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Papillary Dermis01:11

Papillary Dermis

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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The term desmosome derives from the Greek words "desmo" and "soma" meaning "adhesion bodies." This structure was first observed during the late 1800s and described as small, dense nodules in the epidermis. Desmosomes are button-like structures that help form an interlinked network of intermediate filaments across the cells. These junctions are  essential to hold cells together under mechanical stress and to maintain tissue integrity. Desmosomes are multi-protein complexes comprising desmosomal...
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Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Anhidrotic ectodermal dysplasia with palmoplantar keratoderma: an unusual presentation.

Kamaldeep Sandhu1, Sanjeev Handa, A J Kanwar

  • 1Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

International Journal of Dermatology
|June 7, 2007
PubMed
Summary

Anhidrotic ectodermal dysplasia (AED) typically lacks sweat glands, hypotrichosis, and hypodontia. This report details two brothers with classic AED and palmoplantar keratoderma, a rare co-occurrence.

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Area of Science:

  • Genetics and Human Diseases
  • Dermatology
  • Rare Genetic Disorders

Background:

  • Anhidrotic ectodermal dysplasia (AED), also known as Christ-Siemens-Touraine syndrome, is a rare genetic disorder.
  • It is primarily characterized by the absence of sweat glands (anhidrosis), sparse hair (hypotrichosis), and missing teeth (hypodontia).
  • AED predominantly follows an X-linked inheritance pattern, though autosomal recessive forms exist.

Observation:

  • Palmoplantar keratoderma, a thickening of the skin on the palms and soles, is typically associated with hidrotic ectodermal dysplasia.
  • This report presents two brothers exhibiting classical symptoms of Anhidrotic ectodermal dysplasia.
  • Notably, these individuals also presented with palmoplantar keratoderma.

Findings:

  • The study documents a rare co-presentation of Anhidrotic ectodermal dysplasia and palmoplantar keratoderma in two affected siblings.
  • This finding challenges the classical understanding of ectodermal dysplasia subtypes and their associated dermatological manifestations.
  • The observed cases suggest a potential overlap or shared genetic/pathological pathways between these conditions.

Implications:

  • This case report expands the clinical spectrum of Anhidrotic ectodermal dysplasia.
  • It highlights the importance of considering palmoplantar keratoderma in the differential diagnosis of AED patients.
  • Further research into the genetic and molecular mechanisms underlying this combined presentation is warranted.