with systemic corticosteroids is effective and widely used. In our patient, the syndrome appeared when he was being treated methylprednisolone and immunosuppressant (CTX) for pemphigus vulgaris. Why methylprednisolone could not prevent its occurrence? We are only aware of two other cases in which dapsone syndrome occurred when steroids were already in use. One case was a patient with systemic lupus erythematosus complicated by other medications (retinoid, thalidomide, chloroquine).17 The other case was a patient with pemphigus vulgaris18 without other immunosuppressant. Both pemphigus vulgaris and systemic lupus erythematosus are autoimmune diseases, and dapsone can be used as an adjuvant for their treatments. Whether the autoimmune disorder in pemphigus vulgaris and systemic lupus erythematosus a high-risk factor to dapsone syndrome is unknown, and further studies should be carried out to identify high-risk patients to determine optimal treatment of this syndrome. Dapsone syndrome in a patient receiving steroids for pemphigus vulgaris was first reported by Barnard.18 Dapsone has been applied to conditions as diverse as asthma,19,20Pneumocystis carinii pneumonia,21 rheumatoid arthritis,22 Melkersson-Rosenthal syndrome.23 With the increasing use of dapsone, this syndrome has been reported in cases other than dermatosis such as Pneumocystis carinii pneumonia in HIV, patient after hematopoietic stem cell transplant,24 cutaneous lupus erythematosus.25 Physicians should be alert for other cases of the dapsone syndrome. Monitoring liver function, blood routine and rash are necessary when dapsone is in use." @default.
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• W161915915 title "Dapsone-induced infectious mononucleosis-like syndrome in a patient with pemphigus vulgaris" @default.
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