Proliferating pilomatricoma

We report on 9 patients with pilomatricomas that showed unusual histopathologic features. Our patients were mainly elderly individuals (age range 42 to 88 years; mean age 70.1 years) who presented solitary cutaneous nodules situated on the head and neck (7 neoplasms), upper arm (1 neoplasm), and back (1 neoplasm). All the lesions were treated by simple excision. Follow-up data available in 7 of the 9 patients (mean follow-up, 17 months) revealed local recurrences in 1 patient whose lesion recurred 3 times. No lymph node involvement or distant metastases were recorded in any of our cases. Histopathologically, most neoplasms were characterized by a relatively large lesion in the clermis that in some cases showed extension to the subcutis. Each lesion was predominantly composed of a lobular proliferation of basaloid cells in association with adjacent focal areas containing eosinophilic, cornified material with shadow cells. In some cases, relatively large areas of shadow cells were present, whereas, in others only small foci of shadows cells were observed. Cytomorphologically, the basaloid cells showed features of matrical and supramatrical cells of a normal hair follicle and exhibited variable nuclear atypia and mitotic figures. The overall architectural pattern of the neoplasms was different from that of large fully developed stereotypical pilomatricomas that maintain a cystic character with basaloid cells predominantly aligned at the periphery. Based on the histopathologic findings, namely the presence of a large, lobular proliferation of basaloid cells in association with small to large foci of shadow cells, we interpreted these neoplasms to be a distinctive proliferative variant of pilomatricoma and propose the designation "proliferating pilomatricoma." Proliferating pilomatricomas should be differentiated from the recently described matricoma, basal-cell carcinoma with matrical differentiation, and matrical carcinoma (pilomatrical carcinoma).     

Life-threatening respiratory failure due to cranial dystonia after dental procedure in a patient with multiple system atrophy.

We report on a woman with a an 8-year history of multiple system atrophy with predominance of parkinsonism who developed jaw-locking oromandibular dystonia within hours after insertion of ill-fitting dentures. Dystonia spread rapidly to involve other facial muscles and the larynx causing stridor with respiratory failure necessitating crush intubation.     

Predicting Skull Loading: Applying Multibody Dynamics Analysis to a Macaque Skull

Evaluating stress and strain fields in anatomical structures is a way to test hypotheses that relate specific features of facial and skeletal morphology to mechanical loading. Engineering techniques such as finite element analysis are now commonly used to calculate stress and strain fields, but if we are to fully accept these methods we must be confident that the applied loading regimens are reasonable. Multibody dynamics analysis (MDA) is a relatively new three dimensional computer modeling technique that can be used to apply varying muscle forces to predict joint and bite forces during static and dynamic motions. The method ensures that equilibrium of the structure is maintained at all times, even for complex statically indeterminate problems, eliminating nonphysiological constraint conditions often seen with other approaches. This study describes the novel use of MDA to investigate the influence of different muscle representations on a macaque skull model (Macaca fascicularis), where muscle groups were represented by either a single, multiple, or wrapped muscle fibers. The impact of varying muscle representation on stress fields was assessed through additional finite element simulations. The MDA models highlighted that muscle forces varied with gape and that forces within individual muscle groups also varied; for example, the anterior strands of the superficial masseter were loaded to a greater extent than the posterior strands. The direction of the muscle force was altered when temporalis muscle wrapping was modeled, and was coupled with compressive contact forces applied to the frontal, parietal and temporal bones of the cranium during biting. Anat Rec, 291:491–501, 2008. © 2008 Wiley‐Liss, Inc.     

Role of neoadjuvant chemotherapy in congenital intracranial haemangiopericytoma

Infantile haemangiopericytoma of the CNS is a rare entity. We report the first case of a congenital haemangiopericytoma successfully treated by preoperative chemotherapy. The patient presented shortly after birth with the diagnosis of a haemangiopericytoma. As neurosurgery was too risky due to size, location and age of the patient an anthracycline‐based chemotherapeutic regimen was applied and resulted in a significant decrease in tumour size, making a postchemotherapy complete surgical resection possible. Chemotherapy may benefit patients with congenital haemangiopericytoma especially if the tumour cannot initially be treated by complete neurosurgical resection. Pediatr Blood Cancer. 2010;56:161–163. © 2010 Wiley‐Liss, Inc.     

Structure–function relations of primate lower incisors: a study of the deformation of Macaca mulatta dentition using electronic speckle pattern interferometry (ESPI)

Teeth adopt a variety of different morphologies, each of which is presumably optimized for performing specific functions during feeding. It is generally agreed that the enamel cap is a crucial element in controlling the mechanical behavior of mammalian teeth under load. Incisors are particularly interesting in terms of structure–function relations, as their role in feeding is that of the ‘first bite’. However, little is known how incisor cap morphology is related to tooth deformation. In the present paper we examine the mechanical behavior of mandibular central incisors in the cercopithecine primate Macaca mulatta under loads similar to those encountered during ingestion. We map three‐dimensional displacements on the labial surface of the crown as it is compressed, using electronic speckle pattern interferometry (ESPI), an optical metrology method. In addition, micro‐computed tomography is used to obtain data regarding the morphology of the enamel cap, which in the M. mulatta lower incisors exhibits missing or very little enamel on the lingual face. The results showed that although compressed along a longitudinal axis, deformation in the incisors mostly occurred in the lingual direction and orthogonal to the direction of the applied load. Both isolated, embedded teeth and teeth in the mandible showed considerable lingual deformation. Incisor deformation in the mandible was generally greater, reflecting the additional freedom of movement enabled by the supporting structures. We show that the association with adjacent teeth in the arch is significant for the behavior of the tooth under load. Finally, loading two teeth simultaneously in the mandible showed that they work as one functional unit. We suggest that these results demonstrate the importance of enamel cap morphology in directing deformation behavior; an ability stemming from the stiffness of the enamel cap overlying the more pliable dentin.     

Malassezia yeasts activate the NLRP3 inflammasome in antigen‐presenting cells via Syk‐kinase signalling

Although being a normal part of the skin flora, yeasts of the genus Malassezia are associated with several common dermatologic conditions including pityriasis versicolour, seborrhoeic dermatitis (SD), folliculitis, atopic eczema/dermatitis (AE/AD) and dandruff. While Malassezia spp. are aetiological agents of pityriasis versicolour, a causal role of Malassezia spp. in AE/AD and SD remains to be established. Previous reports have shown that fungi such as Candida albicans and Aspergillus fumigatus are able to efficiently activate the NLRP3 inflammasome leading to robust secretion of the pro‐inflammatory cytokine IL‐1β. To date, innate immune responses to Malassezia spp. are not well characterized. Here, we show that different Malassezia species could induce NLRP3 inflammasome activation and subsequent IL‐1β secretion in human antigen‐presenting cells. In contrast, keratinocytes were not able to secrete IL‐1β when exposed to Malassezia spp. Moreover, we demonstrate that IL‐1β secretion in antigen‐presenting cells was dependent on Syk‐kinase signalling. Our results identify Malassezia spp. as potential strong inducers of pro‐inflammatory responses when taken up by antigen‐presenting cells and identify C‐type lectin receptors and the NLRP3 inflammasome as crucial actors in this process.