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Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
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Granulocyte-dependent Autoantibody-induced Skin Blistering
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[Chilblain lupus in an adolescent].

Daciana Elena Brănişteanu1, Andreea Dana Molodoi, Laura Stătescu

  • 1Facultatea de Medicină, Clinica Dermatologie, Universitatea de Medicină si Farmacie "Gr.T. Popa" Iaşi.

Revista Medico-Chirurgicala a Societatii De Medici Si Naturalisti Din Iasi
|March 6, 2010
PubMed
Summary

Chilblain lupus (lupus pernio) is a rare skin condition. Early diagnosis and treatment with antimalarial drugs and sun protection effectively cured a 16-year-old patient’s lesions.

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Area of Science:

  • Dermatology
  • Rheumatology
  • Immunology

Background:

  • Chilblain lupus (lupus pernio) is a rare, chronic cutaneous lupus erythematosus variant.
  • It predominantly affects adults and can be challenging to diagnose without characteristic facial or scalp lesions.
  • This case highlights a pediatric presentation mimicking perniosis.

Observation:

  • A 16-year-old female presented with painful, red-to-violaceous, infiltrated plaques on her fingers and toes, worsening in cold weather.
  • Lesions appeared at age 13 and were accompanied by mild facial erythema ('butterfly rash').
  • The patient had good general health with no systemic involvement.

Findings:

  • Initial clinical suspicion of chilblains (perniosis) was ruled out by detailed history, serological tests (positive antinuclear antibodies, anti-dsDNA antibodies), and skin biopsy.
  • Histopathology confirmed lupus pernio, a specific type of cutaneous lupus erythematosus.
  • Laboratory findings indicated autoimmune activity consistent with lupus erythematosus.

Implications:

  • This case underscores the importance of considering lupus pernio in adolescents with chilblain-like lesions, even without typical facial involvement.
  • Prompt diagnosis and treatment, including antimalarial therapy (Plaquenil) and photoprotection, led to complete resolution of cutaneous manifestations.
  • Early intervention can prevent disease progression and improve patient outcomes in lupus pernio.