Clinical and immunological features and outcome of anti-p200 pemphigoid.

BACKGROUND: Anti-p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin γ1. The clinical course of anti-p200 pemphigoid in patients remains poorly investigated. OBJECTIVES: We aimed to describe the clinical and immunological features and the course of a series of patients with anti-p200 pemphigoid. METHODS: We conducted a retrospective study by immunoblotting detection of sera on 200-kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients. RESULTS: A total of 14 patients with a mean age 81·6 ± 6·5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including eczematous, urticarial, prurigo-like, dyshydrosis-like and rosette-like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200-kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme-linked immunosorbent assay detected recombinant human laminin γ1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow-up time of 12·6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy. CONCLUSIONS: Many patients with anti-p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected.

[Flagellate erythema: an uncommon side effect of bleomycin]. / L'érythème flagellé: une complication rare de la bléomycine.

Bleomycin is a cytotoxic agent used in the treatment of various neoplasias. Its cutaneous adverse effects are diverse. Some of them are rare but specific. We report the case of a 40-year-old man presenting with a non-seminomatous testicular germ cell tumour who developed a flagellate erythema related to a bleomycin administration. Clinical features, histopathology and disease course are presented. This side effect is apparently neither related to the dose nor to the mode of administration of bleomycin. The etiopathogenic mechanism remains unknown.

[Auto-inflammatory disease with hyperimmunoglobulinemia D of late onset]. / Maladie auto-inflammatoire avec hyper-IgD de révélation tardive.

BACKGROUND: The term "auto-inflammatory diseases" encompasses a group of disorders characterised by febrile episodes of sudden onset associated with joint, abdominal, lymph node and cutaneous signs, each presenting a genetic and/or laboratory specificity allowing their identification. Polyclonal elevation of serum IgD is highly suggestive of hyper-IgD syndrome, a disease with autosomal recessive transmission that usually begins before the age of one year. CASE REPORT: We report the case of a 46-year-old woman presenting a disease since the age of 30 years clinically very similar to hyper-IgD syndrome except for ocular and pulmonary involvement. However, tests revealed neither mevalonate kinase gene mutation nor elevation of urinary mevalonic acid. The near-monthly attacks were controlled with mycophenolate mofetil. DISCUSSION: Three cases with identical clinical and laboratory profiles have been reported in the literature and diagnosed as late-onset hyper-IgD syndrome. Our case is the only one involving indirect screening for mutation of the mevalonate kinase gene, which proved negative. This finding rules out the diagnosis of classic hyper-IgD syndrome in our case and raises the possibility of auto-inflammatory disease with reactional hyper-IgD of a different cause, either acquired or secondary to an as yet unidentified mutation.

[Impetigo herpetiformis and Ondine curse]. / Impétigo herpétiforme et syndrome d'Ondine.

INTRODUCTION: Impetigo herpetiformis is a rare dermatitis that occurs during pregnancy and may be life threatening for both mother and child. In this case report, we present an Ondine curse involving the baby, and the good response to isotretinoine. CASE REPORT: A first pregnancy, 26 year-old woman developed at 8 months a widespread skin lesion involving the medial side of the thighs, abdomen and intertriginous areas, with a severe systemic toxic condition and fever. Diagnosis of impetigo herpetiformis was made and corticosteroids, methotrexate and cyclosporine were unsuccessful. Isotretinoine rapidly improved the patient with good control of the disease. The full term baby had an Ondine curse. DISCUSSION: Our case is typical of impetigo herpetiformis. Maternal and infant complication may be life threatening and we report a real Ondine curse the etiology of which remains unknown. Moreover, this observation is unusual because the lesions did not clear despite delivery and good treatment. In our opinion, the great improvement with isotretinoine would suggest it could be used as first line treatment.

Cannabis arteritis.

The main causes of arteriopathy in young patients include drugs, metabolic diseases, pseudoxanthoma elasticum and Buerger's disease. Arteritis due to Cannabis indica was first reported in 1960, and the role of this drug as a risk factor for arteritis was confirmed in several subsequent publications. A 38-year-old smoker with no previous contributory medical history except for long-standing cannabis abuse developed a dry necrotic lesion of the left big toe. Imaging examinations revealed proximal arteriopathy of the lower limbs that predominated on the left side. He had no atherogenic or thrombogenic risk factors, and no signs of pseudoxanthoma elasticum were found. Remarkably, the development of arteritis paralleled cannabis abuse. The course was slowly favourable after weaning from the drug, vasodilator treatment and hyperbaric oxygen therapy. Despite some subtle clinical differences (more proximal than distal involvement), cannabis arteritis may be considered as a particular form of Buerger's disease, where cannabis, along with tobacco, seems to cause arterial lesions. Along with the noxious effects of cannabis on vessels, a role for contaminating arsenic is also possible. Cannabis arteritis is not widely known, but may prove not to be so rare if one considers consumption of cannabis besides that of tobacco.

Capillary leak syndrome induced by acitretin.

The mucocutaneous side-effects of systemic retinoids are well known. We report a patient with diffuse oedema, which is a very rare side-effect of retinoids. The pathophysiology of this condition is poorly known because of the rarity of relevant observations, but apparently corresponds to capillary leak syndrome. The outcome is invariably favourable following withdrawal of the drug, which should be definitively contraindicated in the patient.

[Gemcitabine-induced thrombotic microangiopathy]. / Microangiopathie thrombotique due à la gemcitabine.

INTRODUCTION: Thrombotic microangiopathy (TMA) regroups the hemolytic and uremic syndrome (HUS) and thrombocytopenic thrombotic purpura (TTP). The TMA associated with cancer can be secondary to cancer, hence similar to TTP, or to chemotherapy, creating an HUS. Gemcitabine, used in the treatment of pulmonary, pancreatic and urothelial carcinomas, is generally well tolerated, but has recently been implied in the occurrence of TMA. OBSERVATION: In a patient treated for a metastatic urothelial carcinoma, HUS developed after 8 cues of gemcitabine used alone. After symptomatic treatment and withdrawal of gemcitabine, the hematological abnormalities disappeared and renal function returned to preceding values. DISCUSSION: The incidence of TMA is of around 5 to 6% of metastatic carcinomas. Gemcitabine-induced TMA are of recent occurrence and some twelve cases have been reported. Their occurrence is delayed with regard to the initiation of gemcitabine. They lead to HUS with good prognosis since, on withdrawal of gemcitabine the renal abnormalities regress. Search for TMA should therefore be proposed after more than 10 cycles of treatment with gemcitabine.

[Primary infection with West-Nile virus]. / Primo-infection à virus West-Nile.

BACKGROUND: The recent outbreak of an epidemic of West-Nile fever in New-York revealed this virus' responsibility in some cases of fatal encephalitis. However the clinical manifestations of the infection may be mild as in the case reported herein. CASE-REPORT: A 41-year-old woman without previous medical history presented an acute febrile rash after a trip to Senegal. The course was spontaneously favorable after 5 days, with a biphasic fever at day 3. Reverse passive hemagglutination test confirmed the diagnosis of acute West-Nile fever. DISCUSSION: West-Nile virus, a mosquito-borne flavivirus is usually responsible for a flue-like disease presenting a maculopapular rash in half of the cases. The biphasic evolution of fever is suggestive of the diagnosis. The diagnosis relies on serological tests but cross-reactivities with heterologous flaviviruses may render the interpretation difficult. A rapid recovery is usual but fatal meningoencephalitis can occur especially in the elderly. Treatment is supportive and prevention is limitation of human exposure. West-Nile virus is widely distributed (Africa, Asia, Middle East), but is also endemic in european areas and responsible for seasonal infections.

Life-threatening eosinophilic pleuropericardial effusion related to vitamins B5 and H.

OBJECTIVE: To report a case of eosinophilic pleuropericarditis resulting from concomitant use of vitamins B5 and H. CASE SUMMARY: A 76-year-old white woman was admitted to the hospital because of chest pain and dyspnea related to pleurisy and a pericardial tamponade. This patient had no history of allergy and had been taking vitamins B5 and H for two months. Blood tests performed showed an inflammatory syndrome and a high eosinophil concentration (1200-1500 cells/mm3). Pleurocentesis and pericardiotomy yielded a sterile exudative fluid with an eosinophilic infiltrate. There were no nuclear antibodies and no rheumatic factor; screenings for viruses, parasites, bacteria, and malignant tumor were negative. A myelogram, biopsy of the iliac crest bone, and concentration of immunoglobulin E were also normal. After withdrawal of the vitamins, the patient recovered and the eosinophilia disappeared. DISCUSSION: Prolonged hypereosinophilia has marked predilection to damage specific organs, including the heart, but pleuropericardial effusion is uncommon. Drug-related pleuropericarditis usually occurs without an increased eosinophil count. Other drugs responsible for eosinophilic pleuropericarditis are cephalosporins, dantrolene, propylthiouracil, and nitrofurantoin. To our knowledge, this is the first case report of pleuropericarditis related to vitamins B5 and H. CONCLUSIONS: This case suggests that vitamins B5 and H may cause symptomatic, life-threatening, eosinophilic pleuropericarditis. Physicians prescribing these commonly used vitamins should be aware of this potential adverse reaction.

[Spontaneous portal vein thrombosis associated with acute cytomegalovirus infection in an immunocompetent patient]. / Thrombose porte spontanée associée à une infection aiguë à cytomégalovirus chez une patiente immunocompétente.

BACKGROUND: Portal vein thrombosis (PVT) generally results from a local cause (hepatocellular and pancreatic carcinoma). Spontaneous PVT related to a general cause is less common. We report here a case of spontaneous PVT associated with acute cytomegalovirus (CMV) infection in an immunocompetent patient. CASE REPORT: A 31-year-old white female was admitted with fever and lymph node enlargement. The patient was a heavy smoker and was taking oral contraception. Blood tests revealed an inflammatory syndrome. Liver enzymes were elevated (2N) and platelet count was 118 G/l. Abdominal ultrasonography disclosed partial portal thrombosis. Computed tomography did not show any evidence of a hepatic or pancreatic mass. Protein S, protein C, and anti-thrombin III levels were normal. No antiphospholipid antibodies, no mutation for factors II and V and no abnormality suggestive of paroxysmal nocturnal hemoglobinuria at flow cytometry could be demonstrated. Acute CMV infection was diagnosis with positive viremia (PCR). The patient was given anticoagulation treatment and has done well with no relapse. CONCLUSION: Cytomegalovirus, by damaging endothelial cells, is thought to trigger a cascade of events leading to deep vein thrombosis. CMV infection does not appear to act alone on endothelial cells but rather behaves as an associated factor.

[Transformation of mycosis fungoides to pleomorphic T-cell lymphoma and central nervous system involvement]. / Mycosis fongoïde avec transformation en un lymphome cutané pléiomorphe et localisation secondaire cérébrale.

INTRODUCTION: Although mycosis fungoides is a malignant T-cell lymphoma involving mainly the skin, neurological complications are possible, with a poor prognosis. EXEGESIS: A 59-year-old man, treated for mycosis fungoides with transformation to a pleomorphic T-cell lymphoma for 1 year, was seen for mental status changes with confusion. A brain parenchyma localisation was found. CONCLUSION: This observation emphasizes the exceptional neurological tropism in the patients with mycosis fungoides. A transformation to a more aggressive cutaneous T-cell lymphoma seems necessary to induce a central nervous system involvement.