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Pemphigoid, Cicatricial

Naureen Rafiq, MBBS and Keidren Lewi, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Cicatricial pemphigoid (CP) is a rare, chronic, acquired autoimmune subepithelial blistering disorder that mainly affects mucous membranes.

  • In descending order of involvement, CP affe...

DIAGNOSIS

Evaluate by clinical assessment. Direct immunofluorescence (DIF) is considered the gold standard for diagnosis. 

HISTORY

  • Oral: pain, sensitivity, bleeding, mucosal lesions

  • Ocular: burning, dryn...

TREATMENT

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

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

    • Topical corticosteroids (moderat...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

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

  • All patients should be seen by an ophthalmologist. Early detection of disease is key.

  • Long-te...

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.2013.07.003. [View Abstract on OvidMedline]
2
Kirtschig G, Murr...

ADDITIONAL READING

  • Allen CP, Venning V. Mucous membrane pemphigoid. In: Lebwohl MG, Heymann WR, Berth-Jones J, et al, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Phi...

CODES

ICD10

L12.1 Cicatricial pemphigoid 

ICD9

  • 694.60 Benign mucous membrane pemphigoid without mention of ocular involvement

  • 694.61 Benign mucous membrane pemphigoid with ocular involvement

SNOMED

  • 34250006...

CLINICAL PEARLS

  • Patients diagnosed with CP require thorough mucous membrane and skin examinations.

  • Scarring is the clinical hallmark of disease, although it is not always evident.

  • Treatments are disease...

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