Pilomatrix carcinoma of the face. Two case reports

Pilomatrixial carcinoma is the malignant variety of pilomatrixoma. It is a malignant lesion, locally aggressive that can reappear, specially if not completely removed. Scanty are the number of cases listed in the literature (2). Although metastases are uncommon one or twice references can be found in the writings (1). We report 2 cases of pilomatrixial carcinoma located in the middle canthal and malar zones, which were treated surgically: excision of the tumor and reconstruction by means of a local rotation flap.     

Gómez–López-Hernández syndrome: reappraisal of the diagnostic criteria

Gómez–López-Hernández syndrome (GLHS) is a rare and possibly underdiagnosed condition. So far, 21 patients have been reported and all of them were sporadic observations. We report six additional patients. The hallmark triad of GLHS, also named cerebellotrigeminal dermal dysplasia, consists of rhombencephalosynapsis, trigeminal anesthesia (often giving rise to corneal opacities), and bilateral parietal or parieto-occipital alopecia. Our patients had rhombencephalosynapsis and alopecia, but none had trigeminal dysfunction. In this respect, the term cerebellotrigeminal dermal dysplasia is potentially misleading. In conclusion, only rhombencephalosynapsis and alopecia are consistently present in GLHS and are required diagnostic criteria, while trigeminal anesthesia, dysmorphic features, and ataxia are inconsistent findings. A high index of suspicion is required to diagnose GLHS, particularly as alopecia tends to be hidden by surrounding scalp hair.     

Dermoid cyst of the floor of the mouth. A case report

This is the case of a young woman, 16-year-old, calling to our Department because of the appearance, in the floor of the mouth, of a mass progressively growing until about 5 cm diameter. Through fine needle biopsy was diagnosed of epidermoid cyst. Despite its size and infrahyoid site was removed successfully. Perusal review of last years bibliography and its variants.     

Dyskinesia as a new adverse effect of hormonal treatment in West syndrome

West syndrome is an age‐dependent epileptic encephalopathy. Despite potential side effects, hormonal therapy remains the main treatment for West syndrome. Here, we report on 10 patients receiving steroid treatment who presented with unusual, mostly hyperkinetic, movements. Facial grimacing, repetitive mouth opening, adduction and abduction of upper and lower extremities, and periodical strabismus in different combinations were observed in all patients, independent of formulation, dose, duration, and efficacy of treatment. Symptoms disappeared in sleep and reappeared immediately on arousal. Dyskinesias stopped gradually after a month of discontinuation of treatment. Repeated EEGs did not show corresponding epileptiform activity. We conclude that these abnormal movements can be attributed to side effects of hormonal treatment. [Published with video sequences]     

Infective stomatitis of the palate

Having on base a wide and selective review of the literature, it has seemed to us interesting to detail the infective pathology on this location or sometimes occurring. This region is, as well will see, the seat of a rich pathology. We pretend on the other hand to reassess the importance of the meticulous examination of the oral cavity in "systemic problems". We describe the palatal infections divided in four groups: bacterial, viral, fungal and parasitory, according to the causal agent. We review the palatine manifestations of other fewer current syndromes as well.