Matthew Kiernan DSc, FRACP

• Professor of Medicine ?Neurology, University of New South Wales
• Consultant Neurologist, Prince of Wales Hospital, Sydney, NSW

Anteroinferiorly there are two horizontal fissures in the cartilaginous canal allergy symptoms baby buy 5 mg deltasone with amex, the fissures of Santorini medisana medinose nasal allergy treatment 45030 order 5mg deltasone with mastercard. These can allow infection or tumour to pass beyond the external auditory canal allergy medicine for 5 yr old cheap deltasone uk, for example to the parotid gland allergy shots psoriasis generic deltasone 20 mg free shipping. Subcutaneous tissue is scanty allergy testing kid cheap deltasone 5mg without a prescription, and the epithelium is firmly bound to the perichondrium allergy x amarillo discount deltasone online american express. Sebaceous glands are plentiful, and open into the follicles of extremely fine vellus hairs. Eccrine sweat glands are not present in the auditory canal but modified apocrine (ceruminous) glands are numerous. There is great individual and racial variability, and although concentrated in the cartilaginous part of the canal, they may also occur, albeit sparsely, in the osseous portion. The inner osseous part of the acoustic canal constitutes twothirds of its total length. The epidermis here is thin and easily traumatized, and rete ridges are absent [1]. A slight narrowing of the canal, the isthmus, occurs at or just medial to the junction of the two parts. Microbiology the skin of the external auditory canal in most healthy individuals supports the growth of multiple bacterial species, especially Staphylococcus epidermidis, Corynebacterium spp. Pseudomonas aeruginosa, often relevant to external otitis, and fungi are not normally found [2]. The normal flora can include organisms such as Turicella otidis, which can cause otitis media [6]. Part 10: SiteS, Sex, age Cerumen (wax) [7] Cerumen is the combined product of sebaceous and apocrine glands. Extrusion is aided by mastication and by the peripheral movement and desquamation of the epithelial cells of the canal. Wax phenotype is determined by a single gene pair, the wet wax allele being dominant [7]. It is likely that antimicrobial peptides play a role [9,10]; other possible reasons include the presence of lysozyme, immunoglobulins and polyunsaturated fatty acids. Two populations have been shown to have excessive production and/or impaction of cerumen: individuals with learning difficulties and the elderly [7]. An increased secretion of cerumen occurs in patients treated with aromatic retinoids [11]. If wax becomes impacted or adherent, it can cause various symptoms such as hearing loss, tinnitus, vertigo, pain and itching, and can be a contributory factor to external otitis. It may be removed by irrigation techniques or by suction under direct vision [12,13]. Regular use of an emollient liquid may have a role in prevention of impaction [15]. Inflammation interferes with normal epidermal migration and tends therefore both to induce and to encourage the retention of scale. Equipment available should include a headlight or equivalent, otoscope, several sizes of ear speculae, ear curettes, metal applicators, bayonet forceps, ear irrigation apparatus and cotton. General inspection of the auricles should take account of their symmetry, size, shape and position, and completeness of development. The ear canal is best inspected when the auricle is pulled gently upwards, outwards and backwards, and the largest possible speculum is used. It is essential to avoid traumatizing the thin skin of the canal, particularly beyond the isthmus. If inspection reveals accumulation of cerumenous debris, this can sometimes be removed carefully using a curette or wire loop along the posterior wall. If a biopsy is required from the canal, this should be devolved to a surgeon with the necessary expertise. Those defects of the ear sufficiently common to constitute a part of general dermatological practice are therefore considered here, together with some general principles relating to congenital ear abnormalities and their more important medical and otological associations [3,4,5,6]. Pinna abnormalities are associated sufficiently often with conductive hearing loss that screening tests should be carried out [7]. Environmental factors may be implicated as in fetal alcohol syndrome and fetal hydantoin syndrome, and maternal exposure to isotretinoin and thalidomide. Congenital ear abnormalities exhibit great variability, even within syndromes or families, and any one aetiological factor may be associated with a variety of ear malformations. External ear malformations as part of a genetic syndrome account for less than 10% of all external ear abnormalities; isolated cases of ear malformation may either be nongenetic in origin or may have a genetic basis but with poor gene penetrance [9]. Developmental defects the auricle begins to develop at the end of the fifth week of embryonic life in the first branchial groove, contributed to by the first (mandibular) and second (hyoid) arches [1]. Six hillocks appear on these arches and later fuse to form the complex shape of the fully developed auricle. Inspection of the external ear at birth is important in the overall assessment of the neonate but interpretation can be complicated by the effects of birth trauma [2]. Small ears are often associated with hearing deficit and may be a feature of many syndromes. In addition to being small, the pinna may be rudimentary, resembling the hillocks from which it is embryologically derived. The more primitive the appearance, the greater the likelihood of hearing abnormalities, in most cases due to defects or atresia of the ossicles. There may also be a narrowing or atresia of the auditory canal [10,11,12,13] and various abnormalities of the middle ear [14] and inner ear [15]. Familial microtia inherited as an isolated autosomal dominant trait has been described [17]. Microtia is one of the birth defects that occurs more on the right than the left side [18]. Part 10: SiteS, Sex, age Macrotia (large ears) Macrotia is a developmental variation in which the amount of tissue between the helix and antihelix is increased, causing the ears to wing out. Such changes are common in Turner syndrome, and there may be associated sensorineural deafness. These include bat ear or protruding ear, in which the antihelix lacks the usual bulge; lop ear, in which there is an unrolled helix, a poorly developed antihelix and scapha, and a large concha resulting in a somewhat floppy ear; and prominent auricular (Darwin) tubercle. These minor ear anomalies can be a syndromic feature or can be associated with conductive and occasionally sensorineural hearing loss, but in most instances they are isolated. A distinctive railroad track abnormality with marked prominence of the crus of Lowset ears Normally, the top of the helix is at the same level as the eyebrow, the earlobe is above the angle of the mandible and the external auditory meatus is at the level of the ala nasi. Although it may be isolated, it is often associated with major middleear or systemic malformations, appearing for example in Turner, Noonan, Patau and Crouzon syndromes. In this rare disorder the ears are large and floppy, in association with a bulbous soft nose, gingival fibromatosis and a variety of other findings including absence or dysplasia of nails and/or of terminal phalanges, hyperextensibility of joints, hepatosplenomegaly, and rarely hypertrichosis and mental retardation. They may be single or multiple, and may occur anywhere in a line from the tragus to the angle of the mouth. Accessory auricles, congenital fistulae and other external ear manifestations may occur alone or may be associated with more widespread first and second branchial arch abnormalities, for example Treacher Collins and Goldenhar syndromes [4,5,9,23], or with developmental abnormalities of the genitourinary tract [9,24], as well as with isolated hearing defects. Because of the association with renal abnormalities, it has been recommended that a renal ultrasound scan should be performed if there is a preauricular pit or sinus associated with one or more of the following: another malformation or dysmorphic feature, a family history of deafness, auricular and/or renal malformations, or a maternal history of gestational diabetes [25]. It has long been observed that the pinna grows progressively throughout life, more so in males than females, and this has been confirmed in a large study [1]. The increase in length of the male ear from the age of 30 years onwards may have a 7year periodicity [5]. Various abnormalities of the configuration of the pinna have been described in the distinctive lumpy scalp syndrome [27], in which other features include absent or rudimentary nipples and dermal nodules on the scalp [28]. Absence of the lobule is, however, usually associated with a syndrome of a more serious nature [7]. An autosomal dominant genetic basis for hairy ears has also been noted in South Indians [30] and Maltese [31]. The crease can be graded in terms of length and depth, and deeper, longer creases have the strongest association. The ear crease appears to be separate from other risk factors for coronary artery disease, and is not simply a function of age [11]. A distinctive condition known as tin ear syndrome has been considered pathognomonic of child abuse: a triad of isolated ear bruising, haemorrhagic retinopathy and a small, ipsilateral subdural haematoma [3]. Following trauma, blood and serum collects in the plane between the perichondrium and cartilage, and will undergo fibrosis if not removed early. The patient should be carefully examined for concurrent auditory canal, middle ear, parotid and central nervous system trauma. Repeated trauma may result in the distorted nodular deformity known as cauliflower ear, which is due to varying degrees of cartilage necrosis, fibrosis and dystrophic calcification. Part 10: SiteS, Sex, age Management the infant with obvious malformation of the pinna that might have auditory system or other associations should be assessed by a paediatrician. Investigations may include radiological evaluation [35], an auditory brainstem evoked response hearing test and a renal ultrasound. The elderly exposed pinna often shows varying degrees of dermal and epidermal atrophy, solar keratoses and lentigines, solar elastosis, telangiectasia and venous lakes. If the pinna is at least partially light Treatment Subperichondrial haematomas must be treated promptly, with full aseptic technique to avoid secondary perichondritis. Small collections of fluid can sometimes be aspirated by syringe, but usually need to be drained through a small incision and a laterally placed traumatic conditions 108. Another useful technique is to use a throughandthrough suture technique to maintain bolsters over the area where the haematoma has been evacuated [6]. Other approaches include a posterior incision and suction drainage [7], or fenestrations in the cartilage to promote adhesion of the opposing perichondrial layers [8]. Trauma this can occur from pressure on the lobe and postauricular skin, or from inaccurate insertion of the post of the earring. Repair of the latter is probably best by excision of the cleft and simple closure with eversion of the edges [21], although a staggered repair such as a Zplasty may be appropriate in some cases. Sensitization Earrings remain a major source of sensitization to nickel, and ear piercing is one explanation for the higher incidence of nickel allergy in females [22]. Even stainless steel studs and clasps, which can produce irritant as well as allergic effects, may release sufficient nickel to elicit contact dermatitis [23]. Gold sensitization, although less common [24], can be a protracted cause of dermatitis even after the earrings are removed [25]. Contact dermatitis from other materials used in earrings, such as olive wood [26], copper [27], cobalt [28,29] and chromium [30], has been described, and may also occur from the use of topical antiseptics, antibiotics and dressings used to treat infection. When nickel in ear piercings has been banned, as in Denmark in 1992, there has been a substantial fall in nickel sensitization [31]. Current trends include using rings or studs in almost all parts of the body, the use of up to 10 or more in a single ear, and the piercing of cartilage. Body piercings other than earlobes represent a marker for risky behaviour [1,2,3]. Complications of ear piercing are very common, with rates of about 30% whether the procedure is carried out by medical personnel, a friend or a relative, or in a store; they are also independent of technique, there being little difference in frequency of complications from piercing by needle, staple gun or sharpened stud [4]. Minor infection is the most common adverse effect, with contact dermatitis, keloid and traumatic tear occurring less often [5]; other consequences occur occasionally. The ear is also pierced in acupuncture as used in traditional medicine, and complications have been reported [10,11]. Embedded earrings the springloaded gun method of ear piercing can result in the earring backing becoming embedded in the back of the ear [41]. The embedded metal can usually be pulled out, or if necessary an incision can be made to locate it. Predisposing factors include skin disease, such as atopic eczema or contact dermatitis. Infants with unsuspected immunodeficiency and individuals with valvular heart disease may be at particular risk [13]. When cartilage is pierced the usual bacterial infection is with Pseudomonas aeruginosa, which causes perichondritis [6] or chondritis [8], and for which the best treatment is ciprofloxacin [7]. Any purulent material should be cultured, since other pathogens have been described. Epidermoid cyst formation Implantation epidermoid cysts due to ear piercing often present as tender, chronic, inflammatory swellings, sometimes with drainage. There is usually an epithelial lined track as well as cysts, and all epithelial tissue must be removed, for example with a skin punch [44]. Piercing before the age of 11 years is more likely to be followed by keloid than after that age [45]. The keloids seem to occur more on the back surface than the front of the earlobe [46]. Treatment options include intralesional steroid, pressure [47], excision alone [48,49], with or without concurrent use of intralesional steroid [50] and radiotherapy [51] (see Chapter 10). A relatively Viral infection Viral hepatitis may occasionally be a hazard [17,18]. Oedema and haematoma [1,19] these commonly occur, and usually respond to cold compresses, pressure and removal of the earring. Males are more commonly affected than females, and most patients are middle aged or elderly.

The course is long and recurrent allergy history order deltasone 5mg mastercard, but fever allergy testing reading results order deltasone american express, arthralgia and visceral involvement are absent allergy medicine for 18 month old buy generic deltasone on-line. Presentation Recurrent pruritic papules and urticarial lesions occur at any site allergy otc meds discount deltasone 20mg online, especially the lower limbs allergy treatment sugar discount deltasone 40 mg visa, head and neck allergy shots ogden utah order generic deltasone canada, with angiooedema of the face and extremities. Digital occlusions manifesting as the Raynaud phenomenon or digital gangrene have been reported in patients with cutaneous eosinophilic vasculitis associated with the hypereosinophilic syndrome [5,6], but they can also occur in the hypereosinophilic syndrome in the absence of cutaneous eosinophilic vasculitis [7,8]. In 1945, Wigley described a 46yearold woman with recurrent, multiple, raised, discrete, smooth, greyish brown, facial lesions. The histology demonstrated pleomorphic infiltrate with predominant eosinophils, but also polymorphs and plasma cells. In the absence of any bony involvement, this was diagnosed as an eosinophilic granuloma [1]. Clinical variants An eosinophilic vasculitis, typically with hypocomplementaemia, also occurs in connective tissue diseases [9]. Differential diagnosis this condition was recently distinguished from other eosinophilic vasculitides that affect mediumsized vessels (eosinophilic granulomatosis with polyangiitis; see separate section this chapter) and from eosinophilic disorders in which pruritic papules and/ or angiooedema may occur, such as hypereosinophilic syndrome, episodic angiooedema with eosinophilia, dermatitis herpetiformis, Wells syndrome, polymorphic eruption of pregnancy or drug eruptions. Complications and comorbidities Ulceration and secondary infection of necrotic lesions may occur. By contrast with eosinophilic granulomatosis with polyangiitis, systemic features are not reported. Ethnicity Granuloma faciale has been reported from various parts of the world and does not seem to have any ethnic predilection. Associated diseases Investigations Investigations are guided by history and clinical examination and will be needed to exclude the differential diagnoses, listed above. As noted by Wigley [1], the dermal infiltrate consists of eosinophils and plasma cells. This raises the possibility that some patients with granuloma faciale may have IgG4related disease. Management Pathophysiology First line the few cases described have been treated with oral corticosteroids with good effect, intermittently or as prolonged maintenance therapy depending on response [10]. Second line Secondary infection of ulcerated lesions may require topical or systemic antibiotics according to sensitivities. Although the aetiology is unclear, this disease is considered to be a histological variant of leukocytoclastic vasculitis with a prominent eosinophilic infiltrate and confined to the skin [4]. The presence of plasma cells and IgG deposition in and around the dermal vasculature has been demonstrated, indicating that granuloma faciale may be immune complex mediated [5]. The vascular changes may be mild (perivascular distribution of inflammatory cells) to florid (leucocytoclastic vasculitis with fibrinoid necrosis). They are almost always asymptomatic, although some patients may describe itching, burning or pain associated with the lesions. They are smooth, with prominent follicular orifices and telangiectatic surface IgA vasculitis 102. Dermoscopy shows parallel, arborizing blood vessels, brown dots and globules and dilated follicular openings [8]. It often involves the skin and gastrointestinal tract, and frequently causes arthritis. In a study of 66 patients, only five patients had extrafacial lesions [4]; all these lesions coexisted with facial lesions. Eosinophilic angiocentric fibrosis is thought to be a mucosal variant of granuloma faciale that may occur in the nasal passages or upper airways in conjunction with skin lesions of granuloma faciale [13,14]. Eosinophilic angiocentric fibrosis may cause fibrotic stenosis of the affected site with localized extension and damage [15]. For example, epiphora and proptosis have been reported in patients with obstructive sinonasal eosinophilic angiocentric fibrosis [16]. Differential diagnosis Granulomatous rosacea does not have vasculitis on histology. Disease course and prognosis Granuloma faciale is a chronic disease with intermittent acute flares that is notoriously resistant to treatment. Introduction and general description William Heberden, in the 1780s, described two children with petechiae, purpura and ecchymosis in conjunction with arthritis. For the purposes of homogeneity and classification, there are two sets of classification criteria in use. These criteria were modified in a combined effort by the European League Against Rheumatism and the Paediatric Rheumatology Society for classifying childhoodonset vasculitis. The presence of any one of the following four features in the presence of palpable purpura satisfies a classification of IgA vasculitis: (i) diffuse abdominal pain (ii) any biopsy demonstrating predominant IgA deposition; (iii) any acute arthritis or arthralgia; and (iv) renal involvement in the form of haematuria or proteinuria. Investigations A definitive diagnosis of granuloma faciale requires clinically consistent lesions and a confirmatory biopsy. Although most laboratory studies are normal, mild peripheral blood eosinophilia may be present [10]. The incidence of nephritis in conjunction with IgA vasculitis is lower in children at about 3. Ethnicity There is a higher incidence of IgA vasculitis reported from Scotland (20. IgArelated nephritis has been more commonly reported in American Indians as compared to Hispanics [14]. Patients with IgA vasculitis are typically younger and have more extrarenal manifestations [15]. Respiratory infections may be a precursor in a small number of cases and may be the second hit in patients with a genetic predisposition [21,23]. Streptococcal infections are the most commonly observed predisposing infections [24,25]. There is perivascular leukocytoclasis and fibrin deposition, and eosinophils are present. In IgA vasculitis, IgA1 rather than IgA2 is the main IgA subclass deposited in skin lesions [26,27]. Diminished glycosylation of the prolinerich hinge region of the IgA1 heavy chain is thought to be an important factor in allowing the IgA to be deposited in the mesangium and in activating the alternative pathway of complement in IgA, as it makes such IgA1 molecules more prone to forming macromolecular complexes [28]. The activation of several cytokines is documented, although these are unlikely to be a primary cause. Neutrophil activation, elevated nitric oxide levels, reactive oxygen species and increased urinary leukotriene are all documented. Streptococcal infections predispose to IgA vasculitis, and antistreptolysin O titre positivity confers a 10fold risk of IgA vasculitis [25] but the exact role of the bacteria is unknown. It is likely to be a complex interplay between genetic predisposition, bacterial infection and perhaps other environmental factors. Bartonella and Haemophilus have been implicated [29,30]; Helicobacter antibodies have been reported in adults with IgA vasculitis [31]. Clinical features (a) History Most commonly, IgA vasculitis manifests at the outset with the classic findings of purpura, arthralgia and abdominal pain. Presentation the cutaneous findings are typically erythematous, urticarial papules, which may evolve within 24 h into palpable purpura with haemorrhage. Although it typically involves the extensor aspects of the limbs (especially the elbows and knees) and buttocks in a symmetrical fashion, IgA vasculitis may also affect the trunk and face. Gastrointestinal involvement is common (65%), with frank gastrointestinal bleeding in 30% of patients with IgA vasculitis. Painful arthritis is seen in about 75% of patients, most frequently affecting the knees and ankles. Clinical variants Rarely, gastrointestinal involvement and arthritis can occur in the absence of skin disease. The tool is able to describe the nature of the clinical involvement, with higher scores suggestive of more severe disease, and is responsive to changes in clinical activity [33]. Complications and comorbidities Endstage renal disease is uncommon but, if it occurs, may need renal transplantation. Introduction and general description Cryoglobulinaemic vasculitis is a smallvessel vasculitis affecting the skin, joints, peripheral nerves and kidneys. Investigations IgA vasculitis is a clinical diagnosis, with confirmation by direct immunofluorescence and routine histology. Epidemiology Incidence and prevalence Management the treatment of IgA vasculitis is supportive. Glucocorticoid agents may be of value in children with renal involvement and in most adults. Pulsed intravenous methylprednisolone, ciclosporin A, cyclophosphamide, azathioprine and mycophenolate mofetil have all been tried in open label fashion. Systemic glucocorticoid treatment may be effective in the treatment of abdominal pain, arthritis and nephritis [38]; in this study the dose was prednisolone 1 mg/kg/day for 2 weeks, tapering over a further 2 weeks. Cryoglobulinaemic vasculitis is a rare disease and its incidence and prevalence are not known. Associated diseases Hepatitis C is responsible for about 80% of cryoglobulinaemic vasculitis. Since most patients with hepatitis C do not develop vasculitis, but have circulating cryoglobulins, there may be a failure of a separate mechanism responsible for the disease manifestations. There are significantly lower circulating Tregulatory cells in patients who develop vasculitis as compared to those with just cryoglobulinaemia [2]. Typically, a polyvalent IgM rheumatoid factor binds to antigen (although other monovalent immunoglobulins can also be responsible) to produce immune complexes that activate complement resulting in endothelial activation and tissue damage. Cryoglobulinaemia is the condition characterized by the presence of circulating cryoglobulins; the accompanying vasculitis that affects the small vessels is due to cryoglobulins deposited as immune complexes. This chapter only deals with the vasculitis manifestations; details of the history, aetiology and pathogenesis of cryoglobulinaemia are provided in Chapter 125. Pathology Cryoglobulinaemic vasculitis affects capillaries, arterioles and venules. In the skin, it produces a pandermal leucocytoclastic vasculitis that may extend into the subcutis. More chronic lesions develop a mononuclearpredominant infiltrate and may become granulomatous. Most other causes of leukocytoclastic vasculitis cause a more superficial vasculitis on biopsy specimens, and if cryoglobulin deposits are seen histologically then the diagnosis is usually suspected (although this is much commoner in type I cryoglobulinaemia). Clinically, head and neck involvement, significant livedo, acrocyanosis, Raynaud phenomenon or larger vessel occlusion are all more suggestive of type I cryoglobulinaemia. Complications and comorbidities In patients with hepatitis Cinduced disease, the complications are those of liver involvement. Associated glomerulonephritis is common and important and may be more frequent in those with hepatitis C [6]. There is an increased risk of myeloproliferative disorders, particularly a Bcell nonHodgkin lymphoma [7]. Disease course and prognosis membranoproliferative glomerulonephritis, immune complex deposition in the lungs causing bronchiolitis obliterans organizing pneumonia, and vasa nervosa vasculitis causing a peripheral neuropathy. In patients with hepatitis Cinduced disease, the viral disease will determine the prognosis. In patients without hepatitis C, renal involvement is associated with greater morbidity. Investigations A pivotal consideration when testing for cryoglobulinaemic vasculitis is transport of the specimen. A renal biopsy will often be positive in the presence of significant renal disease, although occasionally the lesions may be due to minimal change disease [5]. Environmental factors Exposure to cold and immobility can trigger gelling in cryoglobulinaemia, resulting in cutaneous necrosis. Renal involvement is usually in the form of membranoproliferative glomerulonephritis, and presents with nephrotic range proteinuria [5]. A smaller proportion of patients may present with proliferative mesangial lesions or thrombotic lesions [5]. The Raynaud phenomenon may be seen in patients with associated connective tissue disease. In patients with hepatitis C infection, the treatment should be coordinated with a hepatologist and will need a combination of glucocorticoids, antiviral therapy and immunomodulatory agents [9]. Interferon a in combination with ribavirin is beneficial, but relapses are common, necessitating longterm treatment [9].

Sitting with legs dependent causes periods of high venous pressure and consequently high microvascular fluid filtration (falling asleep in a chair without leg elevation is particularly bad) allergy under eyelid cheap 5mg deltasone with mastercard. Sleep apnoea syndrome leads to periods of arterial and pulmonary hypertension and fluid retention [3] allergy shots tallahassee cheap 10 mg deltasone. Lymphoedema can be a difficult diagnosis allergy of water buy generic deltasone 40 mg online, particularly if mild or in the early stages allergy medicine 75 generic 5 mg deltasone otc, therefore it is frequently underdiagnosed allergy medicine 7 year program discount deltasone online. One survey allergy forecast montreal quebec buy generic deltasone 5 mg on-line, which determined the problem of chronic oedema (as a surrogate for lymphoedema) in the community, ascertained 823 patients in a catchment area of 619 000 in southwest London. A plexiform neurofibroma (neurofibromatosis) may cause tissue swelling from both the neural tumour and lymphoedema. A far better approach is to consider if the oedema represents pure lymphatic failure, or, as is most common, lymphatic failure due to the lymph drainage being overwhelmed by increased capillary filtration. Most cases of chronic oedema have more than one factor contributing to the impaired lymph drainage and increased capillary filtration (Table 105. Consequently, treatment of chronic oedema should be to enhance the lymph drainage and address any factors causing increased filtration. If lower limb oedema is unilateral or asymmetrical then local factors need to be considered such as pelvic lymph or venous obstruction, postthrombotic syndrome or inflammation from dertable 105. Simply put, the blood vessel supplies tissue fluid and the lymphatic drains it away. For oedema to develop either the microvascular filtration rate is high, the lymph flow is low, or there is a combination of the two. The colloid osmotic pressures influencing filtration across both fenestrated and continuous capillaries are exerted across the endothelial glycocalyx; the osmotic pressure of the interstitial fluid does not directly determine transendothelial fluid exchange. There is substantial evidence that with important exceptions such as the renal cortex and medulla, downstream microvessels are not in a state of sustained fluid absorption as traditionally depicted. Tissue fluid balance thus depends critically on lymphatic function in most tissues [5]. History Lymphoedema does not usually respond to elevation or diuretics, except in the early stages or when it is compounded by increased capillary filtration. Chronic oedema that does not reduce significantly after overnight elevation is likely to be lymphatic in origin. Chronic oedema associated with bacterial cellulitis indicates lymphatic insufficiency because of the important role the lymphatic plays in tissue immunosuveillance. Calcium channel antagonists are a common cause of peripheral oedema, with amlodipine one of the worst offenders. Other drugs described to cause oedema are corticosteroids, taxanes, nonsteroidal antiinflammatories, clonidine, morphine, gabapentin, olanzapine, pramipexole and thiazolidinediones. If increased microvascular filtration is suspected then the following should be considered and investigated. There are limited methods available that permit reliable investigation of the lymphatics. Lymphoscintigraphy (isotope lymphography) involves the interstitial (dermis or subcutis) injection of a radiolabelled protein or colloid. Radioactivity, measured using a wide field ofview gamma camera, is determined over the injection site depot and at regions of interest over vessels or nodes. Measurement of transit times and time activity curves permits a quantitative analysis of lymph drainage [6]. Thickening of the skin is also a characteristic feature of Presentation Interstitial fluid volume must increase by over 100% before oedema is clinically detectable through pitting or indentation of the skin from pressure. Lymphoedema differs from all other oedemas (in which increased capillary filtration is the major factor) in that cells, proteins and fat accumulate in addition to water. Easy displacement of tissue fluid on pressure can often be demonstrated, particularly in the early stages. In circumstances where the cause of the chronic oedema is not obvious a search for reasons for high microvascular fluid filtration should be pursued, for example raised jugular venous pressure in heart failure, local inflammation from dermatitis or infection, and low plasma proteins. Images show patent lymph routes draining tracer from the feet to the ilioinguinal nodes. Magnetic resonance lymphangiography has recently been introduced to overcome the invasive nature of Xray lymphography. However, it must be remembered that it is the lymphatics and not the veins that are responsible for the drainage of tissue fluid. If the lymph drainage is compensating, no matter how severe the venous disease, there will be no oedema. By implication, oedema in the presence of venous disease indicates lymphatic failure and treatment needs to address improvements in lymph drainage as well as control of the venous disease. Therefore compression is the treatment of choice for venous oedema because compression garments (hosiery) have the advantage of reducing microvascular filtration (from venous hypertension) while at the same time improving lymph drainage. The empirical use of diuretics is to be discouraged as they are often ineffective over time. Lymphatic obstruction usually produces wholelimb swelling that is worse proximally. Genital oedema occurring in isolation is usually a result of local inflammation, for instance due to infection, anogenital granulomatosis (cutaneous Crohn disease), hidradenitis suppurativa or sarcoidosis. Genital oedema can be part of more widespread oedema from heart failure or nephrotic syndrome. Lymph or chylous reflux can produce genital oedema often with lymphangiectasia (weeping lymph blisters). Upper limb oedema is much less common than lower limb oedema and usually results from either proximal venous obstruction. Chronically swollen leg Definition and nomenclature Swelling of the lower limb, due to oedema, is caused by increased microvascular fluid filtration overwhelming lymph drainage. Causes of increased filtration such as increased venous pressure, low plasma proteins and inflammatory states need to be considered as well as reasons for impaired lymph drainage. Inflammation of a joint or periarticular structure may cause oedema that is not primarily vascular. A patient may perceive one leg to be swollen when in fact the other leg has become smaller, for example through atrophy of muscle or fat. A recent report from Denmark indicated that of 595 hospitalizations of patients aged 75 years or above in the emergency department, 6. While usually attributed to chronic venous disease, oedema is always a feature and cases do occur with lymphoedema in the absence of venous reflux. Lymphoscintigraphy is the investigation of choice to confirm a lymphatic aetiology. Venous duplex ultrasound will identify whether venous reflux is contributory to the fluid swelling. A skin biopsy may be necessary if pathologies such as Kaposi sarcoma, pretibial myxoedema or malignancy are considered. As lymph flow is responsible for the drainage of all tissue fluid, except for transient periods of venous reabsorption, a chronically swollen leg due to fluid indicates lymph drainage failure. Causes of increased microvascular fluid filtration are: (i) increased venous pressure due to chronic venous insufficiency, postthrombotic syndrome, venous obstruction or heart failure; (ii) hypoproteinaemia from protein loss. In cases of obesity and infirmity, lymph drainage routes in the leg may be patent but nonfunctional due to lack of mobility, for example in arthritis. In addition sitting for long periods without moving will cause sustained venous hypertension and increased fluid filtration into the legs, while the lack of movement will result in poor lymph drainage. Furthermore, a large obese abdominal apron pressing on the thighs when sitting will obstruct venous outflow. The general principle for treating a swollen limb is to control for increased microvascular filtration and to enhance lymph drainage. Part 9: Vascular phleboedema and mixed lymphovenous disease Definition and nomenclature Phlebolymphoedema or mixed lymphovenous disease is a mixed aetiology swelling of the lower limb due to chronic venous insufficiency and lymphatic insufficiency. Phlebolymphoedema refers to chronic oedema arising from chronic venous hypertension causing increased microvascular fluid filtration overwhelming lymph drainage. Features that indicate primarily a lymphatic cause are: (i) persistent swelling, which can be intermittent at first; (ii) oedema that does not resolve with overnight elevation; (iii) a poor response to diuretics; and (iv) recurrent cellulitis. Presentation Chronic, noninflammatory, asymmetrical lower limb oedema should always suggest a cause within the hindquarter. Systemic causes of oedema including cardiac disease, renal disease or hypoproteinaemia should cause bilateral leg swelling. With increasing chronicity and severity, so skin changes of phleboedema and mixed lymphovenous disease 105. Their endothelial parentage is identical as lymphatics and veins have a common embryological origin [1]. It is assumed that venous oedema is the sole consequence of increased capillary filtration from venous hypertension. As lymph drainage is the main buffer against oedema, it is in fact the failure of local lymphatics to compensate for the increased lymph load from filtration that leads to oedema [2]. The Bonn vein study identified up to 20% prevalence depending on age and severity of the venous disease [3]. Because the calf muscle pump is important for venous drainage, long periods spent with the legs dependent and therefore subject to gravitational forces causes sustained periods of venous hypertension. Patients at risk are those with neurological deficit in the legs preventing movement; those with chronic respiratory or cardiac disease, which requires them to sit upright in a chair day and night; and those with sleep apnoea syndrome who cannot lie flat. Obesity can also result in venous obstruction when a large abdomen compresses the thigh veins when sitting upright in a chair. Treatment with calcium channel antagonists causes lower limb oedema in up to 30% of users [4]. Traditional surgical stripping of varicose veins or harvesting of the great saphenous vein for coronary artery bypass grafting could also damage leg lymphatics and lead to phlebolymphoedema. Fortunately, both procedures are rarely performed since the introduction of endovenous therapy and stenting. Intravenous drug abuse can damage both the veins and lymphatics from both thrombosis and sepsis leading to phlebolymphoedema in the upper and lower limbs. Chronic venous disease may result in symptoms such as heaviness, aching, itching (from varicose dermatitis), skin pigmentation (from purpura or haemosiderin). Symptoms worsen towards the end of the day and are relieved by overnight elevation and are usually exacerbated by heat and alcohol. When lymphoedema dominates, the skin becomes harder and swelling does not resolve as much with overnight elevation. Presentation When chronic oedema is associated with symptoms and signs of chronic venous disease then phlebolymphoedema is likely. Oedema is usually confined to below the knee but severe cases can extend into the thigh. When signs of lymphoedema dominate then tissues will be indurated and pitting more difficult to elicite. Advanced cases develop elephantiasis skin changes with hyperkeratosis and papillomatosis [7]. Recurrent cellulitis can occur due to underlying lymphatic insufficiency and the effect lymphatic dysfunction has on local immune cell trafficking. Chronic regional pain syndrome (reflex sympathetic dystrophy) can present like a phlebolymphoedema, but pain (particularly allodynia) and loss of function are distinctive features. Any oedema, whatever the cause, is due to capillary filtration overwhelming the lymph drainage for a sufficient period of time [5]. Contrary to popular belief, venous reabsorption of interstitial fluid cannot be maintained for any length of time in peripheral tissues. Venous hypertension causes an increase in microvascular fluid filtration that requires greater lymph drainage if oedema is to be avoided. In chronic venous disease the fluid load frequently overwhelms lymph drainage to produce oedema. Over time the high lymph load results in deteriorating lymph drainage and a permanent lymphoedema. Furthermore, high tissue fluid and venous pressure result in lipodermatosclerosis, an inflammatory condition of the most affected skin and subcutaneous tissues. Part 9: Vascular Differential diagnosis When venous hypertension exists with lymphatic insufficiency then phlebolymphoedema is likely. Genetics Many primary lymphoedemas for which gene mutations are known also possess venous reflux because of a genetically determined venous valve failure. Complications Phlebolymphoedema may be complicated by lipodermatosclerosis, dermatitis, ulceration, lymphorrhoea and infection, especially cellulitis. Prognosis Prognosis is poor regarding longterm morbidity unless underlying causes are addressed.

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