[Dual localisation of ischaemic fasciitis with local relapse at one site]. / Une double localisation de fasciite ischémique avec récidive locale sur un des sites.

INTRODUCTION: Ischaemic fasciitis (IF) is a rare pseudosarcomatous proliferation initially described on the pressure points of long-term bedridden patients. Healing is the rule after surgical excision. No multifocal localisations have been reported to date. Herein, we describe the case of a patient with FI affecting two sites and having recurred at one of them. OBSERVATION: A 50-year-old woman with scoliosis and mental retardation consulted for a hard skin lesion next to her right scapula. Elastofibroma was diagnosed on the basis of a surgical biopsy sample. Early local recurrence led to a second resection four months later. Histologically, central fibrinoid necrosis was observed, surrounded by collagenous tissue containing occasionally atypical fibroblasts and numerous capillaries. The diagnosis of ischaemic fasciitis was made by a national expert. Six months later, we observed a 13-cm purplish erythematous infiltrated mass with a 6-cm ulceration at the surgical site. A second 6-cm non-ulcerated indurated purplish lesion was visible next to the right greater trochanter. The scan showed deep soft-tissue infiltration with subfascial extension and contact with the greater trochanter. Superficial biopsies of both lesions showed only an appearance of granulation tissue. MRI performed after five months showed an extension of lesions at the two sites with an appearance evocative of ischaemic fasciitis. Surgical excision was refused by the patient and her family. DISCUSSION: We report a rare case of ischaemic fasciitis at two separate sites with local relapse after surgical excision.

[D-penicillamine-induced pemphigus: changes in anti-32-2B immunostaining patterns]. / Autonomisation d'un pemphigus induit par la D-pénicillamine: évolution de l'immunomarquage par l'anticorps anti-32-2B.

BACKGROUND: It has been reported that D-penicillamine causes pemphigus that is typically superficial. Immunostaining with monoclonal anti-32-2B antibody targeting desmoglein 1 and 3 can help differentiate between drug-induced and classical auto-immune pemphigus. Absence of specific staining militates in favour of drug-induced pemphigus whilst positive staining suggests an auto-immune aetiology that is ongoing despite discontinuation of drug therapy. PATIENTS AND METHODS: A 59-year-old male patient was referred for management of superficial pemphigus 1 year after starting D-penicillamine treatment for scleroderma. The diagnosis of pemphigus was confirmed histologically (intra-epidermal cleavage, acantholysis and perikeratinocytes, deposition of IgG and complement C3). Immunochemical staining with anti-32-2B antibody was initially normal, in keeping with drug-induced pemphigus. Despite discontinuation of D-penicillamine, pemphigus recurred in 2008. A further skin biopsy was undertaken and anti-32-2B staining was abnormal, which is consistent with auto-immune pemphigus. DISCUSSION: Numerous cases of drug-induced pemphigus have been described in the literature. In approximately half of all cases, the pemphigus recedes after cessation of the causative drug. However, there have been no previous reports that changes over time in the immunostaining with anti-32-2B antibodies can mirror a change in form of pemphigus from a drug-induced type to an idiopathic type as well as the associated clinical feature of persistence after drug withdrawal. CONCLUSION: Normal staining with anti-32-2B antibody is associated with a favourable prognosis as regards resolution of drug-induced pemphigus. When, as in this case, status changes to abnormal staining, there is a risk that the pemphigus may become chronic despite discontinuation of therapy.

Visceral and mucosal involvement in neonatal haemangiomatosis.

BACKGROUND: Two types of neonatal haemangiomatosis (NH) are distinguished: diffuse which is associated with a high rate of mortality linked to mucosal/visceral involvement, and benign. OBJECTIVES: First, this study aimed to examine the frequency of mucosal and visceral (especially hepatic) involvement in NH, according to skin extension, and second, it aimed to examine clinical, pathological (with glucose transporter 1 (GLUT-1) immunostaining), and imaging features of NH, including follow-up data. METHODS: This was a descriptive retrospective study carried out in the University Hospital Center of Tours, France. RESULTS: The study included 19 patients with cutaneous NH (number of skin haemangiomas ranging from 5 to >100). Mucosal involvement was observed in 32% of all cases (100% and 19% in diffuse and other cutaneous cases respectively) and hepatic involvement in 42% (67% and 38% respectively). The number of hepatic haemangiomas ranged from 1 to >10. Half of the hepatic haemangiomas cases exhibited increased hepatic arterial blood flow. CONCLUSIONS: Mucosal and hepatic involvement was frequent in cases with a high number of cutaneous haemangiomas (>100), but only frequency of mucosal involvement was statistically significant (P = 0.021).

Long-term impact of subclinical inflammation diagnosed by protocol biopsy one year after renal transplantation.

The long-term impact of subclinical acute rejection (SCAR) on renal graft function remains poorly understood. Furthermore, the interpretation of borderline lesions is difficult and their incidence is variable. The aim of this study was to analyze the characteristics of subclinical inflammation (SCI) in protocol biopsies performed 1-year after renal transplantation. SCI was defined as the presence of borderline lesions or SCAR according to the Banff 2005 classification. The patients included were a subpopulation of the CONCEPT study in which patients were randomized 3 months after transplantation to receive either sirolimus (SRL) or cyclosporine A (CsA) in combination with mycophenolate mofetil. At 1 year, we observed SCI in 37 of the 121 patients observed with an evaluable biopsy. The incidence was more frequent in the SRL group (SRL 45.2% vs. CsA 15.3%). At 30 months , SCI was associated with a significantly lower level of estimated glomerular filtration rate (mean MDRD 50.8 [±13.3] vs. 57.7 [±16.3] mL/min/1.73 m(2) , p = 0.035). In conclusion, SCI at 1-year posttransplantation is associated with worsening renal function and is more frequent in SRL-treated patients. Therefore, evaluation of SCI may be a valuable tool to allow the optimization of immunosuppressive regimens.

[Cutaneous polyarteritis nodosa and Crohn's disease: An association not to be ignored]. / Périartérite noueuse cutanée et maladie de Crohn : une association à ne pas méconnaître.

INTRODUCTION: Cutaneous polyarteritis nodosa (CPAN) is an entity which needs to be acknowledged, since it can have a spontaneously adverse outcome. We report two cases of CPAN associated with Crohn's disease. CASE REPORTS: The first patient was suffering from Crohn's disease for 9 years when she was referred for a necrotic toe. A diagnosis of necrotizing angeitis was confirmed by histological examination of a skin biopsy. Despite systemic corticosteroids, the lesions became more severe, requiring immunosuppressive treatment. The second patient was a female patient referred with forefoot ischemia. Cutaneous histology confirmed the diagnosis of necrotizing angeitis that responded favourably to corticosteroid treatment. The patient had been diagnosed with Crohn's disease 2 months previously. CONCLUSION: CPAN differed from systemic PAN by the absence of visceral involvement. Its association with Crohn's disease, although uncommon, must be recognized as it affects treatment and monitoring.

[Xanthelasma and juvenile xanthogranuloma in a 7-year-old boy]. / Xanthélasmas et xanthogranulomes juvéniles chez un garçon de sept ans.

BACKGROUND: Palpebrum xanthelasma is the most common type of xanthoma seen in adults but it is extremely rare in children. We report an original case of bilateral xanthelasma palpebrarum associated with juvenile xanthogranuloma (JXG) in a 7-year-old child. Only two cases of xanthelasma in children have been described to date. The association of xanthelasma and JXG has never been described. PATIENTS AND METHODS: A 7-year-old boy presented xanthelasmas on both eyelids. At the same time, pinkish JXG papules appeared on the child's trunk. The boy had been diagnosed at the age of 10 months with myelogenous leukaemia, which was in remission. He also had a familial history of hypercholesterolaemia. The skin lesions were removed and microscopic examination confirmed the diagnosis of xanthelasmas and JXG. DISCUSSION: This patient's presentation is unusual in several respects: the presence of xanthelasma in a child, appearance of JXG at an advanced age, and the association of these two diseases in a child with a past history of leukaemia. The occurrence of these skin lesions did not appear to be linked to the history of malignant blood disease in this patient.

Hormonal receptors in cutaneous vascular malformations: 51 cases.

Vascular malformations (VMs) are rare congenital anomalies that develop during embryogenesis in different types of vessels. Several triggering factors of cutaneous VMs include trauma, infections, or hormonal changes. We investigated the expression of hormonal receptors (androgen, estrogen, progesterone) in tissue samples of well-characterized VMs. A secondary objective was to identify self-reported triggering factors for these VMs, including hormonal changes, in the cohort of patients. We included patients with VM samples obtained in the tertiary center for vascular anomalies of the University Hospital Center of Tours, France, from January 1, 2007, to August 1, 2018. Immunohistochemistry was used to detect the expression of hormonal receptors (estrogen, progesterone, androgens). We obtained 51 samples from 51 patients: 13 cystic lymphatic malformations (CLMs), 16 venous malformations (VeMs), 11 arteriovenous malformations (AVMs), 4 combined VMs, 4 PIK3CA-related overgrowth spectrum, 1 Parkes-Weber syndrome, 1 Gorham syndrome, and 1 multiple lymphangioendotheliomatosis with thrombopenia. In total, 38 (74.5%) samples were positive for androgen receptor: 11 (84.6%) CLMs, 12 (75.0%) VeMs, 8 (72.2%) AVMs, and 7/11 (63.5%) other samples. All samples were negative for estrogen and progesterone receptors. Triggering factors were self-reported in 7 cases and were most frequently hormonal changes (n = 6, 18.2%). Hormonal triggers were frequent in AVMs (n = 4). Among patients with identified hormonal triggers, VM samples were positive for androgen receptor in 3 and negative in 3. Three-quarters of our VM samples expressed androgen receptor, and most CLM, VeM, and AVM samples were positive. Hormonal triggers were identified in 6/33 patients, mostly with AVMs.

Correction to: Hormonal receptors in cutaneous vascular malformations: 51 cases.

The original version of this article contained error. Table 2 was shown in the wrong version, thus corrected table is shown in this article.

[Rectal prolapse in a 3-year-8-month-old child]. / Prolapsus rectal chez un enfant de 3 ans 8 mois.

INTRODUCTION: Starting from a recent clinical case, we present the different causes of rectal prolapse and their specific treatments. In this case, the relation to infectious colitis was questionable. OBSERVATION: This 3-year-8-month-old boy had repetitive rectal prolapses with phlegmy and bloody diarrhea, with reduction increasingly difficult. Stool analysis was negative but pseudomembranous colitis was found with coloscopy and was confirmed by biopsy. DISCUSSION: Hygienic and dietary measures are the first steps in the treatment rectal prolapse. Pseudomembranous colitis, often related to Clostridium difficile toxins; is a severe form of postantibiotic diarrhea. Its treatment is based on oral antibiotic therapy with metronidazole or vancomycin for 10 days. Rectal prolapsus in children is cured without recurrence in 98% of cases. CONCLUSION: In the case reported herein, rectal prolapse did not recur after diarrhea recovery with antibiotic therapy, suggesting a causative link with pseudomembranous colitis.

[Infantile fibrosarcoma mimicking rapidly involuting congenital haemangioma (RICH)]. / Fibrosarcome infantile simulant un hémangiome type rapidly involuting congenital hemangioma (RICH).

BACKGROUND: While haemangioma is common, an unusual appearance or course should alert the clinician's concern. Congenital haemangioma, particularly rapidly involuting congenital haemangioma (RICH), may carry a risk of misdiagnosis as congenital malignant tumours such as infantile fibrosarcoma (also known as congenital infantile fibrosarcoma). In this case, histological diagnosis may prove inconclusive, as in the case reported herein. PATIENTS AND METHODS: At birth, a newborn baby presented angiomatous lesions on the sole of the left foot that was initially considered as congenital haemangioma. Histopathological examination suggested highly remodelled immature infantile haemangioma. After surgery, the tumour increased in size within eight weeks. Reanalysis of the histology slides resulted in a diagnosis of infantile fibrosarcoma. This diagnosis was confirmed by the presence of a specific translocation seen in infantile fibrosarcoma (ETV6/NTRK3). CONCLUSION: There is a risk of erroneous diagnosis in newborn infants between angiomatous tumour in RICH and malignant congenital tumours (particularly infantile fibrosarcoma). Clinicians should be attentive for this type of lesion and take all necessary diagnostic measures.

[Eosinophilic fasciitis associated with Borrelia burgdorferi infection]. / Fasciite à éosinophiles survenue secondairement à une infection par Borrelia burgdorferi.

BACKGROUND: Eosinophilic fasciitis (Shulman syndrome) is defined by the association of sclerodermatous skin changes involving underlying fascia and hypereosinophilia. While the aetiology is unknown, some observations suggest an infectious origin. We report the association of eosinophilic fasciitis with an infection involving Borrelia burgdorferi. PATIENTS AND METHODS: A 54 year-old man consulted for a hardened oedema and stiffness of the calves associated with an oedema of the left hand evolving for 4 months. Routine blood tests showed hypereosinophilia at 1.01 G/l and moderate inflammatory syndrome. Diagnosis of eosinophilic fasciitis was confirmed by MRI and muscle biopsy. Since the patient had reported previous tick bites some months before onset, he was tested for Lyme disease. An ELISA test revealed IgG directed against Borrelia burgdorferi and this was confirmed by Western blot analysis. DISCUSSION: The association of eosinophilic fasciitis with Lyme disease raises the question of a real link or a fortuitous association between the two conditions. Similar cases have been described in the literature with or without isolation of the spirochete from skin or fascia lesions. The incidence of eosinophilic fasciitis remains low compared to the prevalence of the infection in endemic areas. We suggest that in some patients, perhaps genetically predisposed, infection with B. burgdorferi may be at the origin of fasciitis.

[Eosinophilic esophagitis in children: Evaluation of practices through a multicenter study]. / L'œsophagite à éosinophiles chez l'enfant : évaluation des pratiques.

Eosinophilic esophagitis (EE) is a recent pathology defined by abnormal immune response of the esophageal mucosa to exogenous allergens, leading to chronic mucosa infiltration by 15 eosinophils per High-Power-Field (Eos/HPF). The present retrospective study was designed to assess the hospital care for children suffering from EE in several hospitals in western France in order to highlight discrepancies and improve future care. Twenty-eight children ranging from 1.5 months to 17 years old were included in the study. Episodes of food blockage were the most frequently reported symptoms (46 %). A ratio of 29 % of EE patients reported macroscopically normal endoscopy; diagnosis was then established upon histological anomalies found in biopsies. The mean eosinophil count was 72.4 Eos/HPF. Centralized immunohistochemical staining revealed the presence of IgG4-responding plasma cells in 76.5 % of patients, as well as IgG4 intraepithelial degranulation in 14 % of them. The evaluation of the treatment plan showed important inter-center discrepancies with only 43 % of patients receiving endoscopic reevaluation. This study objectively highlights heterogeneities in diagnosis and care provided to children suffering from EE. Therefore, improving the consistency of practices seems to be crucial to optimize the patients' outcome. The role of IgG4 as a new diagnosis marker remains to be clarified.

[High-resolution ultrasonography: utility in diagnosis, treatment, and monitoring dermatologic diseases]. / L'échographie cutanée haute résolution: utilité pour le diagnostic, le traitement et la surveillance des maladies dermatologiques.

Although less precise than histological examination, high-resolution skin imaging is a noninvasive technique that provides complementary information to the clinical examination: the lesion's US structure as well as its lateral and depth extension. Ultrasonography helps in diagnosis and initial management by measuring melanoma thickness (so as to remove this lesion with safety margins) before destructive treatment (cryosurgery or radiotherapy), by showing tumor limits, or by detecting subclinical recurrence during the follow-up. In this paper, ultrasound images will be correlated with clinical and histological data in various clinical situations such as tumors, edema, cutaneous infiltration, sclerous tissues, hyperlaxity, and cutaneous aging.

[Interferon-alpha and ribavirin treatment in a patient with hepatitis C virus-associated cutaneous periarteritis nodosa]. / Périartérite noueuse cutanée associée à une infection par le virus de l'hépatite C, régressive après une bithérapie antivirale.

BACKGROUND: Hepatitis C virus (HCV) frequently causes leucocytoclastic vasculitis as a result of type II or III cryoglobulinemia. HCV-associated vasculitis without cryoglobulinemia is less common. PATIENTS AND METHODS: A 33-year-old woman consulted for infiltrative necrotic purpura of the lower limbs, responsible for leg ulcers measuring less than 1 cm. Histopathological examination revealed vasculitis affecting the hypodermic arterioles and caused by periarteritis nodosa. No extracutaneous involvement was observed. The patient had presented asymptomatic untreated HVC infection (genotype 3) for two years. Antiviral treatment resulted in elimination of the patient's viremia and no relapse of skin lesions was observed two years after the end of treatment. COMMENTS: This patient presented vasculitis due to cutaneous nodular periarteritis associated with HVC without cryoglobulinemia. Hepatic impairment was mild and did not require any antiviral treatment. No further skin involvement was seen after treatment with colchicine and because the patient's viral genotype was favorable, we decided to initiate antiviral therapy. This therapeutic approach should be considered by dermatologists, but it is nevertheless important to assess the risk of interferon-induced aggravation of vasculitis.

[Experimental study of the influence of the segmental transverse colic reversion on the intestinal transit time]. / Etude experimentale de l'impact de l'inversion d'un segment colique transverse sur le transit intestinal.

OBJECTIVE: to study the feasibility of the transverse segment colic reversion and to evaluate its impact on the intestinal transit time in the rat Wistar. METHOD: On a test group of the rats Wistar males anaesthetized, we isolated a segment from the transverse colon vascularized by a feeder pedicle. This segment colic was reversed and anastomosed into antiperistaltic out of termino-terminal on its site with polyglactine 6/0. Into post-operative, we studied, the weight, the volume of the ingestats, the intestinal transit time, the survival and the histological lesions of the reversed segment colic. On the group controls, we carried out the anastomosis of a segment of the transverse colon isolated and anastomosed into isoperisaltic. RESULTS: Thirty rats males of 231.5 +/- 8.3g had been operated, 15 for the test group and 15 for the control group. The average weight of the ingestats of the rats of the test group was of 21.87 +/- 3g and of 21.75 +/- 4.8g in the control group without significant difference. The intestinal transit occured at 3 +/- 1 post-operatives days in the test group and at 2 postoperative days in the control group. A mechanical obstruction of the bowels before the 15th post-operative day involved a mortality of 87% in the test group. Thirteen percent of the rats of the test group survived, with a regular catch of weight. The histological analysis showed inflammatory lesions on the proximal portion of the reversed segment and a normal wall without ischaemic injury on its distal portion. No death was observed in the group controls. CONCLUSION: The antiperistaltic anastomosis of a transverse segment colic within the colic in the rat Wistar, causes a stop of the intestinal transit time by mechanical occlusion without ischaemic lesion of the segment colic reversed.

[Evaluation after 20 years of a case of Takayasu's disease that presented with aortic regurgitation]. / Evolution après 20 ans d'une maladie de Takayasu Révélée par une insuffisance aortique.

Takayasu's disease is a segmental multifocal affection of medium and large arteries. The diagnosis is based on the association of stenotic and aneurismal lesions of the aorta and its branches secondary to an inflammatory infiltration of the media and adventitia. Cases of aortic regurgitation associated with aneurismal dilatation of the ascending aorta as the presenting features of Takayasu's disease, as in this case, are rare. Histological examination of the aortic wall may help establish the diagnosis by showing signs of aortitis. The other usual arterial lesions are sometimes missing at the initial phase of the disease. A late histological diagnosis may be difficult as the inflammatory lesions tend to be progressively replaced by fibrotic lesions or a banal atheroma.

[Is procalcitonin able to help in pediatric appendicitis diagnosis?]. / La procalcitonine peut-elle aider au diagnostic des appendicites de l'enfant?

BACKGROUND: Appendicitis clinical diagnosis in children is difficult and the acute inflammatory markers hyperleucocytis and CRP do not contribute to it. OBJECTIVE: To study the predictive value of procalcitonin (PCT) and to assess variations of its serum level according to inflammatory lesions in paediatric appendicitis. PATIENTS AND METHOD: Among 101 children aged two and 15, hospitalized for painful abdominal syndromes, 70 underwent operation for acute appendicitis and appendiceal peritonitis, 31 had no operation. PCT was evaluated in all children, normal serum concentration was <0,5 microg/L. Every appendicectomy part has been examined through histological analysis to confirm the diagnosis and the histological lesions have been classified into three categories according to their severity. RESULTS: Out of 70 appendix analysed at histology, 68 (97%) were healthy and 2 (3%)were unhealthy. Among the 68 cases of healthy appendix, 19 had a PCT higher than 0,5 microg/L and among 33 cases of unhealthy appendix 4 had a PCT higher than 0,5 microg/L. The PCT mean of healthy children was not significantly different from unhealthy appendix population, appendicitis prevalence was 0,67, PCT sensitivity 28%: IC 95% [18-40], specificity 88%: IC 95% [72-97], positive predictive value 83%: IC 95% [61-95], and negative predictive value 37%: IC 95% [26-49]. PCT mean increases with the severity of inflammatory lesions of the appendix (P=0,0051). CONCLUSION: PCT has not a good predictive value in acute paediatric appendicitis but remains a good indicator of severity in paediatric appendicitis.

[Pellagra and panniculitis induced by chronic bacterial colonisation of the small intestine]. / Colonisation bactérienne chronique de l'intestin grêle révélée par un érythème pellagroïde et une panniculite.

INTRODUCTION: Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. CASE REPORT: A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. DISCUSSION: We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.

Detection of acute cardiac allograft rejection with high resolution electrocardiography: experimental study in rats.

BACKGROUND AND METHODS: To diagnose early acute cardiac rejection, we evaluated high-resolution electrocardiography in rats. Heterotopic heart transplantations were performed in allogeneic animals, either treated with cyclosporine or untreated, and in syngeneic animals. High-amplification electrocardiograms were recorded daily, under anesthesia, with two intra-abdominal leads. After amplification (x 5000 to 20,000), the electrocardiographic signal was acquired and analyzed with P-Clamp software. We measured the amplitude (millivolts) and duration (milliseconds) of the auriculogram (P wave) and the ventriculogram (QRS wave), the duration of auriculoventricular conduction (milliseconds; PQ interval) and the heart rate. Twenty-five grafted hearts were fully studied in recipients not treated with cyclosporine (allogeneic n = 16, syngeneic n = 9). RESULTS: In the allogeneic group, acute cardiac rejection was always accompanied by an early and progressive increase in P wave duration and PQ interval, whereas an increase in QRS duration was subsequently recorded. No significant change in P wave, PQ interval, or QRS wave duration was recorded in the syngeneic group, which showed no histologic rejection lesions. A decrease in P wave and QRS wave amplitude was recorded in both groups of animals. In the allogeneic group treated with cyclosporine (n = 21), grafted hearts were removed early (4.5 +/- 0.5 days): 10 cardiac grafts were rejected and 11 were not. An increase in P wave duration > or = 20% was associated with mild rejection in most cases. The sensitivity and specificity of this electrocardiographic sign were excellent (100%). The auricular (right and left atria) and the ventricular (right and left ventricles) tissues were evaluated histologically. In the allogeneic groups (n = 26), the histologic lesions during acute rejection were greater in the auricular myocardium than in the ventricular myocardium. Rejection in the atrial and ventricular myocardium was most often differentiated by one degree according to the Billingham classification. CONCLUSIONS: We concluded that acute cardiac rejection in rats is associated with early conduction disturbances in the atrial myocardium which can be shown by high-resolution electrocardiography.