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Pemphigoid, Cicatricial

Jeremy Golding, FAAFP, MD Reviewed 04/2024
 


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

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

REFERENCES

1
Kirtschig  G, Murrell  D, Wojnarowska  F, et al. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev. ...

ADDITIONAL READING

Wang K, Seitzman G, Gonzales JA. Ocular cicatricial pemphigoid. Curr Opin Ophthalmol. 2018;29(6):543–551.  

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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