More than 70% percent of patients with Human Immunodeficiency Virus (HIV) infection will at some stage present with a Head and Neck or Ear, Nose and Throat manifestation, many of them with multiple lesions (1,2,3,4). There are no Acquired Immune Deficiency Syndrome (AIDS) – defining conditions specific to the Head and Neck, but many AIDS-defining lesions do manifest in the Head and Neck and a wide variety of problems associated with HIV infection are also commonly seen. In addition, there are a number of lesions which if found are indications for offering HIV testing to a patient. A thorough understanding of these HIV related problems is essential to facilitate early diagnosis as well as comprehensive and appropriate care of the HIV-infected person. This chapter discusses the common manifestations of HIV disease in the head and neck region. For clarity, the topic is approached by considering the nature of pathologies (cutaneous and mucosal lesions, inflammatory and infective conditions, neoplasms, and neurologic damage) and the sites affected (skin and face, nose and paranasal sinuses, external, middle, inner ear and skull base, oral cavity, salivary glands, pharynx, larynx, upper oesophagus, and the neck). It is to be noted, however, that there is a considerable overlapping of categories by several of the lesions.
Cutaneousand Mucosal Lesions Cutaneousdisorders are very commonly encountered in HIV-infected patients. Up to 90% ofpatients suffer from skin diseases during their course of illness and skindisorders may be the first manifestation of HIV disease (5). The spectrum ofthese disorders is wide and includes skin infections, inflammatory conditions,cutaneous malignancy and miscellaneous manifestations including drug reactions.In theory, any of the skin manifestations may be present in the head and neck, butthe actual manifestation is determined by the immunologic state, concurrent useof medication and the pattern of infections in the locality, and the number anddegree of manifestations worsens with worsening immunity (5,6,7). Thus, whilehead and neck cutaneous manifestations can be seen even early, the occurrenceand number of lesions increase with advancing disease.
Common head and neckcutaneous lesions include candidiasis, recurrent aphthous ulcers, Kaposi’ssarcoma, oral hairy leucoplakia, molluscum contagiosum, herpes simplex, herpeszoster (Shingles), psoriasis, seborrheic dermatitis and mucosal dryness fromsalivary gland disease as shown in table 1. The management of skin disease isimportant for cosmetic reasons, self-esteem and quality of life issues. Evenminor conditions should not be overlooked and the dermatologist is in the bestposition to manage these lesions especially those that are refractory to normaltreatmentInflammatory and infective conditionsAsexpected in immunocompromised states, infection is common in the Head and Neckand they could be life-threatening.
The entire spectrum of infective diseasescan he found: viral (HSV 1, Varicella Zoster, Cytomegalovirus), bacterial(usually caused by expected organisms for various infections, but tuberculousand non-tuberculous mycobacteria are common), fungal (Candidiasis,Aspergillosis, Cryptococcosis Histoplasmosis Coccidioidomycosis) and parasitic(Toxoplasma). Usually, infections in the various tissues are caused by thepathogens expected in patients with a normal immune system, though they tend tooccur more frequently and run a more severe course. Treated promptly, themajority of patients respond to standard medical management. Unusual organismsare however found in the later stages of disease as are unusual opportunisticinfections such as those caused by mycobacteria, fungi and parasites.NeoplasmsKaposi’ssarcoma and non-Hodgkin’s lymphoma are famously associated with HIV disease.
Kaposi’sSarcoma is an idiopathic multiple sarcoma and is the commonest tumour in HIVinfection (1,8). It is an AIDS-defining cancer and can manifest even early inthe course of the disease. It may manifest as multiple synchronous tumours inthe body and there may be more than one tumour arising from the skin or mucosalsurfaces of the head and neck. Non-Hodgkin’s lymphoma usually appears late inthe course of HIV disease and presents with fever, night sweats and weight lossassociated with a mass. Squamous cellcarcinomas have also been reported found arising from the epithelia in the headand neck of HIV patients.
The incidence is not clear, and association with HIVcontroversial. However, such tumours have been found to be very aggressivedespite highly active antiretroviral therapy and need to be promptly andaggressively treated (9). NeurologicdamageHeadand Neck neurologic damage is most commonly in the form of a seventh cranialnerve palsy (1). Damage to the facial nerve is more common in HIV-infectedpatients than in immunocompetent individuals (10). It can be a manifestation ofcentral nervous system disease in the so-called Facial Nerve/ Central NervousSystem Facial-Paralysis Syndrome or it may be an idiopathic (or Bell’s palsy)believed to be due to an infection of the nerve in the facial canal by theherpes simplex virus (.
1,10). Central nervous system disease causing an uppermotor neuron facial nerve palsy has been reported from CNS toxoplasmosis, HIVencephalitis and CNS lymphoma (1). The palsy may be unilateral or bilateral. CNSdisease must be promptly treated, but even so, the prognosis for full recoveryof nerve function is poor. In cases of Bell’s palsy, the paralysis is a lowermotor neuron type and prompt treatment with a course of oral prednisolone andacyclovir commenced within the first two weeks of onset (the earlier, thebetter) is the standard treatment and most patients recover full functionwithin three to four months (11,12)Skinand Face: FungalinfectionsFungalinfection predominates due to its opportunistic nature.
It is the most commonskin disorder found among HIV positive patients and occurs very frequently onthe face presenting most commonly as Dermatophytosis and Candidiasis. Othercommon skin fungal infections include Aspergillosis, Penicilliosis and Cryptococcosis(5). Mostly the fungal infections run a chronic indolent course and can bemanaged with routine topical and systemic antifungals. However, there is anacute invasive and life-threatening form usually involving aspergillosis whichis rapidly progressive and may necrose the face and facial bones within a veryshort period. Prompt recognition and treatment are essential for survival inacute invasive aspergillosis.ViralinfectionsCommonviral infections include herpes simplex, herpes zoster, molluscum contagiosumand facial warts. Herpes simplex is usually due to the reactivation of latentinfection with Herpes Simplex Virus and usually manifests as oro-labialvesicles, and rarely folliculitis or (13) verrucous lesions and ulcers inadvanced HIV disease. Herpes Zoster is a recrudescence of varicella zosterinfection.
It is common in early stages of HIV infection and may be the firstclue of infection. Multi-dermatomal Herpes Zoster, common in advanced HIVdisease can also occur in the head and neck along the courses of more than onecranial nerve (13). Molluscum contagiosum is caused by pox virus thatselectively infects human epidermal cells and presents with pearly papules withcentral umbilication, or atypically with lesions such as giant mollusca inadvanced HIV disease (5). Infection with Human Papilloma Virus also frequentlyoccurs and manifests as warts. Antiviral drugs, usually oral (but also systemicin disseminated disease) are used to treat herpes simplex and herpes zoster.Treatment options in Molluscum contagiosum and warts include podophyllotoxin,imiquimod, CO2 laser, cryotherapy, curettage, excision and topical tretinoinand cidofovir (5,13,14). In general, treatment of these viral lesions is moreeffective while the HIV patient is on HAART.
BacterialinfectionsBacterialinfections on the face are also common in HIV-infected patients. Acuteinfections are most commonly caused by Staphylococcusaureus and can manifest as cellulitis, folliculitis, facial abscess, nasalvestibulitis and other skin and soft tissue infections. Acute facial sepsis canhave severe manifestations, progress rapidly and lead to systemic sepsis orintracranial spread of infection in these patients. They should be treatedpromptly according to local antibiotic policies and sensitivity whereapplicable with or without surgical intervention. Chronic infections caused bytuberculosis, atypical mycobacteria and syphilis are also found.
A high indexof suspicion is always needed to direct appropriate assessment and facilitateearly diagnosis and prompt treatment in these chronic infections.OtherSkin LesionsOtherskin lesions include seborrhoiec dermatitis and psoriasis. Seborrhoeicdermatitis presents with a rash and is said to be common in advanced disease.It can occur anywhere in the head and neck but is particularly common in thepost-auricular, nasal, and malar regions and the malar rash can resemble thebutterfly pattern of systemic lupus erythematosus (14). Treatment ofseborrhoiec dermatitis is usually with topical corticosteroids althougheradication of the rash is usually challenging.
Psoriasis has been said tooften occur as the first clinical manifestation of HIV disease although it isoften also seen in advanced disease. The treatment of psoriasis is equally verychallenging and may involve topical treatment, phototherapy and systemictreatment (15). Kaposi’s sarcoma, manifesting as pink, blue or brown lesionsare also commonly found and should not be confused with benign skin lesions.Noseand paranasal sinusesNasaland paranasal sinus manifestations are known to be among the most commonpresentations of HIV disease (1) and estimates from prospective studies havedescribed a 30 to 68% prevalence of sinusitis (1,16,17,18,19). Cutaneouslesions similar to those found on the face are also well documented in the sino-nasalregion (16).
Other problems in this region include nasal obstruction (36) froma wide range of problems that are also commonly found associated with HIV.These include adenoid hypertrophy, allergic rhinitis (18), acute and chronicsinusitis, and sino-nasal or nasopharyngeal neoplasms (20). Kaposi’s Sarcomaand Non-Hodgkin’s lymphoma are both also known to occur in this region in HIVpatients. As a result of many of these lesions, eustachian tube obstruction andeustachian tube dysfunction commonly supervene, associated with sequelae ofmiddle ear effusion and recurrent middle ear infections. Thus, HIV positivepatients who present with nasal obstruction need to be thoroughly evaluated asthe differential diagnosis ranges from benign problems like allergic rhinitisto sinister malignancies. Assessment should include evaluation of hidden areasof the upper aero-digestive tract with a flexible nasal endoscopy, appropriateradiological investigations such as CT or MRI and biopsies of any masses orasymmetrically enlarged nasopharyngeal lymphoid tissue found.Theexternal earTheexternal ear which includes the pinna and the external auditory canal can beaffected by the same spectrum of pathology as the skin since it is lined byskin.
However, the peculiarities of the anatomy may produce additionalsymptomatology. For example, patients with seborrheic dermatitis may presentwith itchy ears and scaly ear discharge. A conductive hearing loss may alsosupervene as debris continues to accumulate. In the same way, neoplasms likeKaposi’s sarcoma may cause hearing loss by obstructing the canal or erodinginto the middle ear, but it can also invade the labyrinth and lead tovestibular symptoms. Also, herpes zoster (affecting the geniculate ganglion ofthe facial nerve, called herpes zoster oticus or Ramsay Hunt syndrome) maypresent with a lower motor neuron facial nerve palsy, deafness, vertigo andpain. Infection of the external ear may present as pinna cellulitis, bacterialotitis externa or a fungal infection (Otomycosis).
The organisms implicated areas expected for the immunocompetent individual. There is, however, anincreasing incidence of unusual infections with organisms like Mycobacterium tuberculosis and Pneumocystis carinii. When otitisexterna does not respond to standard antibiotic regimens, necrotizing otitisexterna, also known as “malignant otitis externa” because of its invasivenature should be suspected. This is a severe manifestation of otitis externausually found in immunocompromised individuals where the infection spreads tothe skull base leading to skull base osteomyelitis and lower cranial nervepalsies usually initially affecting the facial nerve. This diagnosis can beconfirmed using computed tomography (CT) scans of the temporal bone.
The mostcommon pathogen involved is Pseudomonas, but fungi such as Aspergillus may alsobe responsible (22). The middle earInthe middle ear, the most common otologic problems reported in HIV-infectedpatients are middle ear effusion (serous otitis media) and recurrent acuteotitis media. The tendency to develop these conditions is high when there isnasal obstruction, recurrent sinusitis, allergies tumours and subsequenteustachian tube obstruction or dysfunction. The usual organisms found inimmunocompetent patients, Streptococcuspneumoniae and Haemophilus. influenza,predominate but mycobacteria and fungi have also been isolated in HIV patients.
Ear infections are especially common in paediatric patients with HIV diseasedue to a combination of the risk posed by the normal paediatric susceptibilityto middle ear infection (22) as a result of the eustachian tube anatomy inchildren and depressed cell-mediated immunity. HIV patients are also at risk ofsevere morbidity and mortality from complications of otitis media including mastoiditis,labyrinthitis, neck abscesses, venous sinus thrombosis and intracranial spreadof infection. Prompt broad-spectrum anti-infective treatment and closesurveillance for as well as prompt management of complications are mandatory inthese patients. The inner earSensorineuralhearing loss and vertigo can occur in the HIV-infected patient (23,24).
Sensorineural hearing loss can be unilateral or bilateral. It may be due todirect CNS infection by the HIV virus or damage of the cochlear nerve by theneurotropic HIV virus. It may also be due to other CNS infections, for example,syphilis and cryptococcal meningitis, neoplasms or ototoxic medications. Athorough workup is necessary to detect the cause, type and degree of hearingloss and to facilitate appropriate treatment and hearing rehabilitation.Vertigo can also occur in the HIV-infected patient usually co-existing withother neurologic symptoms. Vertigo is frequently secondary to CNS involvementbut can also be due to a direct affectation of the vestibular system by thevirus or as a complication of middle ear infection.
Thorough clinical andlaboratory audio-vestibular assessment is, therefore, necessary to determinethe nature and map out a management strategy. The Oral cavityTheoral cavity is a prime spot in the head and neck where multiple pathologies canand do frequently occur. The spectrum of oral diseases includes infectious,benign inflammatory, neoplastic, and degenerative processes. Oral candidiasis,Recurrent aphthous ulcers, Herpes simplex, Herpes Zoster (Shingles),Xerostomia, Gingivitis, stomatitis , Condylomata, Hairyleukoplakia, Kaposi’s sarcoma and Non-Hodgkin’s lymphoma are some of the morecommon lesionsOralcandidiasis (thrush) is the most common oral condition in HIV-infectedindividuals. It is also one of the commonest the commonest ENT manifestationsof HIV (1,5).
It can present as tender, white, pseudomembranous or plaque-likelesions with underlying erosive erythematous mucosal surfaces (the commonestpresentation), the atrophic form, the chronic hypertrophic form or theclinically obvious angular cheilitis, (a non-healing fissure at the oralcommissure (1,5). Treatment is with topical antifungals in early disease butsystemic in advanced disease with systemic therapy Herpes simplex and varicella zosteralso present in the oral cavity. Oral herpes simplex presents as “cold sores”or “fever blisters” but sometimes with bigger lesions on the palate, gingiva oranother intraoral mucosal surface. Mild oral herpes infections can usually betreated conservatively, but high-dose oral acyclovir should be used for moresevere lesions (1,25).
Oral Varicella Zoster presents along the distribution ofthe trigeminal nerve as crops of vesicles on the hard or soft palate, lips andgingiva or as the corneal infection (zoster ophthalmicus). Verrucae (warts) andcondylomata from Human Papilloma Virus infection are other viral lesions thatcan be found in the oral cavity. Other benign oral cavity lesionsinclude bacterial infections (stomatitis, gingivitis and periodontitis) oralhairy leukoplakia (a whitish, vertically corrugated lesion on anterolateraledge of tongue related to Epstein Barr Virus) and xerostomia (“dryness ofmouth” due to salivary gland disease which may be associated with oral “Thrush”).The major malignancies found are Kaposi’s Sarcoma, Non-Hodgkin’s Lymphoma andSquamous Cell Carcinoma.
Fifty percent of Kaposi’s Sarcomas are found in themouth (95% of these on the palate or gingival surface) (1). Oral Non-Hodgkin’sLymphoma is usually sited on the gingiva and palate with extension toWaldeyer’s ring, especially tonsils. Squamous Cell Carcinoma may also occur inthe oral cavity.
Careful evaluation is needed to ensure that these lesions arenot confused with the many benign lesions that can occur in the oral cavity SalivaryglandsHIV salivary gland disease (HIV-SGD)is a distinct disorder characterized by recurrent or persistent major salivarygland enlargement and xerostomia (26) The parotids are most frequentlyaffected, often with profound bilateral enlargement. Patients usually presentwith several months of progressive parotid swelling with minimal tenderness.Xerostomia leads to loss of the antibacterial properties of saliva creates ahost of other oral cavity problems such as infection, dental caries,periodontal disease, soreness, fissuring of the buccal mucosa and tongue, anddysphagia. HIV-SGD in the parotid glandis uniquely characterised by the formation of lymphoepithelial cysts within thegland (1, 26)) and the finding of lymphoepithelial cysts in a parotid gland andchronic parotitis especially if bilateral are reasons to offer a patient HIVtesting. Pharynx, Larynx and Oesophagus Manyof the problems of the oral cavity can also affect the pharynx larynx andoesophagus due to the anatomical and functional relationship. Prominent lesionsin the area include candidiasis, herpes simplex, recurrent aphthousulcerations, acute adult epiglottitis, benign lymphoid hyperplasia, Kaposi’s`sarcoma and Non-Hodgkin’s lymphoma. Candidiasis in the pharynx or oesophaguscan lead to odynophagia and or dysphagia, and in the larynx to hoarseness andeven aspiration and airway compromise in florid lesions.
Appropriate diagnosticstudies including endoscopy and radiologic investigations will often help inthe diagnosis. Adultacute epiglottitis also deserves special mention since as in theimmunocompetent population, it is rapidly progressive and life-threatening. Thepatient presents with a sore throat and severe odynophagia with drooling, theseverity of which does not correlate with a normal oral cavity andoropharyngeal findings on examination. There may be fever, but this isinconstant and the absence may belie the grave danger that the patient is in.If not promptly treated sore throat worsens, and the patient may developstridor and airway obstruction.
In these cases, it is the lack of clinicallyapparent disease in the setting of such severe symptoms should raise thesuspicion of acute epiglottitis. Diagnosis is confirmed by examination of thehypopharynx and larynx, using a flexible nasal endoscope. Management is withintravenous broad-spectrum antibiotics and close airway observation.Preparation should also be made for intubation if necessary. Lack ofimprovement in 48 to 72 hours is an indication for laryngoscopy and biopsy torule out infection with an unusual organism or underlying malignancy as airwayobstruction is also the most feared complication of malignancy in this area,and these patients are also prone to Kaposi’s Sarcoma and Non-Hodgkin’slymphomaThe neckThemajor manifestation of HIV in the neck is an enlarging neck mass. This ispresent in up to 91% of HIV patients who have head and neck manifestations(27). The commonest causes of these masses are HIV lymphadenopathy, infections,parotid gland enlargement and neoplasms. HIV lymphadenopathy can occur in theneck as part of the persistent generalised lymphadenopathy seen in HIVpatients.
Up to 70% of HIV patients will develop persistent generalisedlymphadenopathy within the first few months of infection (28). Infectiousprocesses in the neck can be due to a variety of organisms. Bacterialinfections are common, and they may progress to cause deep neck space infections.Majority of the organisms causing these infections are similar to those foundin immunocompetent patients, but infections atypical organisms includingtuberculous mycobacteria, atypical mycobacteria, fungi (Cryptococcus,Coccidioides, Histoplasma, Pneumocystis) and parasitic (Toxoplasma). Assessmentof the neck mass in the HIV patient should be thorough especially to able toidentify atypical infections and neoplasms and to chart appropriate treatment.Open neck biopsies are discouraged to prevent seeding of tumours.
Similarly,incisions of neck abscesses are to be avoided until chronic granulomatousdiseases from atypical infections like tuberculosis are ruled out to avoidcreating a wound that would not heal. ConclusionIn a nutshell, while there is no AIDS-definingcondition specific to the head and neck region, there are many Head and Neck manifestationsof HIV. Most patients will initially present to the general practitioner andmany to other clinicians. It is important that all clinicians familiarizethemselves with the ENT manifestations of HIV so that they are recognised earlyand appropriate management is promptly instituted.