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Huntington Disease l: Introduction01:21

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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
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Graves' disease is an autoimmune disorder that causes hyperthyroidism, or overactivity of the thyroid gland. It results from autoantibodies called thyroid-stimulating immunoglobulins (TSIs), which bind to thyroid-stimulating hormone (TSH) receptors, leading to overstimulation of hormone production and a hypermetabolic state.EtiologyAlthough considered idiopathic, Graves’ disease has well-established contributing factors. There is a strong genetic component, with increased prevalence...
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Delayed-Type Hypersensitivity (DTH), or Type IV hypersensitivity, is a cell-mediated immune response. It occurs when T cells, rather than antibodies, mediate a reaction to specific antigens. It is characterized by a delayed onset (1-2 days) and involves the recruitment of macrophages to the inflammation site.The initiation of a DTH response begins with the sensitization of T cells. During this phase, which lasts at least 1-2 weeks, antigen-specific T cells are activated, clonally expanded, and...
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Related Experiment Video

Updated: May 1, 2026

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Hailey-hailey disease responding to thalidomide.

K Bala Nanda1, Celia Soni Saldanha1, Martis Jacintha1

  • 1Department of Dermatology, Venereology and Leprosy, Father Muller Medical College, Mangalore, Karnataka, India.

Indian Journal of Dermatology
|April 5, 2014
PubMed
Summary
This summary is machine-generated.

Hailey-Hailey disease (HHD) is a rare skin disorder causing blisters. This case study shows thalidomide effectively treated a severe, persistent case of HHD resistant to other therapies.

Keywords:
Familial benign chronic pemphigusHailey-Hailey diseasethalidomide

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Area of Science:

  • Dermatology
  • Genetics

Background:

  • Familial benign chronic pemphigus, also known as Hailey-Hailey disease (HHD), is a rare autosomal dominant genetic disorder.
  • HHD is characterized by recurrent blistering and erosions, primarily affecting intertriginous skin areas.

Purpose of the Study:

  • To report a case of severe, recalcitrant Hailey-Hailey disease.
  • To evaluate the efficacy of thalidomide as a treatment option for HHD.

Main Methods:

  • A case study of a patient with severe, recalcitrant HHD is presented.
  • The patient had previously failed multiple treatments including antibiotics, corticosteroids, and dapsone.
  • Thalidomide was administered as a treatment modality.

Main Results:

  • The patient with severe Hailey-Hailey disease showed a significant positive response to thalidomide treatment.
  • This suggests thalidomide may be a viable option for recalcitrant HHD cases.

Conclusions:

  • Thalidomide can be an effective treatment for severe and recalcitrant cases of Hailey-Hailey disease.
  • Further research into thalidomide's role in HHD management is warranted.