Systemic lupus erythematosus arising in a patient with epidermodysplasia verruciformis

Few cases have been published relating systemic lupus erythematosus (SLE) and epidermodysplasia verruciformis (EV). We describe a patient with this association. A 22-year-old female with classical skin lesions of EV since childhood together with systemic lupus erythematosus (SLE) which developed 10 years later. According to the knowledge of the authors, this case is the third report of association of EV and SLE and the first report of a patient with EV who developed SLE several years after initiation of EV.     

Systemic lupus erythematosus and multiple myeloma: A rare association

Objective:

The association of systemic lupus erythematosus (SLE) and multiplemyeloma (MM) is an uncommon event. We report the relapse of SLE in a patient with a previous history of MM, treated with chemotherapy and, subsequently, with alpha-2b interferon (α-2b IFN) as a maintenance therapy. The case is discussed in light of past relevant literature.

Methods:

The history and clinical, laboratory and radiographic findings of the patient, as well as the subsequent therapeutic approach are discussed. In our review of the literature, journal articles are identified by Medline search.

Results:

We describe the case of a woman who developed a multiple myeloma14 years after a diagnosis of SLE. A careful literature review confirms that the association of these two diseases has been reported only in a few cases. When the plasma cell neoplasia occurred, SLE had been quiescent for several years; the patient was treated with prednisone-melphalan and, subsequently, with α-2b IFN as a maintenance therapy. On admission to our department, SLE was in a relapse phase, probably because of IFN treatment. The disease was poorly responsive to steroid therapy and required the use of cytotoxic drugs.

Conclusions:

The coexistence of SLE and MM is very rare and the possible pathogenetic mechanisms underlying this association remain unclear. The use of interferon in a patient with an autoimmune disease always invites caution.     

Myelopathy revealing lupus. Two cases and review of the literature

INTRODUCTION: Myelopathy is a rare manifestation of systemic lupus erythematosus, occurring most often during the course of the disease. EXEGESIS: We report two cases of women with myelopathy as the first manifestation of systemic lupus erythematosus; both had an unusual course. We review the literature for previously reported cases. CONCLUSION: The clinical presentation of myelitis is heterogeneous. Usually, neurologic deficits evolve within a few hours (typically acute transverse myelitis) and outcome is usually poor. However, chronic or recurrent transverse myelitis has also been reported, including relapsing myelitis that resolved spontaneously. Myelopathy can be the first manifestation of the disease and this might be more common than initially thought. Magnetic resonance imaging (MRI) findings depend on the timing of the examination and the stage of the disease; the MRI may therefore be normal. An association with optic neuritis is frequently reported in the literature and differential diagnosis with multiple sclerosis may be difficult. Overlapping features between both diseases have been termed "lupoid sclerosis" and are actually classified as demyelinating syndromes associated with lupus. Myelopathy does not appear to be consistently associated with antiphospholipid antibodies, as has been previously suggested. The best treatment protocol has not been determined; however, in recent years, pulses of methylprednisolone and cyclophosphamide have gained acceptance by most authors.     

Chilblain lupus erythematosus—a review of literature

Chilblain lupus erythematosus (CHLE) is a rare, chronic form of cutaneous lupus erythematosus. Sporadic cases and two families with autosomal dominant-inherited CHLE have been reported. In familial CHLE, two missense mutations in TREX1 encoding the 3'-5' repair exonuclease 1 were described in affected individuals. The pathogenesis of sporadic CHLE remains unknown. Up to 20% of patients develop systemic lupus erythematosus (SLE). An association with anorexia is discussed. In many cases, there is good response to symptomatic therapy. SLE therapeutics have good effects on SLE-typical symptoms but not on chilblains themselves. This article reviews the clinical presentation, pathogenesis, diagnosis and treatment of CHLE. As an index patient with unique features, we report a 13-year-old boy developing CHLE after anorexia nervosa. Sequencing of TREX1 was normal. With psychotherapeutic support for anorexia and after antibiotic therapy, topical steroids, physical warming and calcium channel blockers, the patient experienced significant relief. Improvement of phalangeal perfusion was demonstrated by angio-MRI.     

Early onset multiple myeloma in a patient with systemic lupus erythematosus: a case report and literature review

Patients with systemic lupus erythematosus (SLE) are at increased risk for various plasma cell dyscrasias, but the coexistence of SLE and multiple myeloma (MM) are rarely reported to date. Due to the rarity, the clinical features of MM associated with SLE have not been elucidated, and the pathogenesis under this association remains unclear. In this report, we investigate a 31-year-old woman with 5-year history of SLE, who is diagnosed as IgA λ-type MM with multiple lymph node involvement. We discuss the clinical features of MM in SLE by reviewing previous cases and possible mechanisms connecting the two conditions.     

Two cases of lupus cystitis with no bladder irritation symptoms

Lupus cystitis is a rare manifestation in systemic lupus erythematosus (SLE); it usually occurs in association with gastrointestinal manifestations. We report two cases of lupus cystitis without bladder irritation symptoms. Both cases developed severe abdominal pain, nausea, and diarrhea and showed no bladder irritation symptoms. The diagnosis of lupus cystitis was made by abdominal ultrasonography and bladder biopsy. The patients were treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone. Their symptoms were ameliorated, and hydroureteronephrosis improved. Thus, when a patient with SLE shows gastrointestinal symptoms, further examinations are required to determine whether the patient has lupus cystitis.     

Can immunization precipitate connective tissue disease? Report of five cases of systemic lupus erythematosus and review of the literature

OBJECTIVES: To report a series of five patients who developed systemic lupus erythematosus (SLE) after immunization and review the literature on vaccine-associated connective tissue diseases and the theoretical mechanisms that could explain such an association. METHODS: Uncontrolled retrospective analysis of cases identified sporadically over 7 years at three centers. RESULTS: In our series of 5 patients, symptoms of SLE developed within 2 to 3 weeks after secondary immunization. All patients met American College of Rheumatology (ACR) criteria for the diagnosis of SLE. In most patients, symptoms have been persistent. CONCLUSION: Although a coincidental association between vaccination and the onset of SLE cannot be excluded, the temporal relationship with the development of symptoms makes it immunologically plausible that vaccination triggered systemic autoimmunity in these rare cases. We propose that epidemiological studies be performed to examine this potential association in more detail to quantitate the risk and identify possible genetic risk factors.     

Excellent Effect of Steroid plus Azathioprine in a Young Woman with Pernicious Anaemia and Systemic Lupus Erythematosus

We describe a 29-year-old woman who developed pernicious anaemia 2 years after the diagnosis of systemic lupus erythematosus. This is a rare association despite the relationship between the autoimmune aetiologies of these two conditions. Seven other cases have been described, but our report demonstrates a case with an excellent response to steroid and azathioprine. Received: 3 November 1999 / Accepted: 20 April 2000     

Psoriasis and systemic lupus erythematosus: a rare association with specific therapeutic problems

The association psoriasis and systemic lupus erythematosus (SLE) is a very uncommon association. We report three cases, diagnosed in an Internal Medicine department between 1993 and 2000. Few cases of psoriasis/SLE have been published in the literature. Psoriasis generally precedes the diagnosis of SLE. Psoriasis can also be associated with discoid lupus erythematosus. In some cases, SLE appears as a complication of ultraviolet phototherapy indicated for the psoriasis. The association psoriasis/SLE does not seem to have distinctive immunologic features. Specific therapeutic difficulties may occur. Indeed, hydroxychloroquine may exacerbate the psoriasis. Systemic use of corticosteroids raises the risk of severe psoriasis relapse during withdrawal. In addition, the diagnosis of psoriasic arthropathy is more difficult in this setting. The psoriasis/SLE association might be a good indication for using methotrexate.     

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Introduction

. Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis.

Case reports

. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded.

Conclusion

. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.     

Pathogenic links between Kikuchi's disease and lupus: a report of three new cases

INTRODUCTION: Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto's disease is a rare anatomoclinical entity whose etiology remains unknown. It is mainly reported in young adult female, presenting with cervical lymphadenopathies, fever and asthenia. The diagnosis is based on the histological examination of a lymph node biopsy. The disease course is usually uneventful, but sometimes Kikuchi-Fujimoto's disease can reveal or evolve into a cutaneous or a systemic lupus. EXEGESIS: We report three new cases of Kikuchi's disease: the first one mimicked a systemic lupus, the second one was associated with a lupus-like rash, and a the last one was a severe case with hemophagocytic syndrome and a primo-infection with Epstein-Barr virus revealing a systemic lupus erythematosus. CONCLUSION: Clinical and biological follow-up of patients presenting with Kikuchi's disease is necessary to look for an association with a lupus. We discuss the pathogenic links between Kikuchi's disease and lupus.     

Systemic Lupus Erythematosus and Gastric Carcinoma: A Report of Two Cases and a Critical Review of the Literature

Two cases of systemic lupus erythematosus in association with primary gastric adenocarcinoma are presented. A pathological analysis of the two cases of gastric cancer does not support the hypothesis that lupus and its associated vasculitis is involved in the pathogenesis of gastric carcinoma. A critical review of the literature reveals numerous case reports suggesting the association of systemic lupus erythematosus with various malignancies; however, there is no definitive study which substantiates the association of gastric or other cancers with lupus. Future studies will he required to assess appropriately the possibility of an association between lupus and cancer.     

Association of disseminated lupus erythematosus and Crohn's disease

INTRODUCTION: Association of Crohn's disease and systemic lupus erythematosus is infrequent. We report an observation of Crohn's disease that appeared in an 18-year-old woman followed-up for systemic lupus erythematosus for four years. EXEGESIS: The patient had all the clinical, biological and histological criteria of Crohn's disease and systemic lupus erythematosus was diagnosed according to the American Rheumatism Association criteria. On the base of this observation, we discuss the digestive manifestation of systemic lupus erythematosus and extradigestive manifestations of Crohn's disease. CONCLUSION: The immunological background of both diseases was proposed to explain the mechanism of this rare association.     

CONNECTIVE TISSUE DISEASE MIMICKING MULTIPLE SCLEROSIS

Abstract Systemic lupus erythematosus, primary Sjögren's syndrome and systemic sclerosis may be associated with acute transverse myelitis and chronic relapsing neurological syndromes mimicking multiple sclerosis in the same individuals and/or their relatives. We now present three cases which suggest that there is a wide spectrum of connective tissue disorders mimicking multiple sclerosis and acute disseminated encephalomyelitis. These cases demonstrate that the diagnosis of multiple sclerosis should be kept under constant review by searching for the development of connective tissue disorders in the patients or their relatives.     

Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.

Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). We report here 9 patients with chilblain cutaneous lesions, 6 of them were affected by SLE and 2 by SCLE. The onset of CL preceded the diagnosis of LE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the remaining 4 cases. Raynaud's phenomenon and photosensitivity were other prominent clinical features in patients with CL. Nailfold capillaroscopy revealed pathological changes in every patient examined. ANA and anti-SSA/Ro antibodies were detected in all nine patients. Anti-SSB/La were detected in 2 cases, anti-Sm in one case, and anti-Sm and anti-RNP in a one case. Antibodies to dsDNA and complement consumption were found in the six patients with SLE. The fine specificity of anti-SSA/Ro was determined by immunoblotting: anti-60kD and anti-52 kD were detected in three sera, anti-60kD alone in 5 sera, while one serum did not blot. In conclusion, the present study suggests that chilblain LE is associated with SSA/Ro autoantibodies, as is SCLE, hypergammaglobulinemic purpura and neonatal lupus erythematosus.     

Primary sclerosing cholangitis and systemic lupus erythematosus

We report the case of a patient with primary sclerosing cholangitis associated with systemic lupus erythematosus. Only one similar case has been previously reported. The relationship between the two diseases is discussed.     

Recurrent Kikuchi's disease in a patient with discoid lupus

INTRODUCTION: Histiocytic necrotizing lymphadenitis, also called Kikuchi-Fujimoto's disease (KD), usually occurs in young women and is characterized by localized lymphadenitis (mostly cervical) usually associated with fever. Diagnosis relies on histopathological and immunohistochemical analysis of involved lymph node. KD is considered to be the clinical expression of an inadequate immunological reaction, resulting in a self-limited hyperstimulation of the lymphoid tissue, trigerred by an antigenic infectious stimulation. The clinical course is usually benign with a spontaneous remission of symptoms. Recurrence is very rarely observed. KD may be associated with systemic lupus erythematosus, whereas the association with chronic discoid lupus has only been rarely reported. EXEGESIS: We report a case of KD recurrence, 9 years after the initial diagnosis, in a young eurasian woman, with a chronic discoid lupus erythematosus. CONCLUSION: KD is a benign disease with a spontaneously good outcome. Recurrence may rarely occur many years after the initial diagnosis but needs a new histopathological confirmation. Systemic or even cutaneous lupus erythematosus may be associated with KD. Although unfrequent, this association suggests that both diseases could share a common pathogenesis.     

La pancréatite lupique : une série de 6 cas

Purpose

The onset of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and exceptionally revealing. Its pathogenesis is multifactorial, it is extremely difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of antiphospholipid syndrome, or iatrogenic complications or intercurrent. We present six cases of lupus pancreatitis and discuss the difficulty of diagnosis.

Methods

A retrospective monocenter study of 110 patients with systemic lupus erythematosus. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Pancreatitis was noted in 6 patients (5.4%). There were five female and one male patients; the mean age of these patients was 36.3 years. In four patients pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3) and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in 5 patients, and one patient died.

Conclusion

Most cases of lupus pancreatitis have been described in patients with multiple organ involvement. It is important to recognize the etiology of pancreatitis in the course of SLE (drug, lupus, gallstones ??) because the therapeutic depends on it.