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[The skin manifestations of congenital and acquired hemostatic disorders].

The authors review the literature data on skin symptoms of the most prevalent disorders induced by congenital or acquired defects of blood coagulation. The range of skin changes in hemostasis disorders includes hemorrhages of 5 various types, thromboses, pigmentation disorders, and pustular involvement. Early detection of skin symptoms of hemostasis disorders will improve the diagnosis and timely correction of such conditions.

Blood Coagulation Disorders↗

Skin disease patterns in Hormozgan, Iran.

BACKGROUND: Skin diseases are the most common cause of referrals to general practitioners and dermatologists, and are considered a health problem in developing countries. The aim of this study was to report the extent and patterns of skin diseases in southern Iran. METHODS: All new cases of skin diseases referred to Shahid Mohammadi Hospital, Bandar Abbas, Southern Iran over a 3-year period were retrospectively considered. The dermatologic diagnoses, sex and age were recorded for each patient and the diagnoses were classified according to the International Classification of Diseases (ICD-10). RESULTS: A total of 6841 patients were referred to the dermatology clinic of the hospital (48.27% male and 51.73% female). Infectious and parasitic diseases, including infections of the skin and subcutaneous tissues, were found to be the most common skin diseases (32.1%), followed by dermatitis and eczema (24.5%), disorders of the skin and subcutaneous tissue such as pigmentation disorders (9.7%), papulosquamous disorders (3.8%), urticaria and erythema (2.8%), burns, corrosion and unspecified injuries (3.3%), radiation-related disorders (2.3%), neoplasm (0.8%) and diseases of the oral cavity, salivary glands and jaw (0.3%). CONCLUSIONS: It appears that certain skin diseases such as dermatitis and eczema, superficial mycoses and scabies cause serious health problems in the region. Emphasis should be placed on this group of common skin diseases at all levels of personal health care, and suitable public health policies should be implemented in order to manage this problem rationally.

Adolescent↗

Brightly colored pigmentation in lower vertebrates: wonder searching its mechanisms and significance in the context of phylogeny.

This is a biographical sketch of my research and its related personal episodes with respect to brightly colored pigmentation in lower vertebrates. It includes a brief story of the studies on; (a) pterinosomes as a specific site of pteridine deposition in xanthophores or erythrophores of fish and amphibians, (b) a mosaic phenotype of chromatophores occurring in the reptiles and its implication for their developmental origin and differentiation mechanisms, (c) erythrophoroma as a tumor of erythrophores in goldfish, (d) the pluripotentials of erythrophoroma cells for expression of neural crest-derived characters in vitro, (e) pigment disorders occurring in hatchery-raised flounders and (f) recognition of pigment cell types by murine tyrosinase genes transfected into an orange-colored variant of medaka fish. Some of the personal affairs associated with the history of the Japanese community for pigment cell research were described to illustrate the background of these studies.

Amphibians↗

An association of melanophores appearing at metamorphosis as vehicles of asymmetric skin color formation with pigment anomalies developed under hatchery conditions in the Japanese flounder, Paralichthys olivaceus.

The mechanisms for asymmetric skin color formation in the Japanese flounder are studied with particular concerns to causes for pigment disorder (hypomelanosis) occurring under hatchery conditions. For an analysis of normal pigmentation, fish were raised with wild zooplanktons in an indoor hatchery, whilst for hypomelanosis, they were raised with Brazilian Artemia nauplii, a diet used in the hatcheries. Morphological observations, counting of melanophores, histochemical assay of DOPA-positive immature cells (melanoblasts), and radiometric estimation of tyrosinase activities in skins of developing larvae and juveniles indicate that 1) the structural plan for pigmentation in this species is bilaterally symmetric until metamorphosis, utilizing large-sized melanophores (hence larval melanophores) as main vehicles, and 2) an asymmetric coloration characteristic to metamorphosed juveniles is formed by an intensive development of smaller-sized melanophores (hence adult-type melanophores) appearing selectively in the ocular side at the later stages of metamorphosis and by an absence of it in the blind. These findings apparently indicate that 1) two types of melanophores occur in this species which differ with respect to morphological properties and developmental fate, and 2) selective differentiation of adult type melanophores in the ocular side of the body at or after metamorphosis is primarily responsible for an asymmetric coloration of its adult form. The similar assays on the fish fed with Artemia nauplii indicate that defective development of adult-type melanophores results in hypomelanosis in their ocular-sided skins, yielding a pigmentary pattern seen in the blind side of the metamorphosed juveniles with normal pigmentation.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging↗

Genodermatosis with reticulate, patchy and mottled pigmentation of the neck--a clue to rare dermatologic disorders.

Reticulate pigmentation of the neck is a common finding in numerous genodermatoses and acquired diseases. As the neck is readily accessible to medical inspection, it may serve as a diagnostic window for various dermatoses. Several entities out of the spectrum of ectodermal dysplasia present with reticulate or mottled pigmentation on the upper trunk and neck. The most impressive genodermatoses with punctate and reticulate pigmentation affecting the neck are the Naegeli-Franceschetti-Jadassohn syndrome, dermatopathia pigmentosa reticularis and dyskeratosis congenita. Reticulate pigmented anomaly of the flexures and lentiginosis, disorders of cornification and related entities are further genodermatoses which may involve the neck. In our review we have integrated inherited dermatoses which do not obligatorily affect the neck but have been well documented to do so. Our work should give the clinician a checklist on genodermatoses which may produce reticulate and mottled pigmentation on the neck.

Humans↗

Basal cell epithelioma: an unusual case.

A 59-year-old Caucasian male presented with a darkly pigmented inner canthal tumor with a six-month history of rapid growth. Histopathologic examination demonstrated to be a pigmented basal cell epithelioma. While basal cell epitheliomas of the eyelids are common ophthalmic disorders, pigmented lesions are unusual. Pigmented basal cell epitheliomas pose a difficulty in clinical diagnosis because of their resemblance to melanomas and pigmented nevi. The differential diagnosis and treatment of this lesion is reviewed.

Basal Cell Carcinoma↗

Initiation and regulation of CD8+T cells recognizing melanocytic antigens in the epidermis: implications for the pathophysiology of vitiligo.

Antigen-specific CD8+T lymphocytes play an important role in defense against cutaneous microbial infection and skin cancer as well as in the pathophysiology of autoimmune skin disease such as lupus erythematodes and vitiligo. We have explored the role of CD8+ cytotoxic T lymphocytes (CTL) in an experimental mouse model of vitiligo, a pigmentation disorder characterized by focal loss of melanocytes in the skin. Using genetic immunization techniques we found that pigment cells in the epidermis can be destroyed by CD8+ T cells specifically recognizing a single H2-Kb-binding peptide derived from the model melanocytic self antigen tyrosinase-related protein 2 (TRP2), a melanosomal enzyme involved in pigment synthesis. Experimental evidence suggests that peripheral tolerance of pigment cell-specific cytotoxic CD8+T cells is regulated in two steps. In the induction phase, stimulation and expansion of these T cells in vivo strictly depends on CD4+ T cell help. In the effector phase, autoimmune destruction of melanocytes in the skin depends on local inflammation facilitating the migration of T cells into the epidermis and supporting effector functions. Our results suggest that accidental stimulation of CD8+ CTL recognizing MHC class I-binding peptides derived from melanocytic proteins in the context of an inflammatory skin disease may play an important role in the pathophysiology of vitiligo. Further investigations will address the role of chemokines, chemokine receptors and adhesion molecules in this experimental system and will reveal the role of keratinocytes and Langerhans cells in regulating cutaneous CD8+ T cell responses.

Animals↗

Controlling gene expression in mice with tetracycline: application in pigment cell research.

Genetic manipulation techniques are widely used in mice to study the functions of genes. The most common strategy for assessing in vivo function involves making irreversible changes in the genome by homologous recombination. To complement this approach, a number of systems have been developed that allow specific and controlled expression of a gene. One of the more versatile and promising systems is based on the tetracycline (tet) responsive bacterial tetracycline repressor (TetR). In recent years, the tet system has proven to be a valuable method for understanding the function of genes involved in a number of physiological processes, including mouse models for human diseases such as cancer and neurological and pigment disorders. This review will highlight the power and elegance of the tet system by focusing on its utility in the study of two pigment cell-related biological problems, the pathogenesis of melanomas and melanocyte development in the embryo.

Animals↗

Free radical reduction in the human epidermis.

The human epidermis presents the first line of defense against invading free radicals. Therefore, the surface of the skin must be equipped to deal with both the penetration of ultra-violet light as well as the neutralization of reactive photochemical products such as superoxide anion radical, hydrogen peroxide and especially hydroxyl radicals. Consequently, the human epidermis contains a variety of anti-oxidants to reduce oxygen radicals and hydrogen peroxide. The photochemical production of hydroxyl radicals, from both extracellular and intracellular hydrogen peroxide, is of special significance to the integrity of cells in the human epidermis. Recently, both biochemical and clinical studies on the healthy human population, and on patients with pigmentation disorders, suggested a connection between free radical defense by plasma membrane associated thioredoxin reductase and melanin biosynthesis. This research provided the first evidence for a direct relationship between free radical concentration and pigmentation. Furthermore, this system has been shown to be regulated by both extracellular and intracellular calcium concentrations. Clinical studies show depigmentation disorders vitiligo and tyrosinase positive albinism (Hermansky-Pudlak syndrome) appear to have defective calcium uptake systems influencing both free radical defense and melanin biosynthesis.

Epidermis↗

Leopard spot retinal pigmentation in infancy indicating a peroxisomal disorder.

BACKGROUND: Neonatal adrenoleucodystrophy (NALD) is a rare disorder resulting from abnormal peroxisomal biogenesis. Affected patients present in infancy with developmental delay, hypotonia, and seizures. Blindness and nystagmus are prominent features. The authors suggest a characteristic leopard spot pigmentary pattern in the peripheral retina to be diagnostic. METHODS: Three patients are reported with this presentation; the characteristic retinal appearance resulted in early diagnosis for one of these. CONCLUSION: Leopard spot retinopathy in an infant with hypotonia, seizures, developmental delay, with or without dysmorphic features and hearing impairment, is a clue to the diagnosis of NALD.

Developmental Disabilities↗

Eyelid tattooing.

Tattooing has recently regained popularity in medicine. Cosmetic blepharopigmentation for eyelid enhancement by permanent eyeliner has received considerable attention. In addition, permanent pigmentation has been used for eyebrow simulation, camouflaging of scars, nipple areolar pigmentation following breast reconstruction, and the management of several other pigment disorders. This article emphasizes the principles and technique of blepharopigmentation.

Burns↗

Hypopigmented skin lesions associated with atopic dermatitis in asthma.

The association of vitiligo and other skin pigmentary disorders has been well shown with autoimmune diseases, i.e., systemic lupus erythematosus, rheumatoid arthritis. Furthermore, in certain diseases such as diabetes mellitus and adrenal insufficiency the incidence of pigmentation disorders is higher than in the general population. A total of 53 male and 46 female patients with a diagnosis of asthma, based on clinical findings and pulmonary function tests (PFT), were thoroughly examined for the presence of hypopigmented skin lesions and compared with 100 nonasthmatic controls with a similar demographic distribution. The patients were questioned regarding the history of steroid treatment in the past and present and subdivided into five groups based on the duration of therapy. Adrenal gland function was tested by 8:00 A.M. serum cortisol concentration in the group of patients with a history of steroid use as well as in some patients without this history. These patients were also tested for absolute lymphocyte and eosinophil count. Hypopigmented lesions were noted in 36.4% of asthmatic patients and in 11% nonasthmatic controls (p-value < 0.01). There were no differences in the location, but the size of lesions per patient was larger in the asthmatic than the control group. Twenty of 55 patients who had a history of steroid treatment in the past had hypopigmented skin lesions as compared to 16 of 42 patients who had no history of steroid treatment. We conclude that there is a higher coincidence of hypopigmented skin lesions, mainly vitiligo-type in asthmatic patients, compared to the general population, which is not related to a history of steroid treatment or incidence of adrenal insufficiency in these patients.

Adrenal Cortex Hormones↗

A retrospective study of the efficacy of intense pulsed light for the treatment of dermatologic disorders presenting to a cosmetic skin clinic.

BACKGROUND: One hundred seven patients presenting to a cosmetic skin clinic were treated with intense pulsed light (IPL) over a 12-month period. The main categories of patients offered treatment were those with vascular problems such as rosacea, facial telangiectasia, and spider nevi; pigmentation disorders such as solar damage, lentigines, and hyperpigmentation; and assorted problems such as scarring and poikiloderma. METHODS: Each patient who entered into the study had the full medical history taken and a dermatologic assessment. Polaroid photographs were taken and the images used for comparison before and after treatment. Outcomes were assessed by physicians' global assessment and a patients' postal questionnaire. Patients were treated with a Lumina IPL (Lynton Lasers Ltd) using a multiple pulsing facility with variable interpulse spacing, incorporating a 585-nm head. Fluence levels varied between 10 and 40 J/cm(2), although the average fluence over all patients was 25 J/cm(2). The delay was set between 10 and 30 ms and two to four pulses were used. Results Excellent results were seen in 80% of patients treated. There was a high patient satisfaction rate and low prevalence of side effects. CONCLUSIONS: IPL has been shown to provide a safe and effective noninvasive treatment for a wide range of dermatologic disorders and is suitable for wider use in primary care.

Journal Article↗