[Anatomoclinical and dermatoscopic study of trichoadenoma]. / Trichoadénome : description anatomoclinique et dermatoscopique.

INTRODUCTION: Trichoadenoma is a very rare follicular tumour with a remarkable histopathological appearance. In this article we present a series of 12 cases of trichoadenoma, as well as the anatomoclinical and dermatoscopic findings in a typical case. We discuss these findings in the light of an extensive literature research. PATIENTS AND METHODS: We collated 12 cases of trichoadenoma of indisputable diagnosis made at the dermatopathology laboratory of the Dermatological Clinic of the University Hospitals of Strasbourg over a 30-year period (1989-2018). RESULTS: The 12 cases comprised 7 women and 5 men, of average age 58.9 years, the majority having lesions on the cephalic extremity followed by the buttocks and thighs. Histopathological examination, which was similar in all 12 cases, showed multiple epidermal cysts containing an eosinophil lamellar keratin with a stratified wall without any visible hair, located in the superficial and mid dermis and appearing to be stacked on top of one another. In immunohistochemistry, broad spectrum keratin markers were still positive, the follicular marker Ber-EP4 weakly expressed and PHLDA1 was negative. For the case examined using polarized-light dermatoscopy, small rounded white-yellow areas were observed corresponding to cystic structures surrounded by irregular linear vessels. DISCUSSION: Trichoadenoma is a rare tumour seen in middle-aged adults of mean age 45 years, and has no sexual predominance. It is asymptomatic, slow-growing, variable in colour, measures less than one centimeter and is most often located on the face or buttocks. In terms of histology, the juxtaposition of multiple small cystic structures suggests a follicular origin. Differential diagnosis is made with trichoblastomas, which always intensely express PHLDA1 and/or Ber-EP4, desmoplastic trichoepithelioma, which consists of multiple much thinner spans in a highly fibrous stroma with clearly visible arborescent vessels over a white-yellow ivory background at dermatoscopy, microcystic carcinoma, which has a deeper extension, and plaque milium, in which the cysts are larger.

[Nested melanoma]. / Mélanome en thèques.

BACKGROUND: Nested melanoma in elderly subjects is an entity that has been reported in the literature only since 2012. In this paper, we describe its distinctive clinical, dermatoscopic and histopathological features and compare them to previous published cases, with the aim of highlighting certain specific criteria of this melanoma subtype. CASE REPORT: A 52-year-old man was referred for the presence on his chest of a large suspicious pigmented lesion of irregular shape and colour. Dermatoscopically, the lesion was chaotic and characterized by a black, structureless, eccentric area with some peripheral globules as well as some segmental radial lines. Histopathological examination revealed the presence of an asymmetric lesion with large junctional melanocytic nests showing a focal tendency to gathering and some cytological atypia. A diagnosis of nested melanoma was ultimately made. DISCUSSION: Nested melanoma of the elderly represents a distinct anatomoclinical variant of superficial spreading melanoma. Clinically, the lesion is usually large and occurs in photodamaged skin. We would stress that the "elderly" criterion is not mandatory given the numerous cases reported in people under 60 years. The main dermatoscopic feature is a globular pattern, but several features characteristic of superficial spreading melanoma may also be present. Histological diagnosis may be difficult because of the mainly nested pattern, and the condition may be confused histologically with a benign junctional nevus. But these large junctional nests of different sizes, with bridging and cytonuclear atypias, together with asymmetry of the lesions are the hallmark of this special kind of melanoma.

[Multiple familial "eruptive" dermatofibromas]. / Dermatofibromes multiples « éruptifs ¼ familiaux.

BACKGROUND: Multiple eruptive dermatofibromas (DF) are rare and frequently associated with immune and neoplastic diseases. There have also been reports of rare familial cases. Herein we report a new such case. PATIENTS AND METHODS: A 79-year-old woman and her 37-year-old daughter were seen for disseminated DF over a period of several decades, from adolescence onwards. Neither had any history of diseases or treatments normally associated with multiple DF. History-taking revealed similar lesions in other family members. DISCUSSION: DF are common benign cutaneous tumours, generally seen on the lower limbs of young or middle-aged women. These lesions occur either in isolation or are relatively few. Multiple or so-called eruptive DF, defined by the presence of more than 15 lesions in a single patient, is rare and is associated in 60% of cases with autoimmune diseases, HIV infection, neoplastic disease or immunosuppressant therapy. Familial forms such as those described herein are extremely rare.

[Maxillary sinus osteoma associated with a mucocele]. / Ostéome du sinus maxillaire associé à une mucocèle.

INTRODUCTION: Maxillary sinus osteomas are rare benign tumours with a poorly documented clinical evolution. Their craniofacial localization may be part of a syndrome. We report a case of maxillary sinus osteoma associated to a mucocele and a cyst probably of dental origin, with no sign of associated Gardner syndrome. CASE REPORT: A 52-year-old woman consulted for chronic maxillary sinusitis and an oral vestibular fistula. The CT-scan suggested a mucocele with reactive bone formation. Macroscopically, a pediculated bone tumour was found next to a mucocele, and to a cyst probably of dental origin. The anatomopathological examination led to a diagnosis of cancellous osteoma. DISCUSSION: Several hypotheses have been made on the etiology of sinus osteomas. When identified, screening for Gardner's syndrome should be implemented because of the associated risk for colic malignancy.

[Modified functional genioplasty]. / Génioplastie en tenon-mortaise modifiée.

Functional genioplasty is indicated to correct excessive lower anterior facial height. Michelet's surgical technique is commonly used for these indications because of improvement of the stability of the fixation of the bone. The authors present a modified genioplasty of Michelet's technique with preservation of suprahyoid muscles' insertions.

Hepatic microcirculation in the perfused cirrhotic rat liver.

Liver microcirculation in the perfused rat liver was assessed by the multiple indicator dilution technique. Comparative studies were carried out in noncirrhotic rats and in rats with cirrhosis secondary to chronic exposure to phenobarbital and carbon tetrachloride. The alterations of the sinusoidal bed were characterized by changes in the displacement of hepatic venous outflow curves of various diffusible substances (labeled albumin, sucrose, and water) relative to that of labeled erythrocytes (vascular reference). Outflow recoveries of lidocaine (a substance that penetrates the liver cell membrane freely and completely) and of labeled microspheres (15 microns diam) were also appraised. In all cirrhotic rats, unimodal erythrocytes and albumin curves were obtained. The sinusoidal space was significantly decreased when compared with normal rats (P less than 0.001) and the total space accessible to albumin became progressively restricted. In seven cirrhotic rats, the profiles of labeled sucrose and water curves were compatible with a flow-limited diffusion and the total distribution volumes were not significantly different from values found in noncirrhotic rats (P = NS), which indicates that sucrose and water were still able to diffuse into an extravascular space not accessible to albumin. In the other cirrhotic rats, labeled sucrose and water curves showed progressive bimodal changes not compatible with a flow-limited diffusion. Such alterations were not due to large intrahepatic shunts, since only 0.25% of the 15-microns microspheres were recovered in the outflow of cirrhotic rats. However, an early lidocaine outflow peak related in time to the peak erythrocyte curve was observed in cirrhotic, but not in noncirrhotic, rats. Lidocaine recovery varied greatly in cirrhotic rats and appeared to increase as the liver disease progressed. These data can be explained by capillarization of sinusoids and/or by the development of channels with poor permeability. Electron microscopic observations of these rat livers favored the latter. Thus, in cirrhotic rat liver, two kinds of alteration are likely: (a) the vascular space is decreased with collagenization of the extravascular space, limiting the diffusion of large molecules such as albumin; and (b) small channels with poorly permeable walls develop, limiting the diffusion of small molecules such as lidocaine, sucrose, and water. Large intrahepatic shunts are not a common feature.

Assessment of liver microcirculation in human cirrhosis.

Alterations in the liver microcirculation were characterized by use of the multiple-indicator dilution technique in 25 cirrhotic patients undergoing hemodynamic evaluation of portal hypertension. Hepatic vein outflow dilution curves were obtained after portal vein or hepatic artery injections of a vascular reference substance (labeled erythrocytes) and of diffusible substances (labeled albumin and sucrose). In 23 of these patients (19 with alcoholic cirrhosis and 4 with postnecrotic cirrhosis), unimodal erythrocytes and albumin curves were obtained; the immediately accessible albumin space ranged from normal values (that were substantially larger than the erythrocyte space) to low values (that were little larger than the erythrocyte space). In parallel with this, the hepatic extraction of indocyanine green decreased and was correlated with the albumin space (r = 0.821, P less than 0.001). The form of labeled sucrose curves showed progressive changes indicating limited diffusion into the interstitial space. In contrast, bimodal curves were found in two patients (with macronodular cirrhosis); a large proportion of all labels appeared simultaneously in the early part of the outflow curves. Model analysis of the unimodal data indicated that the spectrum of findings could best be explained by progressive development of a barrier to exchange by progressive capillarization of the microvascular bed, and the form of the bimodal data suggested that large vessel shunting was occurring. Both changes, in turn, will contribute to the reduced extraction of protein-bound materials in cirrhosis.

[A rare cause of oral pain: The pterygoid hamulus syndrome]. / Une cause rare de douleur orofaciale: le syndrome de l'hamulus ptérygoïdien.

INTRODUCTION: Pterygoid hamulus syndrome (PHS) is a rare cause of orofacial and oropharyngeal pain. PHS can be associated with a hamulus hypertrophy or with a bursitis of the palatosalpingeus but it has not always an anatomic cause. OBSERVATION: A 36-year-old woman was seen for a constant posterior palatal pain spreading towards oropharynx, increasing during swallowing and lasting for more than 6 months. Physical examination showed an erythema of the soft palate, medially to the hamulus. Hamulus palpation was painful and revealed hamulus hypertrophia on both sides. A bilateral PHS was evocated. DISCUSSION: This observation is typical of a PHS. We propose a review of the literature of this little-known syndrome. Treatment is initially conservative (corticosteroids) but surgery can be proposed in case of morphological anomalies of the hamulus.

Hepatic handling of vitamin D3 in micronodular cirrhosis: a structure-function study in the rat.

The response to vitamin D3 (D3) was studied in a model of micronodular cirrhosis induced by CCl4. The uptake and C-25 hydroxylation of D3 were then studied in isolated-perfused liver preparations. CCl4-treated rats had a significantly lower fractional hepatic D3 uptake than controls; they also had lower 25-hydroxyvitamin D3 (25(OH)D3) concentrations in both liver and perfusate following 150 min of perfusion. CCl4 induced a wide spectrum of hepatic morphologic changes ranging from mild to large collagen infiltration, but micronodular cirrhosis was present in more than 90% of the animals. Histomorphometric analysis of the liver indicated an overall highly significant increase in the volume density (Vv) of collagen infiltration, and a reduction in the Vv normal hepatocytes following CCl4. Linear relationships were also observed between the Vv normal hepatocytes and the liver, perfusate, and total 25(OH)D3, while the 25(OH)D3 production decreased in a logarithmic fashion as the collagen infiltration of the liver parenchyma increased. These data show that the overall production of 25(OH)D3 is decreased in micronodular cirrhosis; they also indicate, however, that the D3-25 hydroxylase seems to stay unimpaired in the remaining hepatocytes of the diseased liver, and that the Vv normal hepatocytes constitute one of the major determinants of the 25(OH)D3 production by the cirrhotic rat liver.

Influence of Ca2+ concentration on the clearance and circulating levels of intact and carboxy-terminal iPTH in pentobarbital-anesthetized dogs.

The role of hormone secretion and hormone clearance in the differential control of circulating levels of intact (I-) and carboxy-terminal (C-) immunoreactive parathyroid hormone (iPTH) was evaluated in 18 pentobarbital-anesthetized dogs. Catheters were installed in the aorta, left renal, and hepatic veins for sampling. Hepatic and renal blood flows were calculated from sulfobromophtalein (BSP) and p-aminohippuric acid (PAH) extraction and clearance. I- and C-iPTH were measured during a 1 h of infusion of CaCl2 or Na2EDTA. High-performance liquid chromatography (HPLC) profiles of I- and C-iPTH in and out of the liver and kidney were also obtained. Data on two dogs (one CaCl2 and one Na2EDTA infusion) were pooled for the analysis of one parathyroid function using a four-parameter mathematical model. Results obtained in the basal state and during analysis of the parathyroid function were also compared with those of 24 awakened dogs. Results are means +/- SD. Anesthetized dogs had lower levels of Ca2+ (1.29 +/- 0.03 vs. 1.34 +/- 0.04 mmol/l; p < 0.001) and higher levels of I- (11.5 +/- 5.7 vs. 3.0 +/- 1.9 pmol/l, p < 0.001) and C-iPTH (52 +/- 20.9 vs. 22.8 +/- 10.5 pmol/l; p < 0.001) than awakened dogs. Their stimulated (S) and nonsuppressible (NS) I-iPTH levels were increased 2- and 4-fold, respectively, while similar C-iPTH levels rose only 1.35- and 1.75-fold; this caused their S (4.4 +/- 0.7 vs. 6.8 +/- 1.9; p < 0.001) and NS (24.6 +/- 11.8 vs. 49.8 +/- 27.5; p < 0.05) C-iPTH/I-iPTH ratios to decrease. This was not explained by different renal clearance rates of I- and C-iPTH since both were similar at approximately 10 ml/kg/minute and unaffected by Ca2+ concentration. Clearance of all I- and C-iPTH HPLC molecular forms by the kidney appeared equal. A 50% decrease in the hepatic clearance of I-iPTH to approximately 12 ml/kg/minute in pentobarbital-anesthetized dogs, related to a lower hepatic blood flow, explained the higher levels of S and NS I-iPTH in these animals. I-iPTH hepatic clearance was unaffected by Ca2+ concentration. C-iPTH hepatic clearance was much lower at approximately 5 ml/kg/minute, abolished by hypercalcemia, and reduced by the influence of anesthesia on hepatic blood flow. This also explained the higher S C-iPTH levels in anesthetized animals. I-PTH(1-84) detected by the C-iPTH assay explained only 37.6% of the hepatic C-iPTH clearance in hypocalcemia and 73.3% in hypercalcemia. Overall, our results indicate that total C-iPTH clearance is about 40.2% that of I-iPTH in hypocalcemia and 41.3% in hypercalcemia. This would only explain a 2.4- to 2.5-fold difference in circulating levels of I- and C-iPTH if secretion rates were equal; the larger difference observed in S and NS C-iPTH/I-iPTH ratio values is thus mainly explained by different production rates.