A case of systemic lupus erythematosus complicated with psoriasis vulgaris]

A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.     

Biotin deficiency in amino acid formula nutrition for an infant with milk protein allergy]

We reported a 4-month-old girl with biotin deficiency caused by amino acid formula. Two weeks after birth, she was diagnosed as having a milk protein allergy. After switching to amino acid formula from usual formula, her symptoms and laboratory findings became normal. About three weeks after the beginning of amino acid formula, she developed intractable skin erosions around the eyes, mouth, neck, and anogenital area. By measuring concentrations of some trace elements, she was diagnosed as having a biotin deficit, because of the organic aciduria and undetectable serum biotin concentration. Her serum biotinidase level was normal. Upon administration of oral biotin supplementation, all her symptoms and laboratory findings were dramatically improved. Since amino acid formula contains very few biotin, we should pay attention to biotin deficiency when infants receiving amino acid formula.     

Successful treatment of a pregnant woman with active systemic lupus erythematosus (SLE) and secondary idiopathic thrombocytopenic purpura (ITP) after three sessions of plasma exchange: a case report

We report a case with active systemic lupus erythematosus (SLE) who was treated successfully with plasma exchange (PE). A 26-year-old female, pregnant at 24 weeks, presented to the emergency room with vaginal bleeding, bleeding gums, facial rash, blurry vision, pallor, and lymphadenopathy. A diagnosis of SLE with secondary ITP was made based on her laboratory findings which revealed leucopenia, thrombocytopenia, evidence of hemolysis, positive antinuclear antibody, positive anti-double-stranded DNA antibody, positive lupus anticoagulant, false-positive VDRL, and decreased complement levels. High doses of corticosteroids were given when her illness was worsening with persistent hemolysis and thrombocytopenia. Plasma exchange was conducted after 2 weeks when she was not responding to the conventional therapy. Rapid and excellent responses were achieved when three PE sessions were carried out in combination with the previous therapy. The patient continued PE sessions for another week. A maintenance dose of steroids was prescribed after the sessions. She remained in good condition, on low dose of oral steroids (10 mg), and gave birth to a healthy baby. Based on our case report, plasma exchange initiated early in combination with steroids might be beneficial and could prevent further deterioration in some patients with therapy-resistant active SLE.     

A case report; systemic lupus erythematosus associated with lupus laryngitis]

Systemic lupus erythematosus (SLE) is an autoimmune collagen vascular disease which produces widespread damage to multiple organs. Few studies on laryngeal involvement in SLE have been reported. We report here a case of SLE complicated by lupus laryngitis. A 27-year-old woman was diagnosed as having SLE in October 1996 based on findings of polyarthritis, lymphocytopenia, positive anti-nuclear antibody and anti-Sm antibodies. Polyarthralgia disappeared transiently, and no other clinical symptoms were found. Therefore, she was followed with no medication. She presented hoarseness with high fever and facial erythema in September 1998. Laboratory findings on admission revealed pancytopenia and hypocomplementemia. Anti-nuclear antibody, anti-Sm antibody and anti-RNP antibody were all positive with high titers. Chest X ray examination showed pleural effusion. Laryngoscopy showed a bamboo-joint-like lesion at the middle of the bilateral vocal cords. She was treated with 60 mg of prednisolone (i.v.) which resulted in improvement of hoarseness, pancytopenia, hypocomplementemia and pleuritis. Based on these findings, we diagnosed her hoarseness as the manifestation of laryngitis associated with SLE (lupus laryngitis).     

A case of systemic lupus erythematosus with pure red cell aplasia possibly caused by persistent infection of human parvovirus B 19]

We reported a 68-old female who was diagnosed to have systemic lupus erythematosus 18 years ago. She had been well under 5 mg of prednisolone until 1995, when she felt severe shortness of breath. Laboratory examinations disclosed severe anemia accompanying mild thrombocytopenia. Bone marrow aspiration revealed a complete absence of erythroid progenitor cells. She was diagnosed to have pure red cell aplasia (PRCA) as well as antiphospholipid syndrome. A judicious use of methylprednisolone including pulse therapy resulted in a prompt resolution of anemia as well as thrombocytopenia and the dose of corticosteroid was tapered successfully thereafter. Persistent infection of HPV B 19 in the patient with inactive SLE was considered as a main cause of PRCA because not only IgG-HPV B 19 antibody but viral DNA was demonstrated in her serum at the time of admission. The relationship between PRCA and anti-phospholipid syndrome in this patient was also noted.     

A case of C5 deficiency with polyarthritis]

A 27-year-old female with polyarthritis was found to lack serum complement activity. Her serum CH50 was less than 1.9 U/ml. C5 protein in her serum was less than 2 mg/dl and its activity was not detected. The serum level of the other proteins of complement system examined were within the normal range. At 17 years old, she was diagnosed as rheumatic fever and was admitted to our hospital. She was treated with aminobenzylpenicillin and predonisolone for two months, and she was discharged from our hospital without any abnormalities. But she had no other episode of repeated infections. Family studies of this patient revealed that an elder sister of this patient was also homozygous deficiency of C5 and her parents were considered to be heterozygous deficiency of C5. From these results, the patient was considered to be inherited deficiency of C5.     

Term delivery in a woman with severe congenital neutropenia, treated with growth colony stimulating factor

The patient was diagnosed in childhood as having severe congenital neutropenia and had recurrent admissions with severe infections. In 1987, prior to getting married, she was sterilized. She continued to require i.v. antibiotics when she contracted a severe infection. On one occasion, she was treated with growth colony stimulating factor (G- CSF). Her increased neutrophil count was sustained following this treatment. In June 1993, she wished to start a family and underwent in- vitro fertilization (IVF) treatment. G-CSF was given prior to oocyte retrieval. She conceived on her first cycle and an ultrasound scan revealed a singleton pregnancy. Throughout the course of the pregnancy, her white cell count was monitored closely and remained at <1.0x10(9)/l. The pregnancy progressed uneventfully and at 37 weeks gestation she was admitted for G-CSF injections. At 38 weeks she was delivered of a boy weighing 3350 g, by elective Caesarean section. His white cell count was normal. This is the first case of G-CSF being used before conception and during pregnancy in a patient with congenital neutropenia. It shows that advances in cytokine therapy and close interdisciplinary liaison can lead to a successful outcome and help patients, who would otherwise remain childless, to achieve a family.      

Hyperglycemic hyperosmolar syndrome caused by steroid therapy in a patient with lupus nephritis

A 51-yr-old female was referred to our outpatient clinic for the evaluation of generalized edema. She had been diagnosed with idiopathic thrombocytopenic purpura (ITP). She had taken no medicine. Except for the ITP, she had no history of systemic disease. She was diagnosed with systemic lupus erythematosus. Immunosuppressions consisting of high-dose steroid were started. When preparing the patient for discharge, a generalized myoclonic seizure occurred at the 47th day of admission. At that time, the laboratory and neurology studies showed hyperglycemic hyperosmolar syndrome. Brain MRI and EEG showed brain atrophy without other lesion. The seizure stopped after the blood sugar and serum osmolarity declined below the upper normal limit. The patient became asymptomatic and she was discharged 10 weeks after admission under maintenance therapy with prednisolone, insulin glargine and nateglinide. The patient remained asymptomatic under maintenance therapy with deflazacort and without insulin or medication for blood sugar control.     

Pregnancy outcomes in a patient-heterozygous carrier of R506Q mutation of factor V (Leiden)

The aim was to review currently available evidence on the association between thrombophilia and adverse pregnancy outcomes. Maternal thrombophilia has recently been identified as a major cause of thromboembolism, placental thrombosis and adverse pregnancy outcome including severe preeclampsia, placental abruption, intrauterine growth retardation, recurrent pregnancy loss, and stillbirth. The relatively high prevalence of thrombophilia defects in the general population and the association with adverse maternal and fetal outcomes have prompted obstetricians to focus their interest on this area. We focused on genetic thrombophilias (factor V Leiden mutation) and its possible impact on severe preeclampsia, placental abruption and intrauterine fetal death. A 39-year-old patient was regularly treated at our Department. Her mother had placental abruption and deep venous thrombosis of lower extremities in medical history. Our patient was suffering from multiple sclerosis from 1990. Until this pregnancy she had been pregnant for six times and had delivered one healthy child. She had four cesarean sections, one for preeclampsia (live-born infant died three days after birth), two for placental abruption (both stillbirths), one spontaneous abortion and one artificial abortion. Having in mind her family and medical history, we focused on genetic thrombophilia. Using the PCR-method we identified activated protein C resistance due to factor V Leiden mutation. The patient was treated by low-molecular weight heparin. A healthy infant was born by cesarean section after 37 weeks of gestation. Therapy with low-molecular weight heparin continued for ten days postpartum.     

Quetiapine use for the treatment of manic episode during pregnancy

Summary The foregoing is a case report about a 30-year-old woman, who was referred to our psychiatry clinic with a clinical picture of manic episode, at the 21st week of her first pregnancy. She had a history of bipolar affective disorder for 12 years, had two previous manic episodes and had stopped taking lithium 6 months ago because of her plans to become pregnant. Quetiapine was begun and the dose was slowly increased to 1200 mg/day after 2 weeks. She continued to receive quetiapine throughout her pregnancy. Her obstetrical and perinatal examinations were done by a consultant obstetrician. At the follow-up, she had given birth to a boy, at 39th week of her pregnancy, with an Apgar score of 10. Follow-up of the infant up to 3 months reveals normal physical and psychomotor development. The pros and cons of quetiapine use during pregnancy are discussed.     

Antibody to poly(ADP-ribose) is an indicator of obstetric complications in pregnant patients with systemic lupus erythematosus

Anti-poly(ADP-ribose) antibodies were measured by an enzyme-linked immunosorbent assay (ELISA) in 6 pregnant women with systemic lupus erythematosus (SLE), 11 normal pregnant women and 6 randomly selected female SLE patients. Four pregnant SLE patients who had either an abortion after 13 weeks of gestation or a premature delivery showed very high titers of anti-poly(ADP-ribose) antibodies in week 8 of pregnancy. However, the titers of anti-poly(ADP-ribose) antibodies of all the normal pregnant women were similar to those of non-pregnant female SLE patients, being slightly higher than those of normal non-pregnant women. The isotype of anti-poly(ADP-ribose) antibodies in pregnant SLE patients was IgG, which did not crossreact with either DNA or cardiolipin. The number of pregnant SLE patients tested was small, but the coincidence of abortion with high titers of anti-poly (ADP-ribose) antibody was very close. Therefore, a high titer of anti-poly(ADP-ribose) antibody in pregnant SLE patients seems useful as an indicator of abortion or fetal distress.     

A case of mixed connective tissue disease developed into overlap syndrome of progressive systemic sclerosis, systemic lupus erythematosus, polymyositis and Sj?gren's syndrome

We encountered a patient who developed an overlap syndrome of progressive systemic sclerosis (PSS), systemic lupus erythematosus (SLE), polymyositis (PM) and Sj?gren's syndrome (SjS) while we were treating her for mixed connective tissue disease (MCTD). This 42-year-old woman had been photosensitive since 18 years of age. In 1986, Raynaud's phenomenon, swollen hands and arthralgia appeared; therefore, we started to treat this patient based on a diagnosis of MCTD. At that time, her anti-RNP antibody titer was 82,920, but she was negative to anti-Sm antibody. In 1988, she was admitted to our hospital with chief complaints of aggravation of polyarthralgia and myalgia. On physical examination, she showed difficulty in opening her mouth, systemic dermal sclerosis, a decrease in muscular strength and rales. In laboratory tests, her myogenic enzyme level was increased, and she was found to be positive to LE cells, antinuclear antibody, anti-DNA antibody, anti-ENA antibody and anti-SSA antibody. Furthermore, histological features clearly corresponding to those of PSS were found by skin biopsy, myogenic changes by electromyography, evidence of chronic inflammation of the salivary glands by lip biopsy, and proliferative changes in the mesangium were detected by renal biopsy. The concept of MCTD, especially the differences from overlap syndrome, is vague. Therefore we need further study about many cases. Since there have been no reports on cases having sufficient evidence of the development of the overlap syndrome of PSS, SLE, PM and SjS during a course of MCTD, our patient would provide very useful data contributing to the study of MCTD.     

A case of drug eruption induced by hydroxyzine pamoate]

A 62-year-old woman with rheumatoid arthritis, Basedow's disease and arrhythmia has been treated with antirheumatic, antiarrhythmic drugs and so on. She developed pruritic diffuse erythema with papules on the trunk and extremities 2 days after taking hydroxyzine pamoate for asteatotic eczema. Laboratory data showed increased levels of eosinophils. Histopathological examination revealed a infiltrate of inflammatory cells in the upper dermis. Patch tests with hydroxyzine pamoate and hydroxyzine hydrochloride were positive. From these findings, we diagnosed this case as drug eruption due to hydroxyzine. Her eruption subsided after she discontinued hydroxyzine pamoate and other drugs which were started within 5 days before the onset of the eruption and was treated with systemic steroid, systemic antiallergic drug and topical steroid.     

Epithelioid trophoblastic tumor after induced abortion with previous broad choriocarcinoma: a case report and review of literature

Epithelioid trophoblastic tumor (ETT) is a rare trophoblastic tumor originating from chorionic-type intermediate trophoblasts (ITs). It is usually associated with a prior gestational event. We present a 44-year-old woman who had unusual pregnancy related history. The patient received her second spontaneous abortion at the age of 25 years and had suffered from choriocarcinoma in left board ligament at the age of 29 years. She admitted no more treatment after 3 courses of multiagent chemotherapy when serum β-hCG returned to normal. Then she had Full-term delivery, induced abortion at the ages of 32, 33 years. The patient had high serum levels of beta-human chorionic gonadotropin (6587 IU/L). Microscopically, the tumor was composed of mainly mononuclear tumor cells, grew in cords, nests, and sheets within which were aggregates of hyaline material. Most were with distinct cell borders, eosinophilic cytoplasm. Immunohistochemical staining revealed strong diffuse reactivity for cytokeratins (AE1/AE3, CK18), P63, focal reactivity for beta-human chorionic gonadotropin, human placental lactogen, and inhibin-alpha. The Ki-67 index was 77%. The histological and immunohistochemical features were characteristic of epithelioid trophoblastic tumor. This is the first reported case of these two gestational trophoblastic tumor happened on one person with the intervening normal pregnancy.     

Pregnancy and Systemic Lupus Erythematosus: Review of Clinical Features and Outcome of 51 Pregnancies at a Single Institution

Systemic lupus erythematosus (SLE) is mainly a disease of fertile women and the coexistence of pregnancy is by no means a rare event. How SLE and its treatment affects pregnancy outcome is still a matter of debate. Assessment of the reciprocal clinical impact of SLE and pregnancy was investigated in a cohort study. We reviewed the clinical features, treatment, and outcomes of 43 pregnant SLE patients with 51 pregnancies followed from 1993 to 2007 at a tertiary university hospital. The age of patients was 28.7?±?5.4 years and SLE was diagnosed at age of 23.0?±?6.1 years. Previous manifestations of SLE included lupus nephritis (14 patients) and secondary antiphospholipid syndrome (11 patients). Thirty-five pregnant patients (69%) were in remission for more than 6 months at the onset of pregnancy. Patients were being treated with low doses of prednisone (29), hydroxychloroquine (20), azathioprine (five), acetylsalicylic acid (51), and low molecular weight heparin (13). Sixteen pregnancy-associated flares were documented, mainly during the second trimester (42%) and also in the following year after delivery (25%). Renal involvement was found in 11 cases (68%). Spontaneous abortion occurred in 6%, 16% had premature deliveries, and 74% were delivered at term. No cases of maternal mortality occurred. No cases of fetal malformation were recorded. There was one intrauterine fetal death and one neonatal death at 24 gestational weeks. Pregnant women with SLE are high risk patients, but we had a 90% success rate in our cohort. A control disease activity strategy to target clinical remission is essential.     

A case report of hypertrophic pachymeningitis associated with systemic lupus erythematosus, showing a headache and hearing loss resembling intracranial hypotension]

We report a case of systemic lupus erythematosus (SLE) complicated with hypertrophic pachymeningitis. A 34-year old woman who was diagnosed as SLE in 1985 was admitted to our hospital for a high grade fever and a headache. Laboratory findings showed increased titer of anti-double strand DNA antibody and decreased number of platelets. She complained a severe headache and hearing loss which were worsened by head-up position, resembling the symptoms of intracranial hypotension. MRI findings revealed thickened dura and she was diagnosed as hypertrophic pachymeningitis. Both clinical symptoms and laboratory findings were resolved after methyl-prednisolone pulse therapy followed by a high dose of prednisolone. Although hypertrophic pachymeningitis is a rare complication with SLE, it should be considered in SLE patients with severe headache.     

Do inhaled steroids have similar efficacy? A case of bronchial asthma suggesting different efficacy of inhaled glucocorticosteroids

We report a 35-year-old woman who had had bronchial asthma for 17 years. Her asthma worsened and became unstable on treatment with beclomethasone dipropionate (BDP), budesonide (BUD), and oral glucocorticosteroids (GC). At the age of 31, she had participated in a clinical trial with fluticasone propionate (FP), and after 2 weeks' treatment her asthma was well controlled. Because of pregnancy, her participation was terminated and treatment continued with available inhaled GC; however, the disease deteriorated and treatment with FP was resumed 2 years ago. Lung function normalized after 3 weeks and she has remained clinically stable since.     

Hyperosmolality in central nervous system lupus as a possible complication that results in prolonged consciousness disturbance]

We describe a patient with systemic lupus erythematosus (SLE) with lupus psychosis, who showed prolonged consciousness disturbance due to hyperosmolality. A 51-year-old Japanese woman with SLE was admitted to our hospital for the evaluation and treatment of consciousness disturbance on March 5, 1994. She had not been given prednisolone since 1984, and had been depressive since January 1994. She was diagnosed as active SLE with lupus psychosis due to the presence of thrombocytopenia, proteinuria, positive anti-nuclear antibody (x10,240) as well as the elevation of cerebrospinal fluid (CSF) IL-6 level. A treatment with methylprednisolone (mPL) 100 mg/day was started along with 2 courses of steroid pulse therapy (mPL 1 g/day for 3 consecutive days). She recovered partially from the central nervous system manifestations with a decrease in CSF IL-6 level 2 weeks after this treatment. However, her consciousness level was exacerbated again thereafter. Blood examination disclosed the elevation of plasma osmolality (319 mOsm/kg) with poor responses of plasma antidiuretic hormone (4.6 pg/ml). She died from systemic aspergillosis on April 26, 1994. Pathological examination on autopsy showed no abnormality in hypothalamus and pituitary gland. It is suggested that this patient was complicated with lupus psychosis as well as hyporesponsiveness of osmoreceptor. Rheumatologists should be aware of this complication in patients with CNS lupus as a possible cause for intractable CNS manifestations.     

Kikuchi's lymphadenitis (necrotizing lymphadenitis) and systemic lupus erythematosus: a case report

A 26-year-old Indian lady was admitted for lower abdominal pain, diarrhoea, vomiting, fever and cough. The initial diagnosis was that of peritonitis secondary to ruptured or perforated viscus with lobar pneumonia. On laparotomy, she was found to have necrotizing or Kikuchi's lymphadenitis of the abdominal lymph nodes. The initial two antinuclear antibody (ANA) results came back negative. She was diagnosed to have systemic lupus erythematosus (SLE) when the third sample for ANA came back positive and the double-stranded DNA (dsDNA) antibody test was homogenously positive. This case illustrates a need to be aware that necrotizing lymphadenitis can precede the onset of systemic lupus erythematosus.     

A case of paroxysmal cold hemoglobinuria (PCH) in the elderly found by severe anemia

CASE: 69 year-old female. Two weeks before the admission, the patient had suffered from upper respiratory tract infection. Severe anemia was detected (Hb was 6.0 mg/dl) and she received a transfusion. Four days after the transfusion, she became icteric and was transferred to our hospital on Feb. 3, 1990 for evaluation of anemia. Laboratory data on admission are as follows; Hb 6.3 g/dl, reticulocytes 128%, total bilirubin 4.6 mg/dl, indirect bilirubin 3.4 mg/dl, urobilinogen in urine positive, WR negative, direct anti-globulin test positive, indirect of the test negative, and Donath-Landsteiner test positive (both direct and indirect). Her red blood cells were classified as P2-positive. From these findings, non-syphilitic PCH was diagnosed. She required bed rest and therapy to protect her from cold exposure. The hemoglobin recovered to 8.3 g/dl. The D-L test was positive for 9 months. This is a rare case of PCH because the patient fell ill in old age and PCH was found because of her severe anemia in spite of her unawareness of hemoglobinuria.