Isolated Protein C Deficiency in a Newborn.

BACKGROUND: Congenital protein C deficiency is a rare hereditary thrombophilia, neonatal purpura fulminans is the most serious form of this deficit. The purpose of this observation is two-fold. The first is the need to make an early diagnosis in order to improve the prognosis. The second, is to discuss the need. In case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, in the newborn and in both parents. METHODS: The diagnosis is biological and is based on the quantitative determination of functionally active protein C. We use the Berichrom® Protein C assay on an automated coagulation analyzer from Siemens Healthcare Diagnostics, which allows the chromogenic determination of Protein C activity. RESULTS: We report an observation of cutaneous necrosis in a newborn having developed a purpura fulminans extensive secondary to a total congenital protein C deficiency. In front of this clinical picture, thrombophilia assessment is requested, revealing an isolated deficit in protein C < 1%. CONCLUSIONS: In the case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, is essential in the newborn and in both parents.

Outcomes of newborns to mothers with COVID-19.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) has spread rapidly across the world. Given the sharply increased infection rate, the number of pregnant women and children with COVID-19 is correspondingly on the rise. SARS-CoV-2 infection is transmitted through droplets; though hypothesized, other transmission routes have not been confirmed. As of now, it remains unclear whether and how SARS-CoV-2 can possibly be transmitted from the mother to the fetus. METHOD: This study examines the medical records of 30 neonates born to women with COVID-19, the objective being to provide documented information on maternal-child transmission and infant outcomes. RESULTS: Out of the 30 newborns, 28 had negative PCR test results for SARS-CoV-2; among their mothers, fifteen had fever, nine had cough and twenty had delivered by cesarean section. The median birth term was 37wk2dy, and twenty of the neonates were male. Most of them were asymptomatic, except for the three who presented with shortness of breath. Two of them were intubated and both died, the first because of severe sepsis and the second due to severe hyaline membrane disease. As regards the two infected neonates, the first represents a probable case of congenital SARS-CoV-2 infection, which appears unlikely in the second case. The outcome for both of them was good, without any complications. CONCLUSION: Maternal-fetal transmission of the SARS- CoV-2 virus was not detected in the majority of the reported cases, although two of 30 neonates had positive qRT-PCR test results. Our study supports the hypothesis that though it seldom actually occurs, in utero SARS-CoV-2 vertical transmission is possible.

[Neonatal adrenal hematoma: various modes of presentation]. / Hématome surrénalien néonatal : diversité des modes de révélation.

Neonatal adrenal hematoma is a rare condition, most frequently caused by trauma. We report three cases of adrenal hematoma admitted to the Neonatology and Neonatal Intensive Care Unit in the A. Harouchi Children's Hospital, the Ibn Rushd University Hospital in Casablanca, Morocco, over a 2-year period from January 2011 to December 2012. The average age of these patients was 5 days. The clinical presentations were diverse; the most common manifestations were intense jaundice in one case, acute adrenal insufficiency in one case, and severe anemia in the other case. Abdominal ultrasonography was used to confirm the diagnosis and monitor adrenal hemorrhage in all the patients. Analysis of clinical, laboratory, and ultrasonography data showed a favorable prognosis in all the patients. Based on these observations, we discuss the risk factors, clinical presentations, progression and management of neonatal adrenal hemorrhage.

[Gastric pneumatosis complicated by a neonatal digestive occlusion]. / Pneumatose gastrique compliquée d'une occlusion digestive néonatale.

Gastric pneumatosis is a rare pathology. Its occurrence in the neonatal period requires looking for ulcerative-necrotizing enterocolitis in a context of prematurity or an underlying surgical obstacle. We report a case of gastric pneumatosis at a newborn child born at term, admitted on the third day of life for neonatal occlusion with a flat stomach. The abdomen without preparation showed substantial gastric distension with aspects of gastric pneumatosis and embellishes with images showing a double gastric bubble. The surgical exploration showed gastric pneumatosis, complete duodenal atresia located at the level of the second duodenal portion, and an annular pancreas. Progression was favorable after duodenostomy.

Retraction notice to "Nipple adenoma in a man: An unusual case report" [Int. J. Surg. Case Rep. 3 (2012) 190-192].

[This retracts the article DOI: 10.1016/j.ijscr.2011.05.008.].

Nipple adenoma in a man: An unusual case report.

Adenomatosis (adenoma, papillary adenoma, florid papillomatosis) of the nipple is a rare benign disorder involving the nipple witch can be mistaken clinically for Paget's disease and pathologically can be misinterpreted as an adenocarcinoma. It occurs mostly in middle-aged women and is extremely rare in adolescents and children. We report a 55-year-old man with an adenomatosis of the nipple evolving positively after surgery.

[Generalized bullous pemphigoid induced by radiotherapy]. / Pemphigoïde bulleuse généralisée induite par une radiothérapie.

BACKGROUND: There have been a very small number of reported cases of radiotherapy-induced autoimmune bullous disease. We describe a case of generalised autoimmune sub-epidermal bullous pemphigoid (BP) induced by radiotherapy in a female patient presenting squamous cell carcinoma of the vulva. CASE REPORT: In June 2008, a 48-year-old woman underwent vulvectomy with lymph node curettage for squamous cell carcinoma of the vulva. Following surgery, adjuvant radiotherapy was indicated. At the 16th session (dose of 32 Gy), erythema occurred on the irradiation field. At the 23rd session (46 Gy), the patient presented bullous lesions that became generalised after four days with involvement of the oral mucosa. Skin biopsy revealed sub-epidermal bullae and direct immunofluorescence showed continuous linear deposits of IgG and of C3 along the dermal-epidermal junction. Indirect immunofluorescence revealed the presence of antibodies directed against the basement membrane. A favourable outcome was achieved under systemic corticosteroids. No immunotransfer analysis of serum or any other immunological investigations were performed. DISCUSSION: The clinical and histological features of this autoimmune bullous disease were evocative of BP, despite the absence of any formal proof. Radio-induced BP is extremely rare, with only 28 cases being described to date in the literature. The trigger mechanism is poorly understood; radiotherapy appears to cause changes in the antigenic properties of proteins in the dermal-epidermal junction resulting in production of autoantibodies. The radio-induced nature of BP does not affect therapeutic response. The case we report is novel in terms of both generalisation of the eruption and mucosal involvement.

[Henna symbolic tattoo and real dermatitis]. / Du henné d'un tatouage symbolique à une vraie dermatose.

INTRODUCTION: Henna, or Lawsonia inermis, has been used since antiquity by women in the Orient for dyeing the hair, hands, and feet. Contact dermatitis to pure henna is very rare, most often caused by additives such as perfume oils or paraphenylenediamine (PPD). We report the case of a girl who presented contact dermatitis to henna associated with eczema to draw attention to the dangers related to its use. CASE REPORT: A 12-year-old girl developed erythematovesicular and edematous lesions with very itchy burning, suggestive of contact dermatitis, 48 hours after application of black henna. Lesions were located at the tattooing site exactly following the original design. The patient also had eczema lesions on the left cheek after contact with the tattooed hand. The lesions were improved by treatment with level II corticosteroids. DISCUSSION: Today, henna has become very popular in Western countries. PPD is added to reduce the fixation time or to obtain a darker color. It can cause severe systemic reactions. The most common allergic reaction is contact dermatitis. Treatment is based on topical steroids. Better legislation on temporary tattooing practices and control preparations as well as regular annual information aimed at the general public are essential. CONCLUSION: This observation raises awareness of the importance of information on the serious risks of a labile tattoo, most particularly for the young.

[Extra-cutaneous systemic manifestations in generalized pustular psoriasis]. / Manifestations systémiques extracutanées du psoriasis pustuleux généralisé.

Generalized pustular psoriasis of the von Zumbusch type is a severe form of psoriasis characterized by disseminated pustular skin lesions with high fever and hyperleukocytosis. We report a 32-year-old woman who presented a generalized pustular psoriasis with extra-cutaneous manifestations of the disease that included pulmonary involvement, aseptic arthritis, jaundice, and liver abnormalities. The extra-cutaneous manifestations of generalized pustular psoriasis should be known to physicians caring for patients with psoriasis in order to avoid diagnostic delay.

[Treatment and prevention of acute radiation dermatitis]. / Traitement et prévention des radiodermites aiguës.

Acute radiation dermatitis is a common side-effect of radiotherapy which often necessitates interruption of the therapy. Currently, there is no general consensus about its prevention or about the treatment of choice. The goal of this work was to focus on optimal methods to prevent and manage acute skin reactions related to radiation therapy and to determine if there are specific topical or oral agents for the prevention of this acute skin reaction. The prevention and the early treatment are the two focus points of the management of the acute radiation dermatitis.

[Fixed food eruption caused by liquorice]. / Erythème pigmenté fixe induit par de la réglisse.

INTRODUCTION: Fixed drug eruption is a characteristic cutaneous adverse reaction that recurs at fixed localisations. It is due to medical drugs. However, food may sometimes be responsible for a similar eruption, known as "fixed food eruption". We report a case of fixed food eruption due to liquorice. CASE REPORT: A 22-year-old woman presented with liquorice-induced fixed drug reaction. Topical challenges remained negative both at previously affected sites and in unaffected skin. Therapeutic re-exposure to liquorice confirmed the diagnosis. DISCUSSION: To our knowledge, we report the first case of food drug reaction caused by liquorice.

[Congenital bronchobiliary fistula: a case report]. / Fistule bronchobiliaire congénitale : à propos d'une observation.

Congenital bronchobiliary fistulas (CBBF) are rare developmental anomalies. We report a case of a 6-day-old newborn who presented with respiratory distress and bilioptysis, originally involving a meconium aspiration syndrome. The diagnosis was confirmed by bronchoscopy. Surgery was performed at the age of 26 days and the newborn died the following day. Operative opacification showed communication between the carina and the biliary system. We review the clinical characteristics of CBBF and the value of early diagnosis and surgical treatment to prevent pulmonary complications due to bile salts.

[Orbital location of Kaposi's sarcoma]. / A propos d'une localisation orbitaire du sarcome de Kaposi.

Kaposi's sarcoma is a proliferative disease, probably induced by human herpes virus type 8 (HHV8). Its expression is cutaneous and visceral, with four clinical forms. An orbital location of Kaposi's sarcoma remains exceptional. In order to evaluate the epidemiological, clinical, and therapeutic objectives of this tumor location, we report a case of a 58-year-old patient hospitalized in the ophthalmology department for a surgically treated right orbital tumor.

[Chronic alveolar syndrome]. / Syndrome de condensation alvéolaire chronique.

We report a case of primary pulmonary Hodgkin's disease in a 20 year-old woman. The chest x-ray showed a chronic alveolar syndrome. The diagnosis was established from a pulmonary biopsy. The radiological features and the options for diagnosis of primary pulmonary Hodgkin's disease are discussed.

Cerebellar liponeurocytoma (lipomatous medulloblastoma).

Liponeurocytoma (lipomatous medulloblastoma) is an uncommon clinicopathologic entity. We report a case of cerebellar liponeurocytoma in an adult and attempt to better characterize this uncommon lesion. A 43-year-old woman presented with symptoms and signs of increased intracranial pressure and cerebellar dysfunction. CT and MRI showed a heterogeneous well-circumscribed mass in the left cerebellar hemisphere with hydrocephalus. Complete surgical excision of the tumor was accomplished through a suboccipital craniotomy. Pathological examination with immunohistochemical study revealed a medulloblastoma with neuronal, astrocytic and lipomatous differentiation. Postoperative radiation therapy was given. After surgery, the patient was followed up for a 16 month-period; symptoms improved dramatically and no evidence of tumor recurrence was found. Careful analysis of the histopathological and immunohistochemical studies correctly identifies liponeurocytoma (lipomatous medulloblastoma). To our knowledge, only 16 cases have been reported. Although this lesion appears to have a better prognosis than the conventional form of adult medulloblastoma, complete surgical removal with postoperative radiation therapy is recommended.

[Histoid leprosy with erythema nodosum leprosum]. / Lèpre histoïde avec érythème noueux lépreux.

Histoid leprosy is a particular variant of lepromatous leprosy presenting as cutaneous or subcutaneous nodular and/or plaque-like lesions arising form apparently normal skin. It is characterized histologically by spindle-shaped histiocytes in interlacing bundles and whorls, containing numerous intact and rod-shaped Mycobacterium leprae. It can occur de novo or secondary in patients treated for a long course by dapsone alone. We describe a case of lepromatous leprosy treated according to the national Moroccan protocol who developed histoid lesions during his treatment by dapsone. The patient responded well to fluoroquinolone, rifampicin and clofazimine, with however, the occurrence of erythema nodosum leprosum.

[Multiocular renal cysts in adults. Two case reports]. / Les kystes multiloculaires du rein chez l'adulte. A propos de deux observations.

The authors report two cases of renal multilocular cyst and review the cases reported in the literature, revealing the original features of this benign tumour which has an equal incidence in children and adults with a controversial acquired or malformative aetiopathogenesis, precise histological features, but a difficult preoperative and intraoperative diagnosis despite modern imaging techniques. In the great majority of cases, treatment consists in nephrectomy.