An encyclopedia about voice, swallowing, airway, coughing, & other head + neck disorders.

Pemphigoid

Pemphigoid is an autoimmune condition in which the body mistakenly attacks as “foreign” the attachment between surface tissue and the tissue beneath it. This can cause blistering, erosions, or areas of fragile mucosa.

Pemphigoid most often affects the skin, where it may appear as blisters, sores, or areas of inflammation. In some individuals, it also affects mucous membranes, including those of the mouth, throat, or larynx. When mucosal surfaces are involved, the condition is often called mucous membrane pemphigoid.

In the larynx, pemphigoid may be seen most clearly on the epiglottis, though other supraglottic structures can be involved. Findings may include areas of redness, surface breakdown, blistering, or scarring. If the individual is examined serially, there seems to be migration as though areas heal and nearby areas become involved.

The primary symptom seems to be throat discomfort. Some mention soreness in the night and first thing in the morning before liquids are consumed. They can also experience voice change, difficulty swallowing due to soreness.

Because the appearance can be subtle and may change over time, diagnosis often depends on careful serial endoscopic observation, clinical history, and—when feasible—biopsy. When present, it is easier to biopsy skin lesions. If the only expression is laryngeal (epiglottic, above all), the surgeon tries to take a biopsy that includes the demarcation between normal and abnormal mucosa.

Pemphigoid is typically a long-term condition whose symptoms and lesions may wax and wane. Management focuses on controlling inflammation and suppressing the immune response, using topical or systemic medications depending on severity and extent of involvement.

Pemphigoid Affecting the Larynx

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Textbook chronic sore throat (1 of 4)

This man has a chronic low-grade sore throat. Note the ulcerated areas especially along the right side of the tip of the epiglottis. Surrounding mucosa also appears erythematous. This picture is classic for laryngeal pemphigoid and visual criteria for diagnosis are extremely strong.

Closer range (2 of 4)

At closer range under narrow band light, compare and contrast the ulcerated areas (U) with the inflammatory surround (I).

Four months later (3 of 4)

Four months later, the patient has since developed “blisters” on his back. Biopsy at that site was consistent with pemphigoid. Compare this to photo 1 to appreciate that the pattern of the lesions has changed slightly as is typical for pemphigoid.

Pattern at closer range (4 of 4)

At much closer range under narrow band light, the stippled “autoimmune” kind of vascular pattern is better seen.

Resources for Further Reading

Ahmed, A. R., Kurgis, B. S., & Rogers, R. S., 3rd (1991). Cicatricial pemphigoid. Journal of the American Academy of Dermatology, 24(6 Pt 1), 987–1001.

Bédane, C., & Doffoel Hantz, V. (2011). Pemphigoïde cicatricielle: revue de la littérature [Mucous membrane pemphigoid: a review]. Annales de dermatologie et de venereologie, 138(3), 201–208. 

Chan, L. S., Ahmed, A. R., Anhalt, G. J., Bernauer, W., Cooper, K. D., Elder, M. J., Fine, J. D., Foster, C. S., Ghohestani, R., Hashimoto, T., Hoang-Xuan, T., Kirtschig, G., Korman, N. J., Lightman, S., Lozada-Nur, F., Marinkovich, M. P., Mondino, B. J., Prost-Squarcioni, C., Rogers, R. S., 3rd, Setterfield, J. F., … Zone, J. J. (2002). The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Archives of dermatology, 138(3), 370–379.

Neff, A. G., Turner, M., & Mutasim, D. F. (2008). Treatment strategies in mucous membrane pemphigoid. Therapeutics and clinical risk management, 4(3), 617–626.

Schmidt, E., & Zillikens, D. (2013). Pemphigoid diseases. Lancet (London, England), 381(9863), 320–332.

Syal A, Lott DG, Karle WE. Radiation-Induced Laryngeal Mucous Membrane Pemphigoid. Annals of Otology, Rhinology & Laryngology. 2022;132(10):1261-1264.

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