genitalia, nasal mucosa, larynx, anus, and esophagus. As MMP may lead to serious complications such as blindness and airway compression, early and aggressive treatment initiation may be needed. Presentation Scarring is a common consequence of MMP that distinguishes this variant from mucosal involvement in bullous pemphigoid, which typically does not scar. Reticulated, white striations representing mucosal fibrosis often are present at sites of healed lesions, and functional limitations secondary to scarring may occur. As examples, MMP involving the ocular mucosa can lead to symblepharon, ankyloblepharon, and eventual blindness, and progressive laryngeal and tracheal involvement can result in asphyxiation. Oral disease Most commonly affecting the mouth, including the buccal mucosa, gingiva, tongue, vermillion lips, and palate. Desquamative gingivitis is the most frequent manifestation. The gingiva is erythematous, in which patients usually complaint of bleeding upon brushing. Rupturing of oral vesiculobullous lesions leave clean, noninflamed, painless erosions. The vermilion border of the lips is spared, which is typical in pemphigus. Hoarseness due to laryngeal involvement can be seen in 8% of cases. A subset of patients have only oral disease, which has a relatively benign course compared with patients with oral cavity and other mucosae and skin involvement. Ocular disease The eye is involved in 65% of cases. Initially presented with unilateral conjunctivitis (such as burning or excessive tearing), then fibrosis beneath the conjunctival epithelium. Shrinkage of the conjunctiva leads to obliteration of the conjunctival sac. Symblepharons are fibrous strands connecting the conjunctiva of the lid to the globe. Besides, reduced tearing with erosion and neovascularization of the cornea leads to corneal opacification and perforation. Scarring of the lid results in entropion (inward turning of the lid) and trichiasis (in-turning of the eyelashes). These conditions ultimately lead to blindness in approximately 20% of cases. It is crucial to go for follow-up because relapse occurs in 22% of those who were in remission and not undergoing therapy. Other mucous membranes Less common sites that might get involved are nasopharynx, esophagus, and urethra. Nasopharyngeal involvement can lead to ulcerations of the septum and airway obstruction which might require tracheostomy. Esophageal disease may present with ulcerations, dysphagia, odynophagia, and stenosis. Stenosis at urethra, vaginal orifice and rectal have also resulted from chronic inflammation and scarring. Skin disease About 25% of patients have cutaneous lesions, with tense vesicles or bullae, mainly on the face, neck, and scalp. Healing of erosion is either with or without atrophic