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Anais Brasileiros de Dermatologia 2014Cutaneous hyperpigmentations are frequent complaints, motivating around 8.5% of all dermatological consultations in our country. They can be congenital, with different... (Review)
Review
Cutaneous hyperpigmentations are frequent complaints, motivating around 8.5% of all dermatological consultations in our country. They can be congenital, with different patterns of inheritance, or acquired in consequence of skin problems, systemic diseases or secondary to environmental factors. The vast majority of them are linked to alterations on the pigment melanin, induced by different mechanisms. This review will focus on the major acquired hyperpigmentations associated with increased melanin, reviewing their mechanisms of action and possible preventive measures. Particularly prominent aspects of diagnosis and therapy will be emphasized, with focus on melasma, post-inflammatory hyperpigmentation, periorbital pigmentation, dermatosis papulosa nigra, phytophotodermatoses, flagellate dermatosis, erythema dyschromicum perstans, cervical poikiloderma (Poikiloderma of Civatte), acanthosis nigricans, cutaneous amyloidosis and reticulated confluent dermatitis.
Topics: Diagnosis, Differential; Female; Humans; Hyperpigmentation; Male; Melanins; Skin; Skin Diseases
PubMed: 24626644
DOI: 10.1590/abd1806-4841.20142353 -
British Medical Journal Jul 1968Fourteen cases are described in which the local application of corticosteroid preparations to ringworm infections of the skin have resulted in unusual clinical pictures....
Fourteen cases are described in which the local application of corticosteroid preparations to ringworm infections of the skin have resulted in unusual clinical pictures. A kerion-like lesion due to Trichophyton rubrum, intertriginous infections simulating candidiasis and due to Epidermophyton floccosum, and pictures resembling poikiloderma, papular rosacea, and indeterminate leprosy are among the changes that were seen in these patients.
Topics: Adolescent; Adult; Aged; Betamethasone; Diagnosis, Differential; Eczema; Epidermophyton; Female; Fluocinolone Acetonide; Glucocorticoids; Griseofulvin; Humans; Keratins; Male; Middle Aged; Skin Diseases; Tinea; Triamcinolone Acetonide; Trichophyton; Valerates
PubMed: 5662546
DOI: 10.1136/bmj.3.5611.149 -
Revista Paulista de Pediatria : Orgao... 2023The aim of this study was to describe the disease and treatment and to alert health professionals for the identification of signs and symptoms and the need for an early...
OBJECTIVE
The aim of this study was to describe the disease and treatment and to alert health professionals for the identification of signs and symptoms and the need for an early diagnosis in patients with xeroderma pigmentosum (XP).
CASE DESCRIPTION
An 8-year-old male patient was referred to the Joana de Gusmão Hospital (HIJG) in 2021 for evaluation and specialized care. Previously, the child was followed in his place of origin by oncologic and palliative care, where he was submitted to surgeries and chemotherapy. He was admitted to the HIJG using vismodegib, acitrein, tramadol, and solar protective measures. On physical examination, there were tumors and disseminated macular verrucous and ulcerated lesions. The imaging examination showed solid and expansive lesions on the face, and atelectasis and fibroscarring changes in the lung. The histopathological report proved the existence of melanocanthoma, carcinoma, and pyogenic granuloma. After the evaluation of the case, no surgery, chemotherapy, or radiotherapy was performed. It was decided to maintain the palliative treatment and to continue the use of tramadol for pain, and vismodegib and acitretin were used to control carcinomas and prophylactic measures.
COMMENTS
The XP is a rare disease of autosomal recessive inheritance whose mechanism comes from failure in the DNA repair by exposure to ultraviolet rays, resulting in lesions on the skin and mucous membranes. They start as sunburns and can progress to melanosis, areas with altered pigmentation, premature aging, poikiloderma, and areas of high risk for neoplasms.
Topics: Child; Male; Humans; Xeroderma Pigmentosum; Tramadol; DNA Repair; Skin Diseases; Carcinoma; Skin Neoplasms
PubMed: 36921168
DOI: 10.1590/1984-0462/2023/41/2021390 -
Biology of Blood and Marrow... Dec 2005This consensus document is intended to serve 3 functions. First, it standardizes the criteria for diagnosis of chronic graft-versus-host disease (GVHD). Second, it...
National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group report.
This consensus document is intended to serve 3 functions. First, it standardizes the criteria for diagnosis of chronic graft-versus-host disease (GVHD). Second, it proposes a new clinical scoring system (0-3) that describes the extent and severity of chronic GVHD for each organ or site at any given time, taking functional impact into account. Third, it proposes new guidelines for global assessment of chronic GVHD severity that are based on the number of organs or sites involved and the degree of involvement in affected organs (mild, moderate, or severe). Diagnosis of chronic GVHD requires the presence of at least 1 diagnostic clinical sign of chronic GVHD (e.g., poikiloderma or esophageal web) or the presence of at least 1 distinctive manifestation (e.g., keratoconjunctivitis sicca) confirmed by pertinent biopsy or other relevant tests (e.g., Schirmer test) in the same or another organ. Furthermore, other possible diagnoses for clinical symptoms must be excluded. No time limit is set for the diagnosis of chronic GVHD. The Working Group recognized 2 main categories of GVHD, each with 2 subcategories. The acute GVHD category is defined in the absence of diagnostic or distinctive features of chronic GVHD and includes (1) classic acute GVHD occurring within 100 days after transplantation and (2) persistent, recurrent, or late acute GVHD (features of acute GVHD occurring beyond 100 days, often during withdrawal of immune suppression). The broad category of chronic GVHD includes (1) classic chronic GVHD (without features or characteristics of acute GVHD) and (2) an overlap syndrome in which diagnostic or distinctive features of chronic GVHD and acute GVHD appear together. It is currently recommended that systemic therapy be considered for patients who meet criteria for chronic GVHD of moderate to severe global severity.
Topics: Chronic Disease; Clinical Trials as Topic; Consensus; Consensus Development Conferences, NIH as Topic; Diagnosis, Differential; Female; Graft vs Host Disease; Humans; Male; National Institutes of Health (U.S.); United States
PubMed: 16338616
DOI: 10.1016/j.bbmt.2005.09.004 -
The Pan African Medical Journal 2017
PubMed: 28819515
DOI: 10.11604/pamj.2017.27.94.12959 -
Journal of Korean Medical Science Feb 2012Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic...
Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.
Topics: Abnormalities, Multiple; Adolescent; Elastic Tissue; Fingers; Humans; Hyperpigmentation; Male; Micrognathism; Rothmund-Thomson Syndrome; Sclerosis; Skin Diseases
PubMed: 22323875
DOI: 10.3346/jkms.2012.27.2.225 -
Current Opinion in Hematology Jan 2013Neutropenia is a feature of several primary immunodeficiency diseases (PIDDs). Because of the diverse pathophysiologies of the PIDDs and the rarity of each disorder,... (Review)
Review
PURPOSE OF REVIEW
Neutropenia is a feature of several primary immunodeficiency diseases (PIDDs). Because of the diverse pathophysiologies of the PIDDs and the rarity of each disorder, data are often lacking, leading to the necessity of empiric treatment. Recent developments in the understanding of neutropenia in several of the PIDDs make a review of the data timely.
RECENT FINDINGS
The category of severe congenital neutropenia continues to expand. Mutations in G6PC3 have been identified as the cause of neutropenia in a minority of previously molecularly undefined cases. Recent advances have broadened our understanding of the pathophysiology and the clinical expression of this disorder. A possible function of the C16orf57 gene has been hypothesized that may explain the clinical overlap between Clerucuzio-type poikiloderma with neutropenia and other marrow diseases. Plerixafor has been shown to be a potentially useful treatment in the warts, hypogammaglobulinemia, infection, and myelokathexis syndrome. Investigations of patients with adenosine deaminase deficient severe combined immunodeficiency have identified neutropenia, and particularly susceptibility to myelotoxins, as a feature of this disorder. Granulocyte-colony stimulating factor is the treatment of choice for neutropenia in PIDD, whereas hematopoietic cell transplantation is the only curative option.
SUMMARY
The number of PIDDs associated with neutropenia has increased, as has our understanding of the range of phenotypes. Additional data and hypotheses have been generated helping to explain the diversity of presentations of neutropenia in PIDDs.
Topics: Animals; Hematopoietic Stem Cell Transplantation; Humans; Immunologic Deficiency Syndromes; Neutropenia
PubMed: 23196894
DOI: 10.1097/MOH.0b013e32835aef1c -
Indian Journal of Dermatology,... 2011Facial melanoses (FM) are a common presentation in Indian patients, causing cosmetic disfigurement with considerable psychological impact. Some of the well defined... (Review)
Review
Facial melanoses (FM) are a common presentation in Indian patients, causing cosmetic disfigurement with considerable psychological impact. Some of the well defined causes of FM include melasma, Riehl's melanosis, Lichen planus pigmentosus, erythema dyschromicum perstans (EDP), erythrosis, and poikiloderma of Civatte. But there is considerable overlap in features amongst the clinical entities. Etiology in most of the causes is unknown, but some factors such as UV radiation in melasma, exposure to chemicals in EDP, exposure to allergens in Riehl's melanosis are implicated. Diagnosis is generally based on clinical features. The treatment of FM includes removal of aggravating factors, vigorous photoprotection, and some form of active pigment reduction either with topical agents or physical modes of treatment. Topical agents include hydroquinone (HQ), which is the most commonly used agent, often in combination with retinoic acid, corticosteroids, azelaic acid, kojic acid, and glycolic acid. Chemical peels are important modalities of physical therapy, other forms include lasers and dermabrasion.
Topics: Administration, Topical; Animals; Facial Dermatoses; Humans; Hyperpigmentation; Keratolytic Agents; Melanosis; Tretinoin; Ultraviolet Rays
PubMed: 21860153
DOI: 10.4103/0378-6323.84046 -
The Journal of Investigative Dermatology May 2016
Review
Topics: Adolescent; Blister; DNA Methylation; Epidermolysis Bullosa; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Humans; Melanoma; Nevus, Pigmented; PTEN Phosphohydrolase; Periodontal Diseases; Photosensitivity Disorders; Proto-Oncogene Proteins B-raf; Sensitivity and Specificity; Skin Neoplasms
PubMed: 27140323
DOI: 10.1016/j.jid.2016.03.007