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Giant nevus lipomatosus superficialis with multiple folliculosebaceous cystic hamartomas and dermoid cysts.

Nevus lipomatosus superficialis (NLS) is a rare benign condition characterized by papules and nodules usually in the pelvic and sacral areas, with ectopic mature adipose tissue reaching the superficial dermis. We report the case of a 47-year-old female with large NLS in the sacral region that had been present since birth and, after an asymptomatic course for most of the time, became associated with episodes of mild pain that prompted the patient to look for medical help. After a complete resection of the cerebriform plaque, measuring 23 cm in diameter, histopathological examination revealed typical NLS architecture in as yet undescribed association with multiple folliculosebaceous cystic hamartomas (FCHs) and dermoid cysts.

Dermoid Cyst↗

Naevus lipomatosus cutaneous superficialis on the nose.

Naevus lipomatosus cutaneous superficialis (NLCS) is an uncommon hamartomatous lesion with an exceptional presentation on the face. We report the case of an elderly patient who presented with a classic type of NLCS on the right nasal orifice. This patient was taking inhaled steroids for his allergic rhinitis during 35 years.

Administration, Inhalation↗

The treatment of multiple lipomas by liposuction surgery.

When lipomas reach 4 cm or more in size or are multiple, liposuction becomes an excellent alternative to excisional surgery, which is the treatment of choice for small lipomas. Two cases of multiple lipomas treated by liposuction surgery are presented.

Aged↗

Inhibitory motor seizures: correlation with centroparietal structural and functional abnormalities.

Six adults and 2 children with focal inhibitory motor seizures (ictal paralysis) were evaluated during a 4-year period. Paresthesias at seizure onset occurred during some seizures in all patients, and focal clonic activity followed paralysis in 4. EEG-CCTV recordings of the seizures in 2 patients showed that ictal paralysis coincided with an ictal discharge starting in one centroparietal area. MRI showed centroparietal structural lesions in six patients. One patient with a normal MRI scan had right centroparietal hypometabolism on PET. Inhibitory motor seizures must be differentiated from transient ischemic attacks and migraine. In our patients a centroparietal epileptogenic focus was suggested by neuroimaging studies, and in 2 instances by ictal EEG.

Adolescent↗

Lipomatous infiltration of the canine salivary gland.

Benign connective tumours of the canine salivary glands are rare. This report describes lipomatous infiltration of parotid or submandibular salivary glands in seven dogs in which the glands were enlarged as a result of infiltration by fat cells; they appeared to have been successfully treated by local excision. The precise cause of the lipomatous infiltration in the dogs is unclear but different causes of similar lesions in humans are discussed.

Animals↗

The Proteus syndrome: the Elephant Man diagnosed.

Sir Frederick Treves first showed Joseph Merrick, the famous Elephant Man, to the Pathological Society of London in 1884. A diagnosis of neurofibromatosis was suggested in 1909 and was widely accepted. There is no evidence, however, of café au lait spots or histological proof of neurofibromas. It is also clear that Joseph Merrick's manifestations were much more bizarre than those commonly seen in neurofibromatosis. Evidence indicates that Merrick suffered from the Proteus syndrome and had the following features compatible with this diagnosis: macrocephaly; hyperostosis of the skull; hypertrophy of long bones; and thickened skin and subcutaneous tissues, particularly of the hands and feet, including plantar hyperplasia, lipomas, and other unspecified subcutaneous masses.

Abnormalities, Multiple↗

Classification of benign fatty tumours of the upper limb.

In this paper, the authors offer a classification of benign fatty tumours of the upper limb. There are three histologically distinct types of fat cells: immature fat cells which give rise to lipoblastomas, mature brown fat cells which give rise to hibernomas and mature white fat cells which give rise to lipomas. Lipomas are the most common and they are sub-classified according to the anatomic site of fat cells into dermal, subcutaneous and sub-fascial lipomas; or tumours directly related to muscle, bone, synovium or nerve. Finally, the authors review 67 patients with benign fatty tumours of the upper limb and provide clinical examples of these tumours including their characteristic histological and radiological features.

Adolescent↗