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Pemphigoid, Cicatricial

Marvin Sineath Jr and Alexandra Ranski Reviewed 06/2022
 


BASICS

Cicatricial pemphigoid (CP) is a rare blistering disorder that mostly affects mucous membranes, which may lead to scarring. 

DESCRIPTION

  • Cicatricial pemphigoid (CP) is a chronic condition resulti...

DIAGNOSIS

Evaluate by clinical assessment. Biopsy is confirmatory. 

HISTORY

  • Oral: pain, sensitivity, bleeding, mucosal lesions

  • Ocular: burning, dryness/tearing, itching, foreign body sensation, vision ch...

TREATMENT

  • Primary goal of treatment in CP is to prevent progression to blindness, strictures, and airway obstruction.

  • Low-risk patients (oral mucosa ± skin involvement)

    • Topical corticosteroids (moderate...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Cancer screening for patients with anti–laminin-332 autoantibodies (1)

  • All patients should be seen by an ophthalmologist as early detection of ocular disease is h...

REFERENCES

1
Xu  HH, Werth  VP, Parisi  E, et al. Mucous membrane pemphigoid. Dent Clin North Am.  2013; 57(4): 611– 630. doi:10.1016/j.cden.2013.07.0...

ADDITIONAL READING

  • Buonavoglia A, Leone P, Dammacco R. Pemphigus and mucous membrane pemphigoid: an update from diagnosis to therapy. Autoimmun Rev. 2019;18(4):349–358.

  • ...

CODES

ICD10

L12.1 Cicatricial pemphigoid 

SNOMED

  • 34250006 Benign mucous membrane pemphigoid (disorder)

  • 76092003 Benign mucous membrane pemphigoid with ocular involvement

  • 402441007 Oral involvement by mucou...

CLINICAL PEARLS

  • Patients diagnosed with CP require thorough skin exams, including oral, ocular, and genital mucous membranes.

  • Scarring is the clinical hallmark of disease although it may not be evident...

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