Introduction
Lichen sclerosus is a chronic, benign, inflammatory dermatosis of the skin and mucous membranes. This condition most commonly affects the female genitalia and usually presents as white plaques on the affected region.Reference Jensen, Worsaae and Melgaard1–Reference Mendonca, Ribeiro-Rotta, Silva and Batista3 Although the condition mainly affects genital skin and mucosa, extra-genital lichen sclerosus, and even rare oral presentations, have been reported.Reference Jensen, Worsaae and Melgaard1, Reference Meffert, Davis and Grimwood4, Reference Araujo, Orsini, Marcucci and Araujo5
The condition is often described as lichen sclerosus et atrophicus in the dermatological literature, but is also known as balanitis xerotica obliterans and kraurosis vulvae in penile and vulvar presentations, respectively.Reference Brown, Dunlap, Bussard and Lask2, Reference Meffert, Davis and Grimwood4
Lichen sclerosus et atrophicus affecting only the oral mucosa is extremely rare.Reference Jensen, Worsaae and Melgaard1, Reference Mendonca, Ribeiro-Rotta, Silva and Batista3, Reference Araujo, Orsini, Marcucci and Araujo5
We describe a case of lichen sclerosus et atrophicus of the tonsil, which presented as a painful, enlarging, exophytic lesion mimicking a squamous cell carcinoma.
Case report
A 70-year-old man of Asian origin presented with a six-week history of sore throat and dysphagia. He also complained of a globus sensation. He was an ex-smoker, having ceased 28 years ago, and denied any alcohol consumption. He had significant co-morbidities, including ischaemic heart disease leading to left ventricular failure and ultimately requiring coronary artery bypass grafts, insulin-dependent diabetes mellitus, syphilis, chronic renal impairment, a permanent pacemaker, peripheral neuropathy, fistula-in-ano, and previous liver resection for hepatocellular carcinoma.
On examination, a large, exophytic lesion involving the entire right tonsil was noted, which was white in appearance and hard and very tender on palpation. Examination was otherwise normal.
The patient underwent a barium swallow procedure which showed normal mucosa and motility throughout, with no delay or obstruction. A computed tomography scan some months earlier had shown no evidence of any tonsillar or neck abnormality.
The patient underwent direct laryngoscopy, pharyngoscopy and oesophagoscopy, with biopsy of the right tonsil. Other than the abnormal tonsil, the appearances were entirely normal. The patient subsequently underwent a tonsillectomy, and made a full recovery with complete resolution of his symptoms.
Pathology results
Histological examination of the biopsy verified vacuolisation at the dermo-epidermal interface and homogenisation of the immediate sub-epidermal zone with an underlying band-like lymphocytic infiltrate, as demonstrated in Figure 1. The characteristic homogenised sub-epidermal zone and the ill-defined, vacuolised epidermal basal layer are demonstrated in Figure 2. Further typical appearances of the homogenised sub-epidermal zone and the underlying, band-like lymphocytic infiltrate are demonstrated in Figure 3.
Discussion
As previously mentioned, lichen sclerosus most commonly affects the genital skin and mucosa. Extra-genital lichen sclerosus has been reported, and sites include the lip, buccal mucosa, gingiva, palate, wrist and ankle.Reference Jensen, Worsaae and Melgaard1–Reference Mendonca, Ribeiro-Rotta, Silva and Batista3, Reference Araujo, Orsini, Marcucci and Araujo5–Reference Corbalan-Velez and Perez-Ferriols7 However, oral presentations are generally extremely rare.
The cause of lichen sclerosus is unknown, although a number of aetiologies have been proposed.Reference Brown, Dunlap, Bussard and Lask2, Reference Buajeeb, Kraivaphan, Punyasingh and Laohapand6, Reference Golusin, Jovanovic, Vujanovic and Subotic8 These include an autoimmune-related disorder (similar to thyroiditis, vitiligo and insulin-dependent diabetes mellitus), an infectious origin, and an isomorphic phenomenon occurring in areas of friction irritation.Reference Brown, Dunlap, Bussard and Lask2, Reference Dickie, Horne, Sutherland, Bewsher and Stankler9
The prevalence of the condition is unknown. Women have been reported to be affected six to 10 times more frequently than men, with female genital cases making up the bulk of reports.Reference Jensen, Worsaae and Melgaard1, Reference Brown, Dunlap, Bussard and Lask2, Reference Arican, Ciralik and Sasmaz10
Lichen sclerosus et atrophicus of the oral cavity is extremely rare.Reference Mendonca, Ribeiro-Rotta, Silva and Batista3 Lichen sclerosus involving the tonsil has not previously been described in the literature, to our knowledge. Our literature search identified only 10 previously reported cases of microscopically verified oral lichen sclerosus. These cases also had no associated skin or genital lesions. All were reported in Caucasians, six of whom were female and the rest male.Reference Jensen, Worsaae and Melgaard1–Reference Mendonca, Ribeiro-Rotta, Silva and Batista3, Reference Araujo, Orsini, Marcucci and Araujo5, Reference Buajeeb, Kraivaphan, Punyasingh and Laohapand6, Reference Ravits and Welsh11–Reference Schulten, Starink and Van Der Waal13
Oral cases of lichen sclerosus et atrophicus, as well as many genital cases, are asymptomatic except for pruritus and cosmetic effects.Reference Jensen, Worsaae and Melgaard1, Reference Meffert, Davis and Grimwood4 An increased risk of squamous cell carcinoma may exist in vulvar disease,Reference Meffert, Davis and Grimwood4 but lichen sclerosus et atrophicus is not in itself a premalignant condition.Reference Brown, Dunlap, Bussard and Lask2, Reference Mendonca, Ribeiro-Rotta, Silva and Batista3
Management options range from no active treatment to the use of topical and intralesional corticosteroids, topical testosterone, and excision of the lesion.Reference Jensen, Worsaae and Melgaard1–Reference Mendonca, Ribeiro-Rotta, Silva and Batista3, Reference Araujo, Orsini, Marcucci and Araujo5, Reference Buajeeb, Kraivaphan, Punyasingh and Laohapand6
The presentation of our patient differs from that of previously reported oral lichen sclerosus cases, in that our patient was male and of Asian origin; these factors, along with the patient's unique presentation, are interesting observations.
The histological features of the current case included a homogenised sub-epidermal zone and an ill-defined, vacuolised epidermal basal layer, as demonstrated in Figures 1 to 3.
Photomicrograph of biopsy, showing vacuolisation at the dermo-epidermal interface and homogenisation of the immediate sub-epidermal zone, with an underlying, band-like lymphocytic infiltrate (low power; ×10).
Higher power photomicrograph of biopsy, showing characteristic homogenised sub-epidermal zone and ill-defined, vacuolised epidermal basal layer (high power; ×40).
Photomicrograph of biopsy, showing typical appearances of the homogenised sub-epidermal zone and the underlying, band-like lymphocytic infiltrate (high power; ×40).
Our literature review established that the prognosis of lichen sclerosus is excellent; no recurrence or development of new lesions has been reported upon follow up.Reference Brown, Dunlap, Bussard and Lask2, Reference Mendonca, Ribeiro-Rotta, Silva and Batista3, Reference Buajeeb, Kraivaphan, Punyasingh and Laohapand6
Conclusion
Our patient illustrates a rare case of oral lichen sclerosus et atrophicus; to our knowledge, this case represents the first report of tonsillar involvement of the condition. It is important to remember that unusual pathology can mimic squamous cell carcinoma; in this respect, this case presented a diagnostic challenge.
