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Related Concept Videos

Diabetic Retinopathy01:27

Diabetic Retinopathy

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DefinitionDiabetic retinopathy is a microvascular complication of diabetes affecting the retinal blood vessels.Risk FactorsDiabetic retinopathy is present in almost all individuals with type 1 diabetes and more than 60% of those with type 2 diabetes after two decades of disease.The risk increases with poor glycemic control, hypertension, dyslipidemia, smoking, pregnancy, and puberty.Although cataracts and glaucoma are also more frequent in people with diabetes, retinopathy remains the leading...
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Papillary Dermis01:11

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Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
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Peripheral Artery Disease I: Introduction01:30

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Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
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Diabetic Foot Ulcer01:31

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Definition A diabetic foot ulcer (DFU) is a chronic, non-healing wound that develops in individuals with diabetes. It typically occurs on pressure-bearing areas such as the heel, metatarsal heads, or hallux, and carries a high risk of infection and amputation.Pathophysiology • The development of DFUs can be explained by four interconnected mechanisms: neuropathy, ischemia, infection, and impaired wound healing. • Neuropathy is the most common factor. Sensory...
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Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

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Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
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The Goeckerman Regimen for the Treatment of Moderate to Severe Psoriasis
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Palmoplantar keratoderma with progressive gingivitis and recurrent pyodermas.

Tyler A Moss, Anne P Spillane1, Sam F Almquist

  • 1Department of Dermatology, Kimbrough Ambulatory Care Center, 2480 Llewellyn Ave, Ste 5800, Fort Meade, MD 20755-5129, USA. anne.p.spillane.mil@mail.mil.

Cutis
|May 13, 2014
PubMed
Summary
This summary is machine-generated.

Papillon-Lefèvre syndrome (PLS) is a rare genetic disorder causing palmoplantar keratoderma, gingivitis, and skin infections. This review details a case study and discusses PLS pathophysiology, diagnosis, treatment, and related palmoplantar keratoderma syndromes.

Area of Science:

  • Dermatology
  • Genetics
  • Oral Medicine

Background:

  • Papillon-Lefèvre syndrome (PLS) is a rare autosomal-recessive disorder.

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  • It is characterized by palmoplantar keratoderma (PPK), severe periodontitis leading to premature tooth loss, and recurrent pyoderma.
  • PLS results from mutations in the CTS1 gene, affecting keratinocyte differentiation and immune function.