55: Mucous Membrane Pemphigoid Flashcards
What is Mucous Membrane Pemphigoid characterized by?
Mucous Membrane Pemphigoid is characterized by erosive lesions of mucous membranes and skin that result in scarring of at least some sites of involvement.
What is the incidence of Mucous Membrane Pemphigoid?
The incidence of Mucous Membrane Pemphigoid is 1-2 cases per million per year.
What are the common clinical features of Mucous Membrane Pemphigoid?
Common clinical features include the onset of painful, erosive, and/or blistering lesions on one or more mucosal surfaces, with few skin lesions on the upper body noted.
Which site is most frequently affected in Mucous Membrane Pemphigoid?
The mouth is the most frequently affected site, often being the first and only site affected, including areas such as the gingiva, buccal mucosa, and palate.
What ocular complications can arise from Mucous Membrane Pemphigoid?
Ocular complications can include conjunctivitis that progresses to scarring, characterized by shortened fornices, symblepharons, and ankyloblepharons, as well as ectropion and trichiasis.
What are the risk factors associated with Mucous Membrane Pemphigoid?
Risk factors for Mucous Membrane Pemphigoid include advanced age, female gender, and the HLADQB1*0301 allele.
What findings are typically observed in light microscopy for Mucous Membrane Pemphigoid?
Light microscopy findings often show a subepidermal blister and a dermal leukocytic infiltrate, including lymphocytes, histiocytes, and variable numbers of neutrophils and eosinophils.
A 65-year-old female presents with painful erosive lesions in her mouth and conjunctivitis. What is the most likely diagnosis, and what are the key clinical features to confirm it?
The most likely diagnosis is mucous membrane pemphigoid (MMP). Key clinical features include erosive lesions in the mouth (gingiva, buccal mucosa, palate) and ocular involvement such as conjunctivitis that progresses to scarring.
A patient with MMP has developed adhesions in the oral cavity. What complications might arise from this, and how can they be managed?
Adhesions in the oral cavity can lead to tissue loss, dental complications, and difficulty in oral hygiene. Management includes preventing further progression through immunosuppressive therapy and maintaining oral hygiene.
A patient with suspected MMP presents with bilateral ocular symptoms. What diagnostic steps should be taken to confirm ocular involvement?
The patient should be examined by an ophthalmologist. Diagnostic steps include direct immunofluorescence (DIF) of perilesional tissue to detect immunoreactants in the epithelial basement membrane.
A 60-year-old patient with MMP reports hoarseness and difficulty breathing. What is the likely site of involvement, and what are the potential complications?
The likely site of involvement is the larynx. Potential complications include supraglottic stenosis, airway compromise, and loss of phonation.
A patient with MMP has skin lesions on the upper trunk. What are the typical characteristics of these lesions?
Skin lesions in MMP typically consist of small vesicles or bullae on erythematous or urticarial bases. They rupture easily and may appear as crusted papules or plaques.
What are the primary autoantibodies involved in the pathogenesis of MMP, and what is their target?
The primary autoantibodies involved are IgG and IgA anti-basement membrane autoantibodies. They target autoantigens in the epidermal basement membrane, such as BP180.
A patient with MMP has a biopsy showing subepidermal blisters and a dermal leukocytic infiltrate. What additional findings might be seen in mucosal versus skin lesions?
In mucosal lesions, plasma cells are often present, while skin lesions may show eosinophils and neutrophils. Older lesions may appear relatively ‘cell poor’ with fibroblast proliferation and lamellar fibrosis.
A patient with MMP has chronic sinusitis and impaired airflow. What mucosal site is likely involved, and what are the potential complications?
The nasopharyngeal mucosa is likely involved. Potential complications include scarring, tissue loss, and chronic sinusitis.
A patient with MMP has developed ectropion and trichiasis. What is the underlying cause, and how should it be managed?
The underlying cause is conjunctival scarring. Management includes referral to an ophthalmologist and treatment to prevent further scarring, such as immunosuppressive therapy.
A patient with MMP has difficulty swallowing and weight loss. What is the likely site of involvement, and what are the potential complications?
The likely site of involvement is the esophagus. Potential complications include strictures, dysphagia, odynophagia, and aspiration.
What are the risk factors for developing MMP?
Risk factors include advanced age, female gender, and the presence of the HLA-DQB1*0301 allele.
A patient with MMP has scarring of the lacrimal ducts. What are the clinical consequences, and how can they be addressed?
Scarring of the lacrimal ducts can lead to decreased tear secretion, loss of mucosal goblet cells, and an unstable tear film. Management includes artificial tears and addressing the underlying inflammation.
A patient with MMP has vaginal stenosis. What are the potential complications, and how should they be managed?
Potential complications include pain, difficulty with sexual activity, and urinary issues. Management includes immunosuppressive therapy and possibly surgical intervention.
What are the challenges in diagnosing ocular MMP, and how can they be addressed?
Challenges include the subtle and nonspecific nature of early ocular disease. They can be addressed by early referral to an ophthalmologist and the use of DIF for diagnosis.
A patient with MMP has developed supraglottic stenosis. What are the clinical implications, and what interventions might be required?
Supraglottic stenosis can lead to airway compromise. Interventions may include immunosuppressive therapy and surgical procedures to maintain airway patency.
A patient with MMP has developed ankyloblepharon. What is the underlying cause, and what are the potential complications?
The underlying cause is chronic ocular involvement leading to scarring. Potential complications include impaired eyelid function and vision loss.
What are the common sites of skin involvement in MMP, and how do the lesions typically present?
Common sites of skin involvement include the scalp, head, neck, and upper trunk. Lesions typically present as small vesicles or bullae on erythematous or urticarial bases.
