[Atypical purpuric oedema of the nose during granulomatosis with polyangiitis]. / Un œdème purpurique du nez atypique au cours d'une granulomatose avec polyangéite.

INTRODUCTION: Granulomatosis with polyangeitis or Wegener's disease is a necrotizing vasculitis of small and medium vessels associated with antineutrophil cytoplasmic autoantibodies (ANCA). The most frequent sites are lung, ear, nose and throat and kidney. PATIENTS AND METHODS: We report the case of a 47-year-old woman presenting purpuric oedematous plaque with bullous detachment of the nose and hospitalised for the assessment of two suspicious neoplastic lung lesions discovered as a result of a recent stroke and repeated seromucosal otitis. Granulomatosis with polyangeitis was suspected because of multiple systemic lesions. The histopathology of skin lesions and laboratory investigation results were consistent with this diagnosis. A favourable outcome was achieved with corticosteroids and rituximab. DISCUSSION: The diagnosis of GPA is based on criteria established by the American College of Rheumatology. The cutaneous clinical aspect described in our case confirms the polymorphism of the cutaneous lesions possibly associated with this disease. They are rarely isolated but, in some cases, allow early diagnosis with improved prognosis, which remains severe in the absence of treatment.

[Idiopathic granulomatous mastitis associated with erythema nodosum]. / Mastite granulomateuse idiopathique associée à un érythème noueux.

BACKGROUND: Idiopathic granulomatous mastitis (IGM) is a rare, chronic, granulomatous, inflammatory disorder with potentially misleading clinical and radiological features. IGM is diagnosed after exclusion of infectious or tumoral diseases. Herein we report a case of erythema nodosum associated with IGM, which serves as a reminder that erythema nodosum may constitute an extra-mammary sign of IGM. PATIENTS AND METHODS: A 36-year-old nulliparous woman presented with fever and skin rash at our dermatology clinic at the North Franche-Comté hospital in July 2017. Symptoms had occurred 2 days previously. The patient had a fever of 38.8°C and typical erythema nodosum on the limbs. Physical examination showed an irregular, inflammatory, abscessed mass measuring 20cm with multiple shrinkage zones that had been developing for 4 weeks and for which breast examinations were being performed. Laboratory tests showed an inflammatory state (CRP 155mg/mL, WBC 14.6×109/L), other tests (serum electrolytes and calcium, hepatic and renal tests, Streptotest, T-spot, HIV, HBV, HCV serology, anti-streptolysin, anti-streptodornase, local microbiology samples, antinuclear antibodies, soluble antigens antibodies, hemocultures, angiotensin-converting-enzyme and chest x-rays) were normal. Microbiology investigations were negative. Mammography revealed invasive mastitis. Breast sample biopsies showed giant cell granulomas without caseous necrosis or tumor cells, and histochemical staining (PAS, Ziehl, Grocott, Gram) was negative. The final diagnosis was of IGM associated with erythema nodosum. Symptoms rapidly improved with oral steroids. DISCUSSION: As reported herein, erythema nodosum may be associated with IGM and support the diagnosis thereof. This association is rare, with fewer than 30 case reports described in the literature. IGM is an uncommon benign disorder of the breast that can mimic two frequent breast disorders: breast carcinoma and breast abscess. It usually occurs in young sexually active women. It appears as a tumor with an inflammatory solid painful mass, with nipple and skin retraction, occasionally with abscesses and accompanied by homolateral axillary lymphadenopathies. Radiologic findings are usually not specific for or suggestive of cancer. The histopathological picture of IGM is characterized by the presence of multinucleated giant cells and epithelioid histiocytes forming non-caseating granulomas around lobules. Minor ductal and periductal inflammation is usually present. IGM is rarely associated with autoimmune system manifestations, especially erythema nodosum, arthritis, episcleritis or hidradenitis suppurativa. Although the physiopathology of IGM remains unclear, this case serves as additional evidence that the etiology of IGM is of autoimmune origin. While there is no recommendation for the treatment of IGM, oral steroids remain the cornerstone of therapy. CONCLUSION: We report a case of IGM associated with erythema nodosum. Dermatologists must be aware of this association.

[Multiple granular cell tumours in a patient with Noonan's syndrome and juvenile myelomonocytic leukaemia]. / Tumeurs à cellules granuleuses multiples chez un enfant atteint d'un syndrome de Noonan compliqué de leucémie myélomonocytaire juvénile.

BACKGROUND: Granular cell tumour (GCT) is a rare form of tumour comprising Schwann cells. Herein, we report a case of a child presenting Noonan syndrome complicated by juvenile myelomonocytic leukaemia (JMML) and who also developed a multiple form of GCT. We discussed the molecular mechanisms that might account for this association. PATIENTS AND METHODS: A six-year-old boy with Noonan syndrome complicated by JMML presented three asymptomatic subcutaneous nodules on his back, forearm and neck. Histological analysis revealed GCT. A literature review revealed seven cases of Noonan syndrome presenting GCT, none of which were associated with JMML. Mutation of gene PTPN11, via hyperactivation of intracellular Ras signalling may cause the development of GCT and JMML in children presenting Noonan syndrome. DISCUSSION: Detailed clinical examination is recommended in children presenting GCT to screen for multiple forms and for signs of malformation suggestive of a genetic syndrome. Ours is the first case to be described of Noonan syndrome complicated by JMML associated with multiple GCT. This association once again raises the important question of the role of the Ras-MAPK signalling pathway in the development of benign and malignant tumours of solid organs or blood, associated with genetic syndromes.

[Comparative evaluation of dermoscopy and capillaroscopy in Raynaud's phenomenon]. / Évaluation comparative de la dermatoscopie et de la capillaroscopie dans le phénomène de Raynaud.

OBJECTIVE: Raynaud's phenomenon (RP) is a common cause for consultation. Capillaroscopy is a well-established technique to detect capillary abnormalities suggestive of a connective tissue disease, but it is sometimes unavailable. The aim of this study was to compare dermoscopy and capillaroscopy in the assessment of RP. METHODS: This was a prospective single-centre observational study in adult patients consulting for RP at the Hôpital Nord Franche-Comté between January 2014 and June 2015. Dermoscopy was performed at dermatological consultations and capillaroscopy was prescribed. For each capillaroscopy and dermoscopy, the following parameters were examined: normal appearance, giant capillaries, avascular areas, dystrophic capillaries or tortuosity and haemorrhages. Kappa coefficients were calculated. RESULTS: Twenty-six patients participated in this study. The kappa coefficient was 0.76 for "normal" status, 0.78 for tortuosity, 0.70 for giant capillaries, 0.48 for haemorrhage and 0.62 for avascular areas. The global kappa coefficient was 0.33. Detection of these abnormalities with capillaroscopy was significantly associated with abnormal dermoscopic status (P<0.05). The sensitivity of dermoscopy for the detection of "abnormal" capillaroscopic status was 0.87. CONCLUSION: The correlation coefficients were good. Despite poor global concordance, 80% of patients had the same status, normal or abnormal, for both capillaroscopy and dermoscopy, which resulted in the same clinical management. Dermoscopy is thus a valuable tool screening for periungual anomalies and provides support for clinical examination by the dermatologist, although the reference method continues to be capillaroscopy.

Incidence of amyloidosis over 3 years: the AMYPRO study.

OBJECTIVES: There is a lack of epidemiological information concerning amyloidosis, particularly in France. We started a 3-year prospective study (AMPYRO) to analyze the epidemiological features of amyloidosis in the eastern part of France. METHODS: From 2003 to 2005, all patients with a tissue sample showing amyloid deposits, were included in this study. Immunohistochemistry using anti-P component, anti-SAA, anti-light chains immunoglobulins and anti-transthyretin was applied for each tissue sample. For each patient, past and present medical histories along with biological features were recorded. RESULTS: Seventy-six patients with amyloid were identified over 3 years. The age-standardized incidence rate of amyloidosis was estimated at 14 cases per million person-years. The final entire population included in the AMYPRO study was composed of 66 patients with a mean age of 71.7+/-11.5 years old. The amyloid typing after clinical, biological and immunohistochemistry revealed senile amyloid in 40 cases (60.6%), AL amyloid in 13 (19.7%) and AA amyloid in 9 (13.6%). Neither clinical nor biological features differed significantly between the transthyretin-positive and transthyretin-negative populations. CONCLUSION: Regarding only tissue samples, senile amyloid was the most prominent amyloid type identified. Therefore, the clinician needs to be aware that in most of the amyloid cases identified on the pathologic examination there is no need for additional examination unless there are clinical or biological signs of a primary or secondary amyloidosis.

[Surgical treatment of Verneuil's disease (hidradenitis suppurativa): 15 cases]. / Traitement chirurgical de la maladie de Verneuil: 15 cas.

INTRODUCTION: Verneuil's disease (hidradenitis suppurativa) is a chronic inflammatory, suppurating and fistulizing disease of apocrine sweat gland-bearing skin. The aim of this study was to describe the surgical treatment, conducted in 15 patients suffering from this disease. PATIENTS AND METHODS: We retrospectively analyzed 15 observations (9 men, 6 women, mean age 38.6). The mean delay between beginning of the symptoms and diagnosis was 55.5 months. Surgery was conducted at the stage of abscesses, fistulization and keloids in all patients. The first surgical step was wide and deep excision of affected skin and subcutaneous fat. The second step was secondary intention healing, or ideal suture, Z plasty or dorsalis major flap. RESULTS: Only four patients had complications: two axillary strictures, one anal margin stenosis and one hypertrophic scarring. Three relapses occurred, treated by excision under local anesthesia. CONCLUSION: Surgical treatment seems to be the only definitive treatment of Verneuil's disease.

[Nail manifestations of systemic diseases]. / Manifestations unguéales des maladies générales.

General diseases-associated nail disorders may be classified into 5 types according to their clinical impact. Type I refers to nail disorders considered as peculiar features among the various symptoms of general disease such as peri-ungueal vascular dilatation, onycholysis, Beau lines, opaque nails or melanonychia. Type II refers to nail disorders with a strong clinical value such as Terry's nails, Muerhrcke's lines or koilonychia. Type III refers to nails disorders presenting as significant syndromes of several diseases such as yellow-nail syndrome of subungueal splinter haemorrhages, and type IV to specific disorders considered as true keys for diagnosis such as digital hippocratism, Bazex's acrokeratosis, Koenen's tumor, or nail hypoplasia. In type V are included drug-induced nails disorders especially after chemiotherapy or antiretroviral therapy.

[Prevalence of hepatitis C virus in oral erosive lichen]. / Prévalence du virus de l'hépatite C dans le lichen érosif buccal.

OBJECTIVE: An increased prevalence of hepatitis C virus (HCV)-related hepatitis has been reported in patients with lichen planus. The aim of our retrospective study was to determine whether there is a relationship between the erosive form of oral lichen planus and HCV. PATIENTS AND METHODS: A retrospective study was conducted in a consecutive sample of 28 patients suffering from oral erosive lichen planus and seen in our department between 1992 and 1996. Anti-HCV antibodies were detected in all cases by third generation recombinant immunoblot assay. PCR was performed to detect HCV RNA in the serum sample from 19 patients. RESULTS: Eight patients (29 p. 100) tested positive for HCV antibodies. These results were compared with the rate of HCV chronic carriers which is between 1 and 2 p. 100 in our region (Southern France). Among these 8 patients, 7 had severe chronic liver disease, and 5 of them were actively replicating the virus. DISCUSSION: Our results suggest a strong relationship between erosive oral lichen planus and HCV-related chronic hepatitis. We conclude that patients with the erosive form of oral lichen planus should be systematically evaluated for HCV infection.

Eosinophilic pustular folliculitis in infancy: report of two affected brothers.

We describe two brothers with eosinophilic pustular folliculitis. Both had recurrent crops of papules and pustules, primarily affecting the scalp. The eruption began in the neonatal period. Both children had a leucocytosis with eosinophilia. Histology revealed folliculitis, with an infiltrate in which eosinophils were predominant. Bacteriological and fungal cultures of pustules were negative. There was a good clinical response to treatment with a topical corticosteroid and dapsone. We review the 28 previously reported cases of eosinophilic pustular folliculitis in infancy. The occurrence of this disorder in brothers suggests that an inherited or contagious factor may be involved in its aetiology.

[Septicemic cutaneous localization of cryptococcosis following renal transplantation in a female]. / Localisations cutanées septicémiques d'une cryptococcose chez une greffée rénale.

Cryptococcosis is a systemic disease caused by the yeast-like fungus Cryptococcus neoformans. Cutaneous manifestations, although rare, may precede other organ involvements. A case of cutaneous cryptococcosis is described herein. The skin lesions in our patient (a 47-year old female with renal transplant) consisted of acute cellulitis over her left and right thighs, revealing a septicemic infection. The patient received immunosuppressive drugs (prednisone, azathioprine). Clinical, mycological and histopathological data are described in details. Amphotericin B and fluconazole were initially successful, but a relapse with Cryptococcus meningitis required the adjunction of itraconazole to treatment. This case report underlines the insufficient effectiveness of fluconazole and the success of itraconazole.