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Home , Noma (disease)

MINISTRY OF HEALTH OF UKRAINE Ukrainian medical stomatological academy

“Approved” On the meeting chair Of Propaedeutics Surgical Stomatology The Head of the Department prof. ovikov V.M. ______“ ____ ” ______20 ____

GUIDELINES Individual work of students

Educational discipline Surgical stomatology Module № 2 Inflammatory in maxillofacial region. Nonodontogenous inflammatory diseases in Content module № 4 maxillofacial region Noma. Etiology, pathogeny, clinic, diagnostics, Theme lesson medical treatment. Vegener’s Syndrome, signs of AIDS in the oral cavity. Course 3 Faculty Stomatological

Poltava 2018 1. Actuality of the topic: Noma, also known as cancrum oris and gangrenous , is a rare of childhood that is characterized by a destructive process of orofacial tissues. The mechanism by which predisposing factors allow the microorganisms to become virulent pathogens is not understood. Wegener's granulomatosis is an inflammatory condition of unknown etiology. Efforts to identify a cause have generally focused on infection and immunologic dysfunction but have been unproductive. AIDS is the terminal stage of infection with the human immunodeficiency virus (HIV), which is recognized as undergoing a number of mutations. The underlying severe immunodeficiency leads to a number of oral manifestations which, although not pathognomonic, should raise the possibility of HIV infection. 2. The objectives of the studies: Etiology, pathogens, pathological anatomy, classification, features of a clinical course, methods of diagnostics noma, Vegener’s syndrome and AIDS. To be able: to establish the diagnosis of noma, Vegener’s syndrome and AIDS.

3. Basic knowledge, skills, skills necessary for study topics (interdisciplinary integration). Name of previous courses These skills Microbiology Agents who produce above named diseases, their property. Pathological anatomy An etiology, pathogens, of noma, Vegener’s syndrome and AIDS. Pathophysiology A pathogenesis of above named diseases. Pharmacology Drug for the treating of noma, Vegener’s syndrome and AIDS. Propedeutics of a surgical Methods examination of patients stomatology

4. Tasks for independent work in preparation for the classes. 4.1. A list of key terms, parameters, characteristics that must learn the student in preparation for the lesson:

Term Definition Noma also known as cancrum oris and gangrenous stomatitis, is a rare disease of childhood that is characterized by a destructive process of orofacial tissues Wegener is a rare multisystem autoimmune disease of unknown etiology. granulomatosis (WG) Human Acquired immunodeficiency syndrome (HIV/AIDS) is a disease of the immunodeficiency human immune system caused by the human immunodeficiency virus virus infection (HIV)

4.2. Theoretical questions to lesson: 1. Give definition of of noma, Vegener’s syndrome and AIDS. 2. The etiology and pathogen of noma. 3. Clinic of noma. 4. Complex medical treatment of noma. 5. Medical treatment of noma. 6. Possible complications of noma and Vegener’s Syndrome. 7. The etiology and pathogen of Vegener’s Syndrome. 8. Clinical motion of Vegener’s Syndrome erysipelas. 9. Diagnostics is that differential diagnostics of noma, Vegener’s syndrome and AIDS. 10. Medical treatment and prophylaxis of Vegener’s syndrome. 11. The etiology and pathogen of AIDS. 12. Clinic of AIDS. 4.3. Practical works (tasks) are performed in class: − To take possession of technique of realization observation of the patients with noma, Vegener’s syndrome and AIDS. − To make a treatment planning of noma, Vegener’s syndrome and AIDS. − To acquire technique of carrying out observation of patient with noma, Vegener’s syndrome and AIDS. − To make the plan of examine of the patient with noma, Vegener’s syndrome and AIDS. − To make the plan of local treatment of patients with noma, Vegener’s syndrome and AIDS.

5. Theme contents: NOMA Etiology and Pathogenesis. Noma, also known as cancrum oris and gangrenous stomatitis, is a rare disease of childhood that is characterized by a destructive process of orofacial tissues. Necrosis of tissue occurs as a consequence of invasion by anaerobic bacteria (fusiform bacilli and Vincent's spirochetes) in a host whose systemic health is significantly compromised. Malnutrition is the most frequently cited predisposing factor, although debilitation from systemic disease, such as pneumonia or sepsis, has been described. The mechanism by which predisposing factors allow the microorganisms to become virulent pathogens is not understood. Noma shares many features with the more limited and more benign acute necrotizing ulcerative (ANUG, or Vincent's infection). Both are caused by the same organisms, both require compromised hosts, and both result in tissue necrosis. Noma is rarely seen in the United States. It is typically found in relatively underdeveloped countries, especially those in which malnutrition or protein-deficient states are prevalent. Clinical Features. The initial lesion of noma is a painful ulceration, usually of the gingiva or buccal mucosa, that spreads rapidly and eventually necrotizes. Denudation of the involved bone may follow, eventually leading to necrosis and sequestration. Teeth in the affected area may become loose and may exfoliate. Penetration of organisms into the cheek, , or may also occur, resulting in fetid necrotic lesions. Before antibiotics were developed, fatalities from this disease were common. Treatment. Therapy involves treating the underlying predisposing condition as well as the infection itself. Therefore, fluids, electrolytes, and general nutrition are restored, along with the introduction of antibiotics (usually penicillin). Debridement of necrotic tissue may also be beneficial if there is extensive destruction. WEGENER'S GRANULOMATOSIS Etiology. Wegener's granulomatosis is an inflammatory condition of unknown etiology. Efforts to identify a cause have generally focused on infection and immunologic dysfunction but have been unproductive. Clinical Features. Typically, the triad of upper respiratory tract, lung, and kidney involvement is seen in this condition. Occasionally, only two of the three sites are affected. Lesions may also present in the oral cavity and skin and, potentially, in any other organ system. The basic pathologic process that is common to all foci is necrotizing vasculitis with granuloma formation. This is a rare disease of middle age. Initial presentation often occurs with head and neck symptoms. Symptoms typical of sinusitis, rhinorrhea, nasal stuffiness, and epistaxis may be seen with or without non-specific complaints of fever, arthralgia, and weight loss. In a majority of cases, nasal or sinus (usually maxillary) involvement is seen and is often present early in the course of the disease. Destructive lesions are typically ulcerated, with necrosis and perforation of nasal septum occasionally seen. Perforation of the hard palate is uncommonly seen in Wegener's granulomatosis. Intraorally, red granular gingival lesions have been reported. The process is generalized and results in relatively uniform enlargement. Most patients have kidney involvement that consists of a focal necrotizing glomerulitis. Renal failure is the final outcome of kidney disease. Inflammatory lung lesions, varying in intensity from slight to severe, may eventually lead to respiratory failure. Histopathology. The basic pathologic process is granulomatous, with necrotizing vasculitis usually present. Variable numbers of acute and chronic inflammatory cells are seen in the granulomatous zones. Necrosis and multinucleated giant cells may be seen in the granulomatous areas. The affected small vessels show a mononuclear infiltrate within their walls in the presence of fibrinoid necrosis. Definitive diagnosis in the absence of vascular changes is difficult, since the microscopy would be nonspecific. Diagnosis. Diagnosis is generally dependent upon the finding of granulomatous vasculitis in biopsy tissue of upper respiratory tract lesions, evidence of involvement of lung, or kidney lesions. Development of labeled antineutrophil cytoplasmic antibodies (ANCA) has provided serologic correlation with biopsy-proven vasculitis. On both a clinical and a microscopic level, chronic infectious processes, such as TB, , histoplasmosis, and blastomycosis, and neoplastic processes, such as and undifferentiated squamous cell carcinoma, might be serious considerations in a differential diagnosis of this disease. Culture and tissue identification of microorganisms, when negative, would help rule out infectious processes. Immuno-histochemical staining or immune-fluorescent procedures could be used to help rule out neoplasia, especially on frozen sections. Treatment. Before the development of chemotherapeutic agents, renal failure and death were frequent outcomes of this disease process. The use of the cytotoxic agent cyclophosphamide and corticosteroids has provided patients having this disease with a relatively good prognosis. Remissions are seen in approximately 75% of cases. ORAL MANIFESTATIONS OF HIV INFECTION AND AIDS AIDS is the terminal stage of infection with the human immunodeficiency virus (HIV), which is recognized as undergoing a number of mutations. The underlying severe immunodeficiency leads to a number of oral manifestations which, although not pathognomonic, should raise the possibility of HIV infection. It is virus infections caused by Human Immune Deficiency Virus (HIV) Over 80% of the reported cases were male homosexual. Other groups affected includes intravenous drug abusers and recipient of infected or contaminated blood products. Mortality is high (100%) within 2 years of onset. They have been classified. Group I. Strongly associated with HIV: Candidosis seen in 60% of HIV patients as early manifestation. Erythematous (early), hyperplastic, pseudomembraneous (late), and angular in young people (most common oral feature of HIV). Hairy Bilateral, white, non-removable corrugated lesions of the tongue, unaffected by antifungal but usually resolve with acyclovir or valacyclovir, and associated with EBV. It is a predictor of bad prognosis and possible development of lymphoma. HIV gingivitis Unusually severe gingivitis for the general state of the mouth. Often characterized by , intense red band along gingival margin. Acute ulcerative gingivitis. Occurs in young, otherwise healthy mouths. HIV periodontitis Severe localized destruction out of place with OH. Kaposi's sarcoma (K.S) Commonest malignancy among HIV patients. Rx: radiotherapy is effective. One or more erythematous purplish maculas or swelling, frequently on the palate. 50% occur intra- or perorally. Non-Hodgkin's lymphoma Similar to the above. Less common. Group II. Less strongly associated with HIV: Atypical oropharyngeal ulceration Idiopathic thrombocytopenic purpura. HIV associated HIV children and adults. Similar to Sjogren’s syndrome. Wide range of common viral infections. Group III. Possible association with HIV Wide range of rare bacterial and fungal infections. Cat scratch disease. Neurological abnormalities. Osteomyelitis, sinusitis, submandibular cellulitis. Squamous carcinoma. Clearly, the conditions in Group III are likely to be seen in patients who are HIV -ve at least as often as in patients who are HIV +ve. Persistent generalized lymphadenopathy Otherwise inexplicable lymph-adenopathy > 1 cm persisting for 3 months, at two or more extra inguinal sites. Cervical nodes particularly commonly affected. May be prodromal or a manifestation of AIDS. Rx for AIDS AIDS is currently incurable, but then again so is life. Antiretroviral drugs prolong and improve quality of life in those with active AIDS. Rx with antiretroviral agents: AZT, didanosine (DDI), dideoxycytidine (DCC), HIV protease inhibitors, e.g. nevirapine or delavirdine, are effective in controlling the II in CD4 cells. Early detection and Rx of opportunistic infections and neoplasms also has a major impact on quality of life. Dental Rx for patients with AIDS. This group present two risks with regard to dental Rx. 1. To personnel carrying out the Rx. Affected patients carry an infectious disease with no known cure, which is transmitted by blood and blood products. As it is impossible to adequately identify all such patients, routine cross-infection control is now a necessity. 2. As these patients are immunocompromised, any Rx with a known risk of infective complications, e.g. extractions, should be covered with antiseptic and antimicrobial prophylaxis, and any surgery should be as atraumatic as possible. There may also be a slight tendency to bleeding in these patients, and local haemostatic measures may be needed. Clinical Features: 1. High pyrexia. 2. Sudden loss of weight. 3. Generalized lymphadenopathy. 4. Pneumonia, in particular, pneumocystosis carinii pneumonia is very common in AIDS. 5. Malignancy, in particular, Kaposi's sarcoma affecting the head and neck region in more than 30% of cases. Prophylaxis after contaminated needlestick injury, e.g. hollow point needlestick from AIDS patient. Combination therapy offers best chance of preventing HIV seroconversion. Dental Considerations: Dental professionals should be aware of this appalling syndrome and be able to recognize early the oral manifestations of the syndrome, thereby avoiding the possibility of cross infection and facilitating isolation and treatment in the appropriate centers. In dental practice, the special precautions identical with positive viral hepatitis patients should be taken which includes: 1. Accidental wounds from contaminated instruments with blood, saliva or exudates must be carefully avoided. 2. Use of disposable instruments and other equipments is encouraged and discarded in disinfectant containers. 3. Use of gloves, masks and eye glasses. 4. Surgical instruments and forceps’s should inadequately sterilize. 5. All blood and biopsy specimens should be labelled with AIDS precautions. 6. Proper hand washing before and after oral examination, treatment or cleaning of instruments is mandatory.

6. Materials for self control: А. Assignments for self Recommendations Comment control (tables, charts, drawings, graphs) Main task To acquire procedure of To carry out in such sequence: To pay attention to the common examine of the patient. 1. Interrogation of the patient state of the patient, presence of (the complaint, an anamnesis pathological changes of a skin of disease). of the face. 2. Survey of the patient, a palpation, a percussion, probe. To carry out observation of During examine to reveal To pay attention to correctness the patient. attributes of noma, Vegener’s of filling of a card of the out- syndrome and AIDS. patient patient. To appoint treatment. To prove a choice of a method local treatment of noma, Vegener’s syndrome and AIDS.

B. Self-control tests: 1. Syndromes and diseases Strongly associated with HIV: A. Candidosis, acute ulcerative gingivitis. B. of skins. C. Furuncle. D. Squamous carcinoma. E. Cat scratch disease. 2. The causative agent of noma is: A. Staphylococci. B. . C. S. Spirochete. D. D. Fungi. E. Anaerobic microorganisms. 3. Non-Hodgkin's lymphoma: A. Sign of the Vegener’s Syndrome. B. Sign actinomycosis. C. Sign of noma. D. Strongly associated with HIV. C. Tasks for self-control: Problem № 1. Patient Z. 25 years, over a period 1,5 year has disorders on the in the oral cavity. Objectively: intraorally appears as distinct white patches that can be easily rubbed off with gauze to expose an underlying red, raw surface. Question. 1. To make Diagnose. 2. Name the clinical forms of this disease. 3. To make differential diagnose. 4. What medical treatment of this patient?

7. Bibliography. Basic: 1. Contemporary Oral and Maxillofacial Surgery//Larry J. Peterson, Edvard Ellis III, James R.Hupp, Myron Tucker/ 2003, MOSBY, – 776 p. Additional Sources: 1. Stulberg, Daniel and Penrod, Marc. "Caring for Common Skin Conditions: Common Bacterial Skin Infections." American Family Physician 66. (July 1, 2002): 119-24. 2. Tavelli Bert. "Infectious Diseases of the Skin." Noble: Textbook of Primary Care Medicine, 3rd ed. New York: Mosby, 2001. 787-788. 3. Wilkerson Michael. "Baterial Disaeses of the Skin." Rakel: Conn's Current Therapy, 54th ed. New York: Saunders, 2002. 816. 4. http://intranet.tdmu.edu.ua/data/kafedra/theacher/stomat_hir/sh_osypko/English/Lectu res/Surgical%20dentistry//3%20year/Abscess%20and%20phlegmon%20of%20jaw %20-facial%20area%20.conception%20,%20classificaton%20,%20ethiology%20,%20pathogen

Methodical Instruction is composed by docent Pan’kevych A.I.