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Localized cicatricial pemphigoid of the Brunsting-Perry type with transition into disseminated cicatricial pemphigoid. Report of a case proved by preembedding immunogold electron microscopy.

BACKGROUND: In 1979, Provost described two patients with the clinical features of disseminated cicatricial pemphigoid for the first time. Until now, only four additional cases of disseminated cicatricial pemphigoid have been described. Existence of diagnosis of disseminated cicatricial pemphigoid has been discussed controversially because in four cases investigated by electron microscopy the blister formation was found below the lamina densa, which is indicative of an epidermolysis bullosa acquisita. OBSERVATION: A 78-year-old woman is presented with a generalized eruption of blisters leaving behind scars that developed after a 7-year-long history of mild circumscribed recurrent blisters and scarring eruptions that had been diagnosed previously as Brunsting-Perry type of cicatricial pemphigoid. Immunofluorescence antigen mapping disclosed the blister formation above the lamina densa. Electron and immunoelectron microscopy using a preembedding immunogold technique revealed blister formation and antibody binding within the lamina lucida, predominantly below the subbasal dense plate. CONCLUSIONS: The clinical features of disseminated blistering followed by scarring, the immunofluorescence antigen mapping, and the electron and immunoelectron microscopic findings in our case for the first time clearly prove the existence of a disseminated cicatricial pemphigoid.

Aged↗

Aminolevulinic acid hydrochloride with photodynamic therapy: efficacy outcomes and recurrence 4 years after treatment.

The safety and efficacy of treating individuals who presented with multiple actinic keratosis (AK) lesions with 5-aminolevulinic acid (ALA) in combination with photodynamic therapy (PDT) were documented in a phase III trial. This report highlights results of this phase III trial and reviews 4 specific cases of sustained AK lesion clearance 4 years after treatment with ALA/PDT Long-term recurrence data were collected from patients who participated in clinical trials of ALA/PDT Long-term evaluation extended to 36 to 48 months (4 years) supports primary efficacy findings of the phase III pivotal trial, with a low incidence of AK recurrence in patients treated with ALA/PDT

Administration, Cutaneous↗

Detection of high-risk human papillomavirus type 16/18 in cutaneous warts in immunocompetent patients, using polymerase chain reaction.

Cutaneous warts are caused by human papillomavirus (HPV). Prevalence studies of the types of HPV present in cutaneous warts have been carried out more frequently in immunosuppressed patients. The present study was designed to study the association of high-risk HPV in cutaneous warts of immunocompetent patients. A total of 45 cases of cutaneous warts from various sites in immunocompetent subjects were analyzed for HPV. Samples included both archival material i.e., paraffin embedded and fresh tissue. Highly sensitive and comprehensive polymerase chain reaction (PCR) methodology for detection of HPV of high oncogenic potential, HPV 16/18, was employed. Human papillomavirus 16 was detected in 3 (6.6%) patients. None of the lesions demonstrated HPV 18. None of the cutaneous warts demonstrated histopathological features associated with dysplasia or neoplasia. The identification of HPV 16 in cutaneous warts, which are benign proliferations of the skin, further expands the spectrum of HPV-linked lesions. It remains of critical interest to determine whether these types are specifically associated with the development of malignant lesions analogous to those seen in anogenital cancer.

Abdominal Wall↗

Head and neck atopic dermatitis and malassezia-furfur-specific IgE antibodies.

BACKGROUND: Atopic dermatitis of the head and neck (HNAD) has been recognized as a separate entity. Malassezia furfur, a lipophilic yeast, is considered to be a pathogenic allergen in this form of atopic dermatitis. OBJECTIVE: The purpose of this study was to determine the level of IgE anti-M.-furfur antibodies and their relation to the severity of the disease. METHODS: IgE anti-M.-furfur antibodies were assayed in 106 patients with HNAD. Controls included 25 patients with non-HNAD, 20 with nonatopic dermatitis and 16 with seborrheic dermatitis (including 4 with AIDS). RESULTS: There was a highly significant correlation between the level of anti-M.-furfur IgE and clinical severity. Furthermore, there was a significant but smaller correlation between total IgE and clinical severity. In patients with HNAD, total IgE was higher amongst men. CONCLUSION: IgE anti-M.-furfur antibodies are a good and specific marker for HNAD. IgE M. furfur levels are strongly correlated with the severity of the disease.

Adolescent↗

[Actinic keratoses].

Actinic keratoses are defined as proliferation of cytologically atypical keratinocytes in the zone of epidermal-dermal junction in photodamaged skin. In the northern hemisphere the prevalence of actinic keratoses ranges depending on different epidemiological studies from 11% to 25% for people aged 40 or older. The main cause of actinic keratoses is exposure to UVB radiation in sunlight UVB radiation induces mutations in the telomerase gene and in the tumor suppressor gene P53, which can also be detected in invasive squamous cell carcinoma. The only histological parameter to distinguish between actinic keratoses and SCC is the level of invasiveness. The risk for actinic keratoses to develop into SCC is about 16% over lo years. For this reason and because of the high prevalence of actinic keratoses, it has been suggested to replace the term,, actinic keratosis K with intraepidermal squamous cell carcinoma' to better characterize the lesion. In the following review recent aspects of pathogenesis and therapy of actinic keratoses are discussed.

Adult↗

Netherton's syndrome: an electronmicroscopic study.

An ultrastructural study of 2 patients with Netherton's syndrome (bamboo hair, scaling dermatosis, and an atopic diathesis) showed features of psoriasis and a dermatitis. Although the biopsies from the patients showed histological and ultrastructural similarities, the clinical presentations were different. One patient had ichthyosis linearis circumflexa, while the other had a generalized ichthyosiform eruption. The ultrastructural findings in the 2 patients, while not specific, may help distinguish the eruption of Netherton's syndrome from other scaling dermatoses.

Adult↗

Treatment of severe cutaneous lupus erythematosus with a chimeric CD4 monoclonal antibody, cM-T412.

BACKGROUND: Monoclonal CD4 antibodies are among the most potent immunomodulatory agents in various experimental models of autoimmune disease, including murine lupus erythematosus. OBJECTIVE: The aim of this study was to evaluate the toxicity and therapeutic efficacy of a chimeric monoclonal CD4 antibody, cM-T412, in patients with cutaneous lupus erythematosus (LE). METHODS: Five patients with severe cutaneous LE lesions received intravenously a total of 275, 400, or 475 mg of cM-T412 in single doses of 20 to 50 mg during a period of 5 to 8 weeks. RESULTS: CD4 antibody treatment induced a long-lasting decrease in disease activity. It resulted in healing of LE skin lesions, a reconstituted responsiveness to conventional treatment, or both. Despite a substantial depletion of circulating CD4+ T lymphocytes, no clinical signs of immunosuppression were noted. CONCLUSION: Monoclonal CD4 antibodies should be considered as a novel treatment for the management of severe cutaneous LE.

Adjuvants, Immunologic↗

[Plicate pachyderma of the head].

The authors describe a combination of two rare hereditary dermatoses, wrinkled pachyderma of the head (wrinkled skin of the head) and neurofibromatosis. The triad of clinical symptoms: wrinkled skin of the head, acromegaly, and drumstick symptom permit the diagnosis of pachydermoperiostosis (Touraine-Solente-Golé's syndrome).

Adult↗

Skin diseases of children in Mali: a public health problem.

In order to estimate the importance as a public health problem of skin diseases, we investigated the prevalence and severity of skin diseases in a representative sample of children in Mali. 1817 children were randomly selected in 30 clusters by probability-proportional-to-size sampling in Koulikoro region. The mean prevalence (+/- 2 SD) of skin diseases was 34 +/- 4%. The most frequent dermatoses were pyoderma (12.3 +/- 1.6%), tinea capitis (9.5 +/- 2.5%), pediculosis capitis (4.7 +/- 1.4%), scabies (4.3 +/- 1.5%), and molluscum contagiosum (3.6 +/- 1%). The most troublesome dermatoses were scabies and severe pyoderma. Pyoderma was the only dermatosis associated with poor individual or household hygiene. Public health services were little used by the population for skin diseases, probably because of the lack of an adequate response by the services and the high cost of treatment. The high prevalence and the severity of many of the lesions, and the discomfort caused, make pyoderma and scabies a significant public health problem in Mali.

Child↗

Sarcoidosis.

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Adult↗

Woolly hair nevus. Two case reports and a discussion of unruly hair forms.

Woolly hair is very curly hair that is characteristically unruly and will not form naturally into locks. Woolly hair nevus first appears when the scalp hair begins to grow. It is seen as a patch of unruly, very curly hair, arising in a field of otherwise unremarkable scalp hair. Woolly hair can also be seen as a hereditary trait involving the entire scalp and in acquired forms. It may be distinguished from two other types of unruly hair, pili torti and spun glass hair.

Adult↗