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Everyday Practice THE NATIONAL MEDICAL JOURNAL OF INDIA VOL. 6, NO.5, 1993 221 Everyday Practice ULCERS DUE TO UNKNOWN CAUSES Oral ulcers Aphthous stomatitis The commonest of the non-traumatic oral ulcers seen in daily B. KUMAR practice are aphthous ulcers. Aphthous stomatitis is a painful and recurrent disease of the oral mucous. membrane which can occur at any age. Its prevalence has been reported to range from 20% to 60% in the general population. The ulcers begin as small, INTRODUCTION discrete or grouped papules which, in a few hours, become Most oral ulcers are caused by trauma or are aphthous ulcers. necrotizing ulcerations. They are small (average 5 mm, range However, they may also be manifestations of a wide range of 3-12 mm), round, white ulcers (aphthae) which are usually mucocutaneous disorders or systemic diseases including surrounded by a ring of hyperaemia (Fig. 1). They are tender infections, drug reactions, disorders of the blood and gastro- and may become so painful that they interfere with speech intestinal system or they may be caused by malignancy. 'Oral and mastication. Usually there are 1 to 3 lesions per attack ulcers' should, therefore, not be a final diagnosis. but the number can vary greatly. They are located mostly on The oral mucosa shows striking regional variations in the buccal and labial mucosa, edges of the tongue (Fig. 1), histology which are adaptive in nature. Broadly, there are buccal and lingual sulci and soft palate. Aphthae may also three main types. The functional mucosa of the gingiva and occur on the vagina, vulva, penis, anus and conjunctiva. the hard palate is well keratinized while the lining mucosa Healing occurs in 2 to 3 weeks but recurrences are frequent. seen in the buccal region of the lips and in the floor of the Recurrences may be induced by trauma, allergy, emotional mouth is not. Highly specialized mucosa is present on the stress, hormonal changes in women, allergy to food and dorsum of the tongue. Although these histological charac- diseases such as asthma and hay fever. A familial predisposi- teristics are not of clinical importance, the location of the tion has also been observed. Recurrences may continue for mucosal lesions may provide a clue to the diagnosis, e.g. many years but eventually remit. aphthous ulcers are seen more often on the buccal mucosa or Such ulcerations may also be the presenting feature of the tongue and rarely over the keratinized gingiva of the hard Behcet's disease, gluten-sensitive enteropathy, pernicious palate, whereas lesions due to the Herpes simplex virus are anaemia, neutropenia, ulcerative colitis and Crohn's disease. more likeiy to be seen on the keratinized gums and hard The history, physical examination, relevant investigations palate and do not usually involve the soft palate and uvula. and long term follow up documenting the recurrent course in Lesions in the mucous membranes may be more difficult the absence of other symptoms will confirm the diagnosis. A to diagnose than lesions of the skin. There is less contrast few patients have aphthous stomatitis associated with low of colour and a greater likelihood of alterations in their folate and iron levels, so the possibility of chronic blood loss original appearance because of maceration from moisture or malabsorption secondary to intestinal diseases should be and abrasions from food and tooth infection. Vesicles excluded. and bullae rapidly rupture to form greyish erosions. The Although one of the above factors may be responsible for membrane covering the papules becomes soggy and latex- recurrences, only infections or immunological causes seem to like and can easily be rubbed off to form an erosion. Group- be important. The highly pleomorphic Streptococcus sanguis ing and distribution are less distinctive of lesions in the mouth 2A strain with its traditional 'L' forms has been found to have than on the skin. It is often necessary to establish the diagnosis by the character of the associated cutaneous lesions or by observing their subsequent clinical course. When the history and clinical appearance are not helpful, smears, cultures or biopsy specimens should be examined. There is no uniformly accepted method of classification of ulcerative lesions of the oral mucosa, because many entities are ill-understood. In addition, there may be isolated or simultaneous involvement of the lips and tongue. Moreover, the presenting symptoms in many ulcerative conditions may be so similar as to defy classification based on clinical appearance alone. Although the diseases affecting the oral mucosa do not belong to any set pattern some may be grouped together. Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India FIG 1. Multiple necrotic ulcers on the border of the tongue B. KUMAR Department of Dermatology in aphthosis © The National Medical Journal of India 1993 222 THE NATIONAL MEDICAL JOURNAL OF INDIA VOL. 6, NO.5, 1993 an antigenic overlap with human oral mucosa and might damage the oral epithelium immunologically. Major aphthous ulcer (Sutton's disease) A major aphthous ulcer begins as a small shot-like nodule on the inner lip, buccal mucosa and tongue which breaks down into a sharply circumscribed area with a deeply punched out and depressed crater. As it behaves exactly like a minor aphthous ulcer in its course and recurrence, it is often considered to be a variant of the minor variety. Some patients may develop crops of painful ulcers which enlarge and coalesce to produce large, herpetiform and ragged ones. There is no definite treatment available for aphthosis. Tetracycline mouth washes, a gluten-free diet, phenelzine (normally used to treat agaraphobia), oral zinc, thalidomide, FIG 2. Extensive palatal ulceration in pemphigus vulgaris colchicine, dapsone, levamisole, cauterization with phenol or silver nitrate, corticosteroids and carbenoxolone mouth washes and vitamins have been reported to be effective. Pemphigus vulgaris Other treatments originate from observing what seems to Lesions of pemphigus vulgaris often first appear in the aggravate the condition. mouth. The short-lived bullae quickly rupture to involve Patients with frequent aphthous ulcers are treated with most of the mucosa in painful erosions (Fig. 2). The lesions applications of topical steroids, 6 times a day for 2 days. extend onto the lips and form heavy fissured crusts on the Tetracycline rinses, when started early, are effective in vermilion border. Involvement of the throat produces reducing pain and inflammation. Oral tetracyclines, erythro- hoarseness of the voice and difficulty in swallowing. The mycin and co-trimoxazole can also be given for long periods. mouth odour is very foul and unpleasant. Oral lesions can Treatment of major aphthosis is more difficult and only occur in other varieties of pemphigus as well but are most intralesional or systemic steroids are effective. Thalidomide often seen in pemphigus vulgaris. Erosions of the lips and and colchicine have also been used. buccal mucosa are sometimes seen in benign familial pemphigus (Hailey-Hailey disease). Behcet's syndrome (Oculo-oral-genital syndrome) This consists of recurrent oral aphthosis in the presence of Benign mucosal pemphigoid (cicatricial pemphigoid) any two of the following: recurrent genital ulceration, This is characterized by evanescent vesicles which occur uveitis, cutaneous vasculitis, meningo-encephalitis or mostly on the mucous membranes especially the conjunctival synovitis. In many cases oral lesions persist for many years and oral mucosa. Oral lesions occur in approximately 90% of before other signs or symptoms develop. cases and this may be the only affected site for years. Oral lesions occur on the lips, tongue, buccal mucosa, soft Desquamative gingivitis, diffuse erythema of the marginal and hard palate, tonsils and even in the pharynx and nasal and attached mucosa associated with areas of ulceration, cavity. The lesions are single or multiple, 2 to 10 mm or more vesiculation and desquamation are often the presenting in diameter and are sharply circumscribed with a dirty grey signs. The disease tends to affect middle-aged and elderly . base and a surrounding bright red halo. Eating may be women. Other portions of the oral mucosa which are difficult because of the painful nature of the lesions. A foul involved by cicatricial pemphigoid are the palate, tongue and odour from the mouth is characteristic. tonsillar pillars. Oral lesions rarely result in scarring. The Viral, immunological, coagulopathic, bacterial, genetic disease is chronic and slowly progressive and may lead to and ecological causes have been postulated but the evidence conjunctival scarring and blindness. Other mucosal surfaces is still inconclusive. Men between their second and fourth including the genital mucosa may be involved. It may some- decades are predominantly affected. times be induced by clonidine or by penicillamine. Usually the ulcers heal spontaneously but recur. Cortico- Histological findings are of a subepidermal bulla along steroids alone or in combination with thalidomide or with characteristic direct immunofluorescence showing azathioprine are effective. Levamisole, colchicine and C3 and IgG deposits in the lamina lucida. acyclovir have also been used. Mild cases respond well to potent topical steroids. For more aggressive cases systemic steroids (40-60 mg per day) BLISTERING LESIONS with or without dapsone may be effective. Vesiculo-bullous disorders are often difficult to diagnose and manage. The oral cavity is frequently overlooked
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