Oral submucous fibrosis: An overview of a challenging entity.

The oral cavity is considered to be a mirror of the body's health, as it reflects the manifestations of various systemic disorders. Most of the oral mucosa is derived embryologically from an invagination of ectoderm and thus, like other similar orifices, it may become involved in the disorders that are primarily associated with the skin. Oral submucous fibrosis is one of the commonest precancerous conditions of the oral mucosa involving any part of the oral cavity resulting in tissue scarring, dysphagia and trismus. It is a collagen-related disorder characterized by excessive fibrosis in the oral submucosa, hyalinization and degenerative changes in the muscles. This disease has become a challenging entity for dermatologists due to resemblance of its features to various mucocutaneous conditions. An improper diagnosis can lead to wrong treatment and additional complications. Dermatologists need to be aware of the characteristic features of this disease which can distinguish it from other similar conditions. This review aims to focus on the detailed aspects of oral submucous fibrosis including its historical background, etiological factors, pathogenesis, clinical features, differential diagnosis, investigations, management and future perspectives.

Proliferative verrucous leukoplakia: An update.

Proliferative verrucous leukoplakia (PVL) is a rare form of oral leukoplakia, which was first described in 1985 by Hansen et al. Since then, various published case series have presented PVL as a disease with aggressive biological behavior due to its high probability of recurrence and a high rate of malignant transformation, usually higher than 70%. PVL is a long-term progressive condition, which is observed more frequently in elderly women, over 60 years at the time of diagnosis. The buccal mucosa and tongue are the most frequently involved sites. It develops initially as a white plaque of hyperkeratosis that eventually becomes a multifocal disease with confluent, exophytic and proliferative features with a progressive deterioration of the lesions, making it more and more difficult to control. Tobacco use does not seem to have a significant influence on the appearance or progression of PVL and may occur both in smokers and nonsmokers. Prognosis is poor for this seemingly harmless-appearing white lesion of the oral mucosa. At present, the etiology of PVL remains unclear as well as its management and diagnosis, which is still retrospective, late and poorly defined, lacking consensus criteria. This short review discusses the clinical and histopathological features, diagnosis, traditional treatment and the current management of the disease.

Epidermodysplasia verruciformis: an unusual malignant transformation.

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Granulomatous mycosis fungoides with large cell transformation misdiagnosed as leprosy.

BACKGROUND: Granulomatous mycosis fungoides is an unusual histopathological variant of cutaneous T-cell lymphoma without clinical distinction from classic mycosis fungoides. Symptoms associated with peripheral nerve involvement have rarely been reported in the literature. CASE REPORT: The authors described a case of granulomatous MF stage IIB with large cell transformation who initially presented with leprosy-like condition and chronic left peroneal neuropathy The patient received six courses ofgemcitabine with greater than 90% improvement of skin lesions. The rest of the lesions were successfully treated with local electron beam radiation. CONCLUSION: Granulomatous MF with neuropathy can be clinically misdiagnosed if there is no histopathological and immunohistochemical finding to support the diagnosis of lymphoma.

Marjolin's ulcer: the importance of surgical management of chronic cutaneous ulcers.

Chronic cutaneous ulcers are commonplace in the developing world, especially in rural areas with poor living conditions and often result from the trauma of road-traffic injuries. Chronic cutaneous ulcers may also be due to vascular insufficiency, neuropathy, nodular leprosy, pressure, diabetes, or hemoglobinopathies, or they may be tropical ulcers. If poorly managed, these lesions may undergo malignant transformation. We evaluated the clinical histories and treatment outcomes of patients seen at the University of Calabar Teaching Hospital, between January 2000 and December 2004, who had histologic diagnosis of Marjolin's ulcer, in an attempt to identify risk factors for this problem. The six patients were men, age 30-70 years (mean, 42 years). Trauma was the leading cause of injury leading to ulceration: road-traffic accidents (four patients, 66.7%), fall (one patient, 16.7%), and flame burn (one patient, 16.7%). Most injuries involved the limbs: lower (four patients, 66.7%) and upper (one patient, 16.7%). The histologic diagnosis in all the cases were squamous cell carcinoma and mean latency period from injury to diagnosis of malignancy was 18.5 years. All the patients had been admitted because of poor results from topical treatment. Three patients (50%) were managed with wide excision and skin grafting with the lesions healed. Ignorance as well as economic and sociocultural factors were the underlying issues. Education concerning the risks associated with chronic wounds and the need for prompt and proper surgical management are recommended.

[Squamous cell carcinoma arising from chromomycosis. Report of two cases]. / Carcinome épidermoïde développé sur des lésions de chromomycose. A propos de deux observations.

Madagascar is the principal area of endemic chromomycosis in the world with 1 400 cases registered from 1955 to 1996. Malignant transformation is rare and only 12 cases have been reported. We describe herein two new cases of squamous carcinoma arising from chronic lesions of chromomycosis. These carcinomas which develop slowly and are non invasive, are similar to those arising from burns, tropical phagedenic ulcers and chronic leprosy ulcers. Epidemiological aspects, histopathology and treatment are described.

Neoplastic transformation of chronic ulcers in leprosy patients--a retrospective study of 23 consecutive cases.

A retrospective analysis of chronic ulcers among leprosy patients seen over the last 20 years yielded 23 cases of neoplastic transformation. It showed a peak at the sixth decade, an incidence of 3.66/100 among hospitalised ulcer cases and male/female ratio of 1.6:1. Borderline tuberculoid was the most common type of leprosy involved (40%). Squamous cell carcinoma was the most common neoplasia. Its usual site was plantar ulcers. Heel ulcers showed relatively greater predeliction for malignancy (38.5%). Histopathological proof of malignancy is desirable and that may require multiple biopsies. Metastasis is rare but potentially fatal. The surgical treatment must provide a functional, trouble-free limb. Forefoot or Lisfranc's amputation for distal third ulcers and below-knee amputation for large midfoot and ulcers are procedures of choice. Wide excision may be used in select cases.

[Squamous cell carcinoma in neuropathic plantar ulcers in leprosy: another example of Marjolin's ulcer]. / Plaveiselkarsinoom in neuropatiese plantare ulkusse van leprose: nog 'n voorbeeld van Marjolin se ulkus.

Seven cases of squamous cell carcinoma (SCC) arising in chronic neuropathic plantar ulcers of leprosy are described. These patients (average age 59 years) presented over a 5-year period. The mean duration of neuropathic ulceration until diagnosis of SCC was 24.5 years. Six patients required limb amputations and 3 underwent lymphadenectomy for involved nodes. One patient died of disseminated disease. The history of the eponym "Marjolin's ulcer' is traced and a case put forward for recognition of malignant change in neuropathic ulcers as yet another example of Marjolin's ulcer. A plea is made for an increased awareness of the possibility of malignant transformation in chronic neuropathic ulcers in order to effect an early diagnosis of a potentially aggressive cancer. This is the first report of its kind in South Africa. Similar cases, however, have been reported from other parts of the world where leprosy is endemic.

Malignant transformation of plantar ulcers in leprosy.

Malignant transformation of plantar ulcers in leprosy is not uncommon. The apparent rarity of these neoplasms could be because many observed cases are not reported. To determine the extent of the problem, 133 consecutive cases of plantar ulcers seen over two years were studied clinically as well as histologically. Plantar ulcers were more common in the distal third of foot (64.67%) but malignant transformation was seen more often in plantar ulcers of proximal third of foot (64.29%). Malignant transformation was more common in plantar ulcers of long duration. Histologically, most of the lesions were benign, being instances of pseudo-epitheliomatous hyperplasia (57.89%) or atypical pseudo-epitheliomatous hyperplasia (13.53%). However, squamous cell carcinoma was observed in 10.53% cases. Thus it may be that more cases with this complication will be detected if it is borne in mind that malignant change may be encountered in such ulcers.

Malignant transformation of trophic ulcer in leprosy.

Malignancy developing in the trophic ulcer of leprosy is no more a rarity. In four years period we detected seven cases of squamous cell carcinoma developing in the trophic ulcer in patients with lepromatous leprosy.

Malignant transformation in trophic ulcers in leprosy: a study of 12 cases.

Twelve cases of carcinomata arising in Trophic ulcers of Leprosy are presented. Out of these, 10 were on the plantar surface more commonly on the proximal part of foot, one on lower leg and dorsum of foot, and one in an ulcer over the lateral malleolus. Almost all presented with infected growths and regional lymphadenopathy. Three cases presented with advanced disease with fungating inguinal nodes and were fatal. Nine cases underwent below knee amputation under antibiotic cover as a definitive treatment and the lymph nodes were kept under observation. Histologically, all were low grade squamous cell carcinomas. In most cases lymph nodes regressed after removal of primary and in one case lymph nodes were positive for malignancy. This study was conducted at Dr. Bandorawalla Leprosy Hospital, Kondhawa, Pune 22 from the year 1981 to 1987.