A case of antiepiligrin cicatricial pemphigoid with extensive cutaneous erosions mimicking pemphigus vulgaris

Abstract

Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease with predominant mucosal involvement. In this article, we report a young patient with mucosal and extensive cutaneous involvement in the form of large erosions mimicking those of pemphigus vulgaris thus leading to diagnostic dilemma. We were unable to find any other previous reports with such extensive cutaneous erosions mimicking those of pemphigus vulgaris. Laminin 5 was the antigen found on knockout substrate testing. Antiepiligrin CP is a distinct subtype of CP with antibodies against laminin 5. This subtype is mostly associated with malignancy but no underlying malignancy was found in our case. Present report also highlights the importance of knockout substrate testing when immunoblot is not available.

Keywords: dermatology; skin.

Figure 1

Well-defined erosion of size 8–10×10–25 cm in size with adherent reddish brown crusts present over the upper chest.

Figure 2

Bleeding erosions with adherent crusts present over lip extending to oral mucosa.

Figure 3

Conjunctival congestion, erosion and symblepharon formation.

Figure 4

Histopathological examination showing ulcerated epidermis and subepidermal bulla with intense fibrosis and inflammatory infiltrate comprising lymphocytes, eosinophils and polymorphs in papillary dermis.

Figure 5

Direct immunofluorescence showing intense dermoepidermal deposits of IgG and C3.

Figure 6

Indirect immunofluorescence done on salt-split normal human skin as substrate with serum 1:10 and stained with IgG showing linear band of IgG along the floor of split.

Figure 7

Indirect immunofluorescence on antigen-deficient skin substrates showing positive reactions with the basement membrane zone on type VII collagen-deficient skin.

Figure 8

Indirect immunofluorescence on antigen-deficient skin substrates showing negative findings on laminin 332-deficient skin.