Khushveer Choudhary

Mucous Membrane Pemphigoid कारण, लक्षण और इलाज

Mucous Membrane Pemphigoid (MMP / म्यूकस मेम्ब्रेन पेम्फिगॉइड) एक rare chronic autoimmune blistering disorder है।

  • मुख्य रूप से mucous membranes (मुंह, आँख, नाक, गले, genitals) प्रभावित होते हैं।
  • Skin involvement कम होता है।
  • Condition progressive होती है और scarring (सकलिंग) का कारण बन सकती है।
  • Mostly affects older adults (50–70 years)

Mucous Membrane Pemphigoid क्या है  (What is Mucous Membrane Pemphigoid)

  • Autoimmune disorder: body की immune system mucous membrane को attack करती है
  • Leads to subepithelial blistering and erosions
  • Commonly affects:
    1. Oral cavity (मुंह)
    1. Eyes (conjunctiva)
    1. Nasal cavity (नाक)
    1. Genitals (genital mucosa)
  • Untreated lesions can cause permanent scarring and functional impairment

Mucous Membrane Pemphigoid कारण (Causes of Mucous Membrane Pemphigoid)

1. Autoimmune Mechanism

  • Body produces autoantibodies against basement membrane proteins
  • Key targets: BP180 (collagen XVII), BP230, laminin 332

2. Genetic Predisposition

  • HLA associations observed (HLA-DQB1*0301)
  • Family history may increase risk

3. Environmental / Drug Triggers

  • Certain medications (penicillamine, furosemide) may trigger or exacerbate
  • Trauma or chronic irritation can worsen lesions

Mucous Membrane Pemphigoid लक्षण (Symptoms of Mucous Membrane Pemphigoid)

  • Painful blisters or erosions in mouth
  • Difficulty in swallowing or eating
  • Red, irritated, or bleeding eyes (conjunctivitis)
  • Scarring in conjunctiva → vision problems
  • Nasal crusting, epistaxis (nosebleeds)
  • Genital erosions → pain or discomfort
  • Rare: skin blisters

Symptoms are usually chronic, recurrent, and progressive

Mucous Membrane Pemphigoid कैसे पहचाने (Diagnosis of Mucous Membrane Pemphigoid)

  1. Clinical Examination – oral, ocular, nasal, or genital lesions
  2. Biopsy and Histopathology – subepithelial blistering, inflammatory infiltrate
  3. Direct Immunofluorescence (DIF) – gold standard: linear deposition of IgG, IgA, C3 at basement membrane
  4. Blood Tests – detect circulating autoantibodies
  5. Ophthalmology / ENT Evaluation – assess extent of mucosal involvement

Early diagnosis essential to prevent scarring and functional loss

Mucous Membrane Pemphigoid इलाज (Treatment of Mucous Membrane Pemphigoid)

1. Topical Therapy

  • Corticosteroid gels or ointments for oral or localized lesions
  • Immunosuppressive eye drops for ocular involvement

2. Systemic Therapy

  • Corticosteroids (Prednisone) – control active disease
  • Immunosuppressants: azathioprine, mycophenolate mofetil, cyclophosphamide
  • Biologics: Rituximab in refractory cases

3. Supportive Care

  • Oral hygiene maintenance
  • Lubricating eye drops to prevent ocular scarring
  • Pain management with analgesics

4. Regular Monitoring

  • Ophthalmology follow-up to prevent vision loss
  • Dental follow-up for oral lesions
  • Monitor systemic side effects of immunosuppressive therapy

Early and aggressive therapy improves quality of life and prevents complications

रोकथाम (Prevention)

  • No guaranteed prevention due to autoimmune nature
  • Early recognition and treatment of initial lesions
  • Avoid trauma to oral and ocular mucosa
  • Careful monitoring if on medications that may trigger disease

सावधानियाँ (Precautions)

  • Avoid rubbing or irritating affected mucosa
  • Maintain good oral and ocular hygiene
  • Regular follow-up with dermatologist and ophthalmologist
  • Monitor for side effects of systemic immunosuppressants

FAQs (अक्सर पूछे जाने वाले प्रश्न)

Q1. क्या Mucous Membrane Pemphigoid contagious है?

नहीं, यह autoimmune disorder है, contagious नहीं।

Q2. क्या यह केवल मुंह में होता है?

नहीं, यह oral, ocular, nasal, genital mucosa सभी में हो सकता है।

Q3. क्या यह पूरी तरह cure हो सकता है?

Complete cure मुश्किल है, लेकिन immunosuppressive therapy और early intervention से remission possible है।

Q4. Scarring irreversible है?

हाँ, untreated lesions में ocular और oral scarring permanent हो सकती है।

निष्कर्ष (Conclusion)

Mucous Membrane Pemphigoid (म्यूकस मेम्ब्रेन पेम्फिगॉइड) एक chronic autoimmune disorder है जो mucous membranes में blistering और scarring का कारण बनता है।
Early diagnosis, systemic/topical therapy, eye and dental care, और regular monitoring से complications को prevent किया जा सकता है।
अगर किसी को oral, ocular या genital erosions दिखाई दें, तो तुरंत dermatologist और ophthalmologist से consultation जरूरी है।

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