Cicatricial pemphigoid - A case report

Introduction

Cicatricial pemphigoid is a rare, chronic, subepithelial, autoimmune, bullous disease which mainly affects the mucous membranes, may or may not involve the skin and frequently results in conjunctival and oral mucosal scarring.[1] It is commonly seen in the elderly but has been rarely reported in children also.[2] It differs from bullous pemphigoid by its prolonged course and significant scarring. It has been rarely reported in Indian literature.[3]

Case Report′

A 60-year-old male presented with history of recurrent erosions inside oral cavity, nose and over scalp since last 6 months. He was also complaining of blood stained nasal discharge, painful lesions in both the eyes with diminished vision for the past 4 months. He had photophobia, irritable cough and had noticed a change in his voice since last two months.

Cutaneous examination revealed lesions varying from 3 mm to around 30 mm in diameter, over the scalp and nasal tip. There was crusting and scarring at places in the above areas. He had a small tense vesicle about 2 mm in diameter near the edge of erosive lesions on the scalp. Examination of oral mucosa showed multiple, erythematous, erosive lesions over gingivae, buccal mucosae, soft palate and tongue.

Left eye showed conjunctival erosions and symblepheron formation. The lens was opaque but cornea was clear. The right eye had oedema of both the lid margins, few crusted lesions on upper lid, conjunctival congestion, chemosis, symblepheron and an opaque cornea [Figure - 1]

ENT examination revealed a shallow ulcer and crusting over Little′s area and nasopharynx showed erosions. There were shallow, irregular, erythematous erosions over vestibule floor, ventral surface of the tongue, soft palate and uvula. Indirect laryngoscopy revealed a few erosive lesions involving epiglottis, vocal cords, both the arytenoids and interarytenoid region.

There were no genital or anal lesions. Systemic examination was not contributory. Haematological and biochemical parameters from vesicular lesions on scalp showed a subepidermal bulla with moderate eosinophilic infiltrate in the dermis [Figure - 2]. Immunofluorescence was not done due to lack of facility. The patient was put on tablet betamethasone 40 mg daily. Since the healing was slow, tab. dapsone 100 mg daily was added after three weeks. Methycellulose drops were used for ocular lubrication. Cataract extraction was done in the left eye. The skin and oral mucosal lesions markedly improved in 8 weeks time. However, he lost vision in his right eye.

Discussion

Cicatricial pemphigoid may remain localised to the oral cavity, the eye or the skin (Brunsting - Perry variety) or it may be generalised. Early recognition and treatment can improve prognosis and avoid surgical intervention.[4]

Oral involvement is the most consistent feature of CP, while ocular involvement occurs in 61 to 89 % of patients. Mucosal surfaces of pharynx, nasal mucosa, rectum and oesophagus can also be affected. Cutaneous involvement may be seen in 1043 % of patients.[5] Sometimes CP may present with features of desquamative gingivitis. It may resemble pemphigus vulgaris and oral lichen planus, it may have to be differentiated also from other causes of conjunctival scarring like erythema multiforme, trauma and chronic use of topical ophthalmic ointments. History, clinical examination, histopathology and immuno-fluorescence are helpful in diagnosis. The present case was diagnosed on the basis of classical clinical features and histopathology.

Pathogenesis of CP is not well understood. However, there is circumstantial evidence to suggest its autoimmune nature. Deposition of immunoglobulins and complement at basement membrane zone (BMZ) in vivo, and presence of circulating anti BMS antibodies have been demonstrated.[6],[7]

Most patients require systemic steroids. Dapsone, intralesional steroids and sometime immunosuppressive drugs have also been used.[4] Our patient responded well to oral steroids and dapsone.