New-onset vitiligo and progression of pre-existing vitiligo during treatment with biological agents in chronic inflammatory diseases.

BACKGROUND: The development of vitiligo during treatment with biological agents is an unusual event and only a few isolated cases have been reported. OBJECTIVES: To describe the clinical characteristics and evolution of patients developing new-onset vitiligo following initiation of a biological agent for chronic inflammatory disease; and also to report the clinical course of pre-existing vitiligo under biological therapy. METHODS: This nationwide multicentre, retrospective study, carried out between July 2013 and January 2015, describes the characteristics of a large series of 18 patients (psoriasis N = 8, inflammatory rheumatic diseases N = 8, ulcerative colitis N = 1, uveitis N = 1) who developed new-onset vitiligo while receiving a biological agent. RESULTS: TNFα inhibitors were the most common biological agent involved (13/18) while anti-IL-12/23 and anti-IL-17 agents or abatacept were less common (4/18 and 1/18 respectively). Mean duration of biological agent exposure before vitiligo onset was 13.9 ± 16.5 months. Outcome was favourable for most patients (15/17) while maintaining the biological agent. Data were also collected for 18 patients (psoriasis N = 5, inflammatory rheumatic diseases N = 10, inflammatory bowel diseases N = 2, SAPHO N = 1) who had pre-existing vitiligo when treatment with a biological agent started (TNFα inhibitors N = 15, ustekinumab N = 1, rituximab N = 1, tocilizumab N = 1). Vitiligo progressed in seven patients and was stable or improved in eight cases. CONCLUSION: Vitiligo may thus emerge and/or progress during treatment with various biological agents, mainly TNFα inhibitors and could be a new paradoxical skin reaction. De novo vitiligo displays a favourable outcome when maintaining the biological agent, whereas the prognosis seems worse in cases of pre-existing vitiligo.

[Day hospital in internal medicine: A chance for ambulatory care]. / L'hôpital de jour en médecine interne : un atout pour le virage ambulatoire.

INTRODUCTION: Internal medicine is an in-hospital speciality. Along with its expertise in rare diseases, it shares with general medicine the global care of patients but its place in the ambulatory shift has yet to be defined. The objective of our work was to evaluate the benefits of an internal medicine day-hospital devoted to general medicine. METHODS: Named "Centre Vi'TAL" to underline the link between the city and the hospital, this novel activity was implemented in order to respond quickly to general practitioners having difficulties to synthesize their complex patients or facing diagnostic or therapeutic problems. Using preferentially email for communication, the general practitioners can contact an internist who is committed to respond on the same day and take over the patient within 7 days if day-hospital is appropriate for his condition. The other patients are directed either to the emergency department, consultation or full hospitalization. RESULTS: In 14 months, the center has received 213 (144 women, 69 men) patients, mean age 53.6, addressed by 88 general practitioners for 282 day-hospital sessions. Requests included problem diagnoses (n=105), synthesis reviews for complex patients (n=65), and treatment (n=43). CONCLUSION: In the ambulatory shift advocated by the authorities, this experience shows that internal medicine should engage in the recognition of day-hospital as a place for diagnosis and synthesis reviews connected with the city while leaving the general practitioners coordinator of their patient care. This activity of synthesis in day-hospital is useful for the patients and efficient for our healthcare system.

[Pseudo-autoinflammatory attacks of temporal arteritis]. / Manifestations d'allure auto-inflammatoire d'une maladie de Horton.

INTRODUCTION: Usually, temporal arteritis progresses as a chronic disease. CASE REPORT: The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved. DISCUSSION: The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

Regression of cutis calcinosis with diltiazem in adult dermatomyositis.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

Sacral insufficiency fractures: an easily overlooked cause of back pain in elderly women.

Sacral insufficiency fractures (SIF) that usually present as nonspecific pelvic or low back pain are often overlooked in the elderly. In a retrospective study conducted in a department of internal medicine, 16 patients with SIF were identified during a 6-year period. All patients were elderly women (mean age of 81 years) who presented with low back or pelvic pain. Radicular pain in the lower limb was common. Ten patients were bedridden. All 16 patients were osteopenic. Plain radiographs of the pelvis were nondiagnostic in 11 patients. Radionuclide bone scan showed a typical H-shaped pattern of increased uptake in 11 patients, and computed tomographic scan confirmed SIF (9/9 patients). With bed rest and analgesics, outcome was favorable in all patients. This study confirms the nonspecific presentation of SIF and the need to maintain a high index of clinical suspicion to make a prompt diagnosis and avoid unnecessary and sometimes invasive procedures.

[Setting up a data collection and assessment system of the Permanent Healthcare Access Activities (PASS)]. / Mise en place d'un système de recueil et d'évaluation de l'activité médicosociale des permanences d'accès aux soins de santé (PASS).

INTRODUCTION: Five years after introducing the Permanent Access to Healthcare activity (PASS), it became necessary to analyse how it works. MATERIAL AND METHODS: A computerized data collection and assessment system intended to evaluate the PASS health activities has been set up in 11 University Hospitals and ten General Hospitals. From January 1st to June, 30th 2003 data was captured in a computer. RESULTS: The patients requiring medical advice are young (with an average age of 35 years) and present several signs of poverty in terms of accommodation, social relationships and financial means. Besides, almost all of them are uninsured. The PASS public corresponds completely to the created system. Poverty risk factors vary according to the geographic origin. Indeed, French people often suffer from isolation, whereas foreign patients present financial problems. The major part of patients are foreigners and more than a third of them do not speak French, which is an additional obstacle to care. Most of the time, the PASS patients present digestive disorders, nevertheless there are some differences between French and foreign patients. Indeed, foreigners very frequently have digestive and osteoarticluar problems, whereas French patients suffer from psychic disorders and present addictive behaviours. Some patients are sent to physicians downtown (9%) and to external medicosocial assistance centres (39.5%). DISCUSSION: This study (first one in France) provides us with homogenous data regarding the activities of PASS centres nationwide. The usefulness of computers and its acceptability facilitate data diffusion, with possibilities of adapting to each centre while preserving a common basis.

[Uterine localization of periarteritis nodosa disclosed by fever of long duration]. / Localisation utérine d'une périartérite noueuse révélée par une fièvre au long cours.

We report the case of an 81 year-old woman admitted because of declining general health, fever and elevated sedimentation rate. Identification of a pelvic mass on the computed tomographic scan led to perform hysterectomy with a working diagnosis of ovarian malignancy. Histological examination revealed typical lesions of polyarteritis nodosa of uterus and fallopian tube vessels. Diffuse process of the vasculitis was suggested by persistence of fever and elevated erythrocyte sedimentation rate after hysterectomy. Treatment consisting of high-dose corticosteroids and pulses of cyclophosphamide resulted in prompt clinical and biological improvement. We discuss the incidence, the clinical features and the localized or diffuse nature of genital involvement in polyarteritis nodosa and other vasculitis.

[Febrile form of ankylosing spondylitis]. / Les formes fébriles de la spondylarthrite ankylosante.

INTRODUCTION: Ankylosing spondylitis is a chronic inflammatory disorder that is rarely associated with severe systemic manifestations. EXEGESIS: We report the case of a 29-year-old man presenting with an atypical ankylosing spondylitis. The patient presented with a high spiking fever, a marked weight loss, and an unusual increase of acute phase response markers. Such features are rarely associated with ankylosing spondylitis and the review of the few similar reported cases in the literature showed that this presentation is usually associated with a severe disease course, and an asymmetrical joint involvement that is unresponsive to nonsteroidal anti-inflammatory agents. CONCLUSION: Clinicians should be aware of this unusual presentation of ankylosing spondylitis, which should be included in the list of aetiologies of fever of unknown origin, to avoid useless diagnostic procedures. The severity of the disease course may lead to the consideration of a more aggressive treatment strategy.

[Fracture of the sacrum caused by bone insufficiency in the elderly: apropos of 14 cases]. / Fracture du sacrum par insuffisance osseuse chez le sujet âgé: à propos de 14 observations.

We report a series of 14 elderly patients with sacral insufficiency fractures of the sacrum. All patients presented with pain in low back, pelvis or lower limb which was often misleading. Bone scintigraphy was diagnostic in all the patients. Outcome was favourable in all the cases.

[Pancytopenia in acute brucellosis]. / Pancytopénie au cours d'une brucellose aiguë.

Leuconeutropenia is a common manifestation of acute brucellosis, whereas other hematological abnormalities and pancytopenia are uncommon. We report a patient presenting with acute brucellosis and pancytopenia.

[Adult-onset Still's disease after hepatitis A and B vaccination?]. / Maladie de Still de l'adulte après vaccination contre l'hépatite A et B?

BACKGROUND: Hepatitis A and B vaccination are generally very well tolerated. However, exceptional cases of arthritis and systemic diseases have been reported after hepatitis B vaccination. CASE REPORT: The authors report a case of adult Still's disease apparently triggered by hepatitis A and B vaccination. The patient was a 38-year-old woman who presented with fever, hepatitis, pneumonitis and neurologic symptoms, compatible with the diagnosis of adult Still's disease. DISCUSSION: The authors discuss the rarity of systemic diseases triggered by such vaccination, the link between hepatitis B virus and some vasculitis, and the possibility for viral bacterial infections to trigger adult Still's disease.

[Relapsing polychondritis, thrombosis and antiphospholipid antibodies]. / Polychondrite atrophiante, thromboses et anticorps antiphospholipides.

The authors report a case of relapsing polychondritis associated with antiphospholipid antibodies. The initial manifestations were recurrent venous thrombosis which occurred prior to the chondritis. Antiphospholipid antibodies are found in various diseases, some of which are sometimes associated to relapsing polychondritis. However, antiphospholipid-antibodies have not yet been reported in this disease.

[Psoriasis. Rheumatologic manifestations]. / Psoriasis. Manifestations rhumatologiques.

THE CONTEXT: Psoriasic arthritis lies somewhere between rhumatoid polyarthritis and spondyloathropathy. Its prevalence is about 0.1% with a 1/1 sex ratio. Mean age at onset of symptoms is 40 years. In 10 to 15% of the cases, rhumatological manifestations are observed before skin lesions. Ungueal involvement is particularly frequent. FIVE CLINICAL FORMS: Classically, five clinical forms are described: arthritis limited to the distal interphalangeal joints, mutilating arthritis, symmetrical polyarthritis, asymmetrical mono- or oligoarthritis, and spondylitis. Asymmetrical oligoarticular forms and polyarithrtis predominate. DISEASE SEVERITY: In general psoriasic arthritis is a benign condition. Severe forms have however been described with erosion and osteolysis involving the distal interphalangeal joints. Typical radiological may be observed. THERAPEUTIC OPTIONS: Non-steroidal antiinflammatory drugs help control disease progression in about one-third of the cases. In other patients, gold salts, D-penicillamine, methotrexate, or sulfasalazine may be required.

[Facial diplegias during pregnancy]. / Diplégies faciales au cours de la grossesse.

BACKGROUND: Facial diplegia is a rare event, most commonly of unknown origin. We report the case of a woman who presented bilateral Bell's palsy a few days after a normal delivery. CASE REPORT: Five days after the delivery of gemellary pregnancy, a 34-year old woman developed complete bilateral facial palsy. No treatment was initially prescribed. She was first seen in our department two weeks after the onset of her illness. The diagnostic work-up was negative and we considered that our patient had bilateral Bell's palsy. Treatment with methylprednisolone and intravenous acyclovir, initiated since admission, have had very limited effect. DISCUSSION: As has already been shown for facial palsy, idiopathic facial diplegia, although exceptional, seems to be more frequent during the last trimester of pregnancy and in the early puerperium. Seven cases have been reported in the literature over the last 30 years. We discuss here the pathophysiology.

[Pneumatosis cystoides intestinalis in systemic diseases: 3 cases]. / Pneumatose kystique intestinale au cours d'une maladie systémique. 3 observations.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is usually a complication of digestive tract or respiratory tract diseases, but rare cases have been described in systemic diseases, mainly systemic sclerosis. CASE REPORTS: Three patients, one with temporal arteritis and two with polyarteritis nodosa (complicating rheumatoid arthritis in one case) were treated by prednisone. All three developed PCI, complicated in one case by a retropneumoperitoneum. Medical treatment led to a favorable outcome in all cases. DISCUSSION: Sixty-two cases of PCI have been reported in patients with various systemic diseases (systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, dermatopolymyositis, polyarteritis nodosa, rheumatoid arthritis, Sjögren's syndrome, amyloidosis). Systemic sclerosis is the most frequent condition (45%). In the other cases, corticosteroid therapy or digestive tract vasculitis are the main causal factors. Outcome is usually favorable with medical treatment. Laparotomy is rarely needed.

[Pneumatosis cystoides intestinalis]. / Pneumatose kystique intestinale.

AIR-FILLED CYSTS: Pneumatosis cystoides intestinalis (PCI) is a benign air-filled cystic formation lying in submucosal or subserous digestive tissue. PCI has been reported all along the digestive tract. CLINICAL SIGNS: Manifestations of PCI vary greatly. Some cases are asymptomatic, while others are revealed by abdominal pain or pneumoperitoneum. Outcome is usually favorable. EXPLORATION: The plain radiogram of the abdomen shows gaseous cysts of various forms lying between the liver and the diaphragm. Pneumoperitoneum may be present. Computed tomography is the ideal diagnostic test. Endoscopy may be useful for colonic localizations. PATHOGENESIS: Many causes have been suggested and debated. Fifteen percent of all cases of PCI are idiopathic. In the other cases, digestive tract or respiratory tract diseases, are usually the underlying cause. Exceptionally systemic disease may be associated with PCI, particularly systemic sclerosis. TREATMENT: Surgery should be reserved for particularly severe cases.