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Hereditary hypotrichosis simplex of the scalp is a rare trait with onset in early childhood. This phenomenon has been reported only once previously, in a Spanish kindred. This communication describes a case in a Jewish-Yemenite kindred with 51 affected individuals and confirms autosomal dominant inheritance.
In this report, we describe the presence of a large, posterior scalp defect as another, hitherto nonreported symptom related to a defect in midline development in a 1-year-old boy with Opitz syndrome.
In this report we describe a male patient with the interesting combination of a large congenital scalp defect, distal limb reduction anomalies, right spastic hemiplegia and hypoplasia of the left arteria cerebri media. Follow-up data from birth up to the age of 18 years revealed positive evolution with low to normal intelligence. The findings in the present patient, i.e. an Adams-Oliver syndrome associated with a severe neurological deficit, are best explained as resulting from interruption of the early embryonic blood supply.
The association of scalp defect, unusual ears and absence of nipples was described by Finlay & Marks as an autosomal dominant trait. We report a new case in a 23-year-old woman. Renal insufficiency and cataract seem to be frequent and must be investigated.
A case of bradycardia during the insertion and inflation of tissue expanders under the scalp on two separate occasions is described. The possible mechanisms of this complication are discussed.
A matrical carcinoma (pilomatrix carcinoma) of the scalp is described--the first reported case in this site. This malignant hair follicle tumour must be distinguished histologically from benign pilomatricoma and proliferating trichilemmal tumour, which can have similar features.
The natural history of congenital inclusion dermoid cysts of the scalp is unknown. We report three cases of this condition, which has been followed from birth for up to ten years. Dermoids are congenital subcutaneous cysts, which gradually flatten and scar over a number of years. The lesions are covered with skin showing focal alopecia but they may be surrounded by a collar of hypertropic hair. Later in life flattened cysts may be mistaken for aplasia cutis, but the history of a cyst at birth allows the clinician to distinguish these two conditions. As these cysts have a potential for intracranial extension, this is an important clinical entity.
A female infant with ectodermal dysplasia, bilateral cleft lip and palate and a recalcitrant scalp dermatitis is presented. She had features of both Rapp-Hodgkin syndrome and AEC syndrome. It has recently been suggested in the literature that these two syndrome are the same condition and this case report supports this viewpoint.
We present a case of cicatricial pemphigoid with significant scarring alopecia as a major manifestation and review our series of cicatricial pemphigoid patients in Oxford (UK). Only four of 54 patients had scarring alopecia of the scalp. While rare, this manifestation poses a difficult management problem; patients find this cosmetically distressing and there are no successful treatments available to date.
BACKGROUND: The authors evaluated the usefulness of scalp-recorded ictal electroencephalograms (EEG) in diagnosing the epileptogenic area in epilepsy with complex partial seizures. METHODS: The authors analyzed the ictal EEG of 395 seizures in 43 patients with complex partial seizures. Based on EEG findings the patients were classified according to the degree of localization of their onset areas. The results were then compared with neuroimaging findings. RESULTS: Only 10 patients fell into the category 'discrete', meaning that all the onset areas (as measured by ictal EEG) were localized in the same lobe of the same hemisphere. Seven patients were categorized as 'lateralized', meaning that all the onset areas were clearly lateralized in the same hemisphere but without consistent localization. Eleven patients were classified as 'localized', meaning that the onset area were localized simultaneously in bilateral same lobes, or changed consistently from one lobe in one hemisphere to the same lobe in the opposite hemisphere. The onset area could not be defined in 15 patients and these were categorized as 'not defined'. No patient who underwent seven or more ictal recordings was categorized as discrete. However, when confined only to those patients in whom over 75% of the ictal recordings showed the same onset area, there was a high correlation between the epileptogenic lesions detected by ictal EEG and those detected by neuroimaging techniques. CONCLUSIONS: The findings of the present study indicate that ictal EEG recordings are useful for determining the epileptogenic area in epilepsy with complex partial seizures, provided that more than 75% of the ictal recordings show the same ictal onset area.
BACKGROUND: Seborrheic dermatitis is a common inflammatory skin disorder affecting 1-3% of the population. It is thought to be linked to dandruff via a common etiology, yeasts of the genus Malassezia. Ciclopirox is a broad-spectrum, hydroxypyridone-derived, synthetic antifungal agent with anti-inflammatory properties. METHODS: A total of 203 patients were enrolled in this vehicle-controlled, double-blind, randomized study designed to compare vehicle with three different concentrations of ciclopirox shampoo: 0.1%, 0.3% and 1%, with each applied twice a week. The main efficacy parameters were based on 6-point ordinal scales describing the disease's signs and symptoms (scaling, inflammation and itching), global status of disease, and global change in disease. RESULTS: A tendency towards improvement of the sum score from baseline was found in all ciclopirox treatment groups. The most pronounced improvement was found in the ciclopirox 1% group, which changed from a baseline sum score of 8.3 to 4.4 at the end of the 4-week study period (P-value vs. vehicle 0.0372). In addition, the therapeutic index showed increasing efficacy with the use of increased concentrations of ciclopirox. CONCLUSIONS: The study supports the use of 1% ciclopirox shampoo in the treatment of seborrheic dermatitis of the scalp. Furthermore, ciclopirox shampoo at each concentration was found to be safe and well tolerated.
An 86-year-old man presented with a painful reddish tumour on the scalp with a 3-month history, mental confusion with recent onset and lymphadenopathies. Histological examination of the lymph node and cutaneous lesion revealed a dense infiltrate of atypical and large B cells. There was no evidence of bone marrow invasion. According to REAL (Revised European-American Classification of Lymphoid Neoplasms), this lymphoma was considered as a diffuse large B-cell lymphoma with concurrent cutaneous and nodal involvement. Cerebral computerized tomography (CT) scan showed bone and dura mater invasion in the right parieto-occipital region with collapse of lateral ventricle. The patient was submitted to systemic chemotherapy with cyclophosphamide, vincristine and prednisolone (CVP). There was a good response with regression of the cutaneous lesion, but the patient died after the third cycle. We point out the unusual clinical presentation and aggressive behaviour of this lymphoma.
We present a case of an intriguing mesenchymal neoplasm of the scalp that recurred several times over 10 years before a final diagnosis was possible. The case was sent for expert opinions to various international dermatopathological authorities and was, for a long time, unanimously interpreted as malignant melanoma. This diagnosis was supported by immunohistochemical examinations demonstrating S-100 positivity. Nevertheless, the clinical behaviour, as well as some histopathological features raised doubt regarding the diagnosis. Only after the last recurrence, followed by a repeat extensive immunohistochemical study, the diagnosis of epithelioid angiosarcoma was made. Histologically malignant melanoma can be highly misleading and in literature, reports of misinterpreted cases of melanoma are published. In contrast, tumours that can simulate melanoma are also not infrequent and it is essential to perform immunohistochemistry to confirm diagnosis and exclude a melanocytic lesion.
We report the case of a 3-year-old boy with naevus comedonicus, characterized by confluent clusters of dilated follicular orifices plugged with keratinous material that resemble open comedones, located on the scalp.
The amplitude of the late pain-related negative-positive peak complex, which we have labeled SP3 (134-150 ms) and SP6 (277-331 ms), respectively, increased with increasing interstimulus interval (ISI). This contrasts with the nociceptive spinal withdrawal reflex and subjective pain rating data, which implied that nociceptive somatosensory processes were unaffected by ISI at stimulus levels that were well within the pain range. A scalp topographic analysis strongly suggested that none of the brain areas responsible for SP3 or SP6 are involved exclusively in nociception. We also observed a pain-related positive potential approximately 161-177 ms following sural nerve stimulation that has not been reported by others. A dipole source localization analysis and the effects of ISI and stimulus intensity on this potential suggest that it is generated by the response of primary somatosensory cortex neurons to inputs arising from the innocuous peripheral afferents and that this response is inhibited by noxious inputs.
Normal values for fetal scalp tissue pH during labour were determined by using a glass electrode (Kontron-Roche). A total of 132 recordings was obtained and 84 (64 per cent) were of good quality. After a good quality recording had been obtained, there were 64 infants with an Apgar score of 9 or 10 at one and five minutes after delivery. The 64 recordings showed pH values (Mean +/- 2SEM) of 7.38 +/- 0.12 six hours before delivery and 7.28 +/- 0.12 at the time of delivery. Between the 5th and 55th minutes of the second stage of labour the pH (Mean +/- 2SEM) fell from 7.30 +/- 0.14 to 7.20 +/- 0.13.
Hypoxanthine, xanthine, inosine, urate and uridine, were measured in 149 samples of umbilical cord plasma using high pressure liquid chromatography. In spite of a good correlation with the simpler oxygen consumption method for measuring hypoxanthine, there was no clear discrimination between hypoxic and well oxygenated infants, although mean concentrations were higher in infants with well defined criteria of intrapartum hypoxia or bith asphyxia, there was overlap with the normal range. Fetal scalp blood samples were also found to be clinically unhelpful in the diagnosis of intrapartum hypoxia, at least in part due to variable degrees of haemolysis in the specimens. There were poor correlations between hypoxanthine concentrations and those of hydrogen ion, base deficit and lactate. Uridine concentrations were significantly higher in arterial cord blood than in venous cord blood but hypoxanthine or xanthine concentrations did not show this difference.
The study aim was to identify the time from a decision to perform a fetal blood sample to the result of the test being available. A total of 100 consecutive fetal scalp blood samples taken on women in labour were identified from the blood gas analysers on the delivery suite. Eighty-nine percent of attempts yielded a result. The median time taken was 18 minutes (interquartile range 12-25 minutes). In 9% of women, the result took longer than 30 minutes. This is important clinically when repeated testing is required or in the second stage when operative vaginal delivery is achievable. Furthermore, when retrospectively analysing cases with a poor outcome, the time to obtain a result needs to be taken into account when determining the time at which a baby could have been delivered.