Interferon a in combination with ribavirin is beneficial, but relapses are common, necessitating longterm treatment [9]. Parents who have not noticed hairpulling behaviour in their offspring may deny the diagnosis. They are often applied to individual strands of hair, others being left untreated to give highlights, which lessens the problem of darkened roots. Thinning of the eyebrows occurs in hypothyroidism, erythroderma, follicular mucinosis and secondary syphilis. The granulomatous infiltrate is limited to the subcutis and is mainly lobular with the appearance of a granulomatous lobular panniculitis. Some cases are primary and several causes have been identified including previous trauma, operation and radiotherapy [3]. 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. Minocycline 100 mg twice daily achieved complete resolution of chronic cutaneous lesions in eight of 12 patients and partial response in two [382]. Palmoplantar hyperhidrosis is one component of various syndromes in which palmoplantar keratoderma occurs. Topical tacrolimus [21] or pimecrolimus [22,23] have both been advocated, although a rosacealike eruption has also been reported to arise or worsen as a side effect of these agents. A sarcoidal granuloma in a tattoo may be the presenting manifestation of generalized sarcoidosis [15]. Most of the cases show a neutrophilic lobular panniculitis, although in rare instances a septal component may be predominant [11]. The dermal papilla expands from a tightly packed ball of cells into a flask shaped structure where the cells become separated by an extra cellular matrix rich in proteoglycans and basement membrane proteins. Further molecular studies, together with careful phenotyping, will facilitate the understanding of this spectrum of disease. In the absence of any bony involvement, this was diagnosed as an eosinophilic granuloma [1]. Skinpeeling agents can exacerbate the facial dryness as well and should be avoided. However, the use of epinephrine is of little interest in nail surgery as, to achieve a bloodless field, a tourniquet must be placed at the base of the digit in almost all procedures. Associated diseases Association with connective tissue diseases and with rheumatoid arthritis has been reported [3,4]. Cutaneous lesions of sarcoidosis are more frequent in women than in men (2: 1) and in black people than in other ethnic groups [176]. Skin lesions affect any site, but mainly the trunk and proximal limbs; the face, palms and soles are generally spared. Only rarely is the length of the digitations such that clipping of the free edge of the nail induces bleeding [76]. Histologically, there is a superficial neutrophilic folliculitis with an admixture of lymphocytes [1]. Trichostasis spinulosa is a rare condition that may present with redness of the nose and prominent follicular openings with plugging. It was recognized at an early stage that they were capable of producing profound lipoatrophy if they were injected into subcutaneous fat. Smaller quantities of odiferous sulphanylalkanols and steroids have also been identified [12,13]. When neutrophilic lobular panniculitis is found without indications of other specific diagnoses, measurement of serum levels of 1antitrypsin and electrophoretic mobility studies should be undertaken. Histological demonstration of noninflammatory hyaline thrombosis is more common in patients with type I cryoglobulinaemia, but some such patients have also been reported to have cutaneous vasculitis [11]. A simple modification of the starch/iodine test is to dry the skin, paint it with 2% iodine in alcohol, allow it to dry, and then press the skin against a goodquality paper. Healing results in a porcelainwhite scar, frequently surrounded by telangiectasia. It most frequently affects the trunk but can affect the face and presents acutely in association with systemic symptoms. Rhinophyma tends to be progressive through enlargement to distortion of the shape of the nose unless treated.
It is the recommended procedure in several Cochrane reviews as Chronic paronychia Surgical treatment is indicated when medical treatment has failed. 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. In men, where circulating androgens are surplus to the require ments of the hair follicles for maximal stimulation, local factors determine individual susceptibility and severity of baldness. An alternative is to score a transverse groove in the nail at the proximal margin of the pigment and observe over a few weeks as the discoloration grows out. Clinical features History Generally, these lesions are solitary (though multiple lesions may occur in adulthood) and usually occur on the lower extremities. Pathophysiology the disease is thought to represent an inflammatory response in predisposed individuals towards an environmental factor. In patients that have thickened plaques, efforts should be made to remove the scale. These changes include epidermal atrophy with hyperkeratosis, follicular plugging, vacuolar alteration of the basal layer of the epidermis and basement membrane thickening. The Blaschkoid distribution of the lesions suggests that there is genetic mosaicism that leads to the development of the telangiectasia. In most reported management Treatment of sclerema neonatorum is mainly directed to the underlying disease. In a Belgian study, men in constructionrelated occupations (notably electricians) were 10 times more likely to have systemic sclerosis than the general population [1]. It is associated with a number of different genetic mutations and chromosomal aberrations. The overall course is chronic with new crops of lesions repeatedly reappearing in affected areas, although a few cases have entered spontaneous remission. Disease course and prognosis Jaundice: outcome depends on the management of the underlying disease. Differential diagnosis Venous outflow obstruction due to axillary/subclavian vein compression or stenosis, or occlusion from thrombosis, will produce a discoloured (red/blue) painful swollen arm often with parasthesia. Pathophysiology Predisposing factors Epidemiology Pathology Leukocytoclastic vasculitis with segmental inflammation in an angiocentric pattern, swelling of the endothelium, fibrinoid necrosis of vessel walls, extravasation of erythrocytes, and an infiltrate of neutrophils with karyorrhexis of the nuclei. Intravenous cyclophosphamide has the advantage of lower cumulative dose and lower risk of adverse events, but carries a greater risk of relapse [24,25]. It seems slightly more common in boys and usually resolves spontaneously or with minimal treatment. Cellulitis is a common consequence of lymphoedema irrespective of the cause of the lymphoedema. Keloids and hypertrophic scars: results with intra-operative and serial post-operative corticosteroid injection therapy. Complications and comorbidities Constricting bands may be associated with other congenital defects. However, there is no loss of sensation or sweating, or other evidence of neural involvement in granuloma multiforme. Enzymatic fat necrosis is a specific type of liquefactive fat necrosis characteristically observed in pancreatic panniculitis. In addition to these sim ple criteria a more detailed classification should include the dis ease duration and, with regard to patchy alopecia, the extent of the hair loss. Unlike the apocrine glands they have no developmental relationship with the pilosebaceous follicle, although some glands may eventually come to open into the follicular neck. Management the most important measure is to discontinue application of topical corticosteroids. The acute eruption characteristically settles leaving residual hyperpigmentation, especially in those with darker skin types [1,2].
Diseases
Areas particularly affected are those most frequently shaved including the beard, pubic areas and lower legs. 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. Immobilizing any body part in the face of sustained microvascular filtration will result in (lymph)oedema despite patent, and otherwise normal, lymphatic vessels. In severe disease when entire sites are involved with multiple interconnecting sinus tracts, the only curative method is excision of the entire area involved. In some cases this progresses to a total loss of scalp hair (alopecia totalis) or a loss of all hair on the body (alopecia universalis). These are not true tattoos but represent application of a black dye to produce a tattoolike appearance that lasts for a few days. Although local and systemic factors modulate the hair cycle in some species, in adult humans and some other mammals hair cycling is asynchronous. Oral ascorbic acid 500 mg twice daily was found to diminish the severity of miliaria, as was the degree of subsequent anhidrosis in experi mentally induced disease [12]. Presentation the clinical features depend upon their location in relation to the nail apparatus. In general, only those patients with severe disability from hyperhidrosis of the hands or axillae warrant surgery, and in these selected cases the results can be very gratifying. Intermediate uveitis involves the posterior part of the ciliary body (pars plana) and the extreme periphery of the choroid and retina. The main cause for the deformity is lateral compression of the toe due to illfitting footwear, and the main contributory cause is cutting the toenails in a halfcircle instead of straight across. Brain anomalies primarily involve the tissue surrounding the brain including blood vessels [4]. Investigations There are no diagnostic tests and Kawasaki disease remains a clinical diagnosis. Corticosteroids may have some benefit but this is variable and often difficult to assess due to the intermittent nature of the neurological disease; other immunosuppressive agents are often disappointing. Eight cases have been reported following occupational exposure to a caustic drilling fluid used in the petrochemical industry [27]. Complications Lymphatic malformations of the neck region often lead to obstruction of the upper airways. Management There is no treatment but localized areas of alopecia, malignancy or necrosis may be amenable to surgical excision. Pathophysiology Subungual exostosis was previously thought to be a reactive process. Pathophysiology the intense cell proliferation in the anagen hair bulb means that hair growth is particularly vulnerable to antimitotic medication. In the larger, plaquelike lesions, the colloid change occurs diffusely throughout the dermis. Complications and comorbidities Chronic pruritus can lead to scratching, rubbing and pinching. Corticosteroidinduced rosacealike facial dermatosis Introduction and general description the use of potent topical corticosteroids on the face often results in a papulopustular eruption accompanied by erythema that periorificial facial dermatitis 91. A combination of medical and surgical approaches may be required to manage this refractory condition [698]. Such children are char acterized by facial dysmoprhism, short stature and congenital heart defects. Part 9: Vascular vesicles seen in cutaneous lymphangiectasia are not necessarily structurally or histologically any different from those seen in a lymphatic malformation or lymphangioma. The activity of the glands is androgen dependent, and the glands show marked testosterone 5reductase activity [5]. It is at least as common as psoriasis and affects predominantly fairskinned individuals in middle age. This usually causes lower limb lymphoedema first, but in more severe cases the lower abdominal wall and genitalia are affected as well. Emberger syndrome comprises lateonset (but in childhood) bilateral or unilateral lower limb with or without genital lymphoedema together with myelodysplastic syndrome and/or acute myeloid leukaemia [46].
However, the situation is complicated by the fact that some phaeomelaninlike pigments may be structural variants of eumelanins [4]. This may be achieved by direct microscopy, by in vivo staining, by forming plastic impressions [18] or by indicators that become coloured on contact with water, such as the starch/iodine technique [19], bromophenol blue [13], quinizarin [20] and the food dye Edicol ponceau. There are rare instances of transient or permanent bradycardia complicating the technique. Pathology Genetics Wells described a family with eight cases in four sibships of two generations [6]. 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. These include easy bruising, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heart beat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint and muscle aches [3,5]. A mild perivascular and interstitial lymphocytic infiltrate with frequent plasma cells is commonly seen in this subtype. Due to limitations in the definition, selection of study population and duration of followup, there are considerable differences in its reported incidence and prevalence. The management of purpura may involve a multidisciplinary approach including haematology, general medicine, nephrology, rheumatology and dermatology. If the patient is bleeding, full blood count and clotting studies should be undertaken. The fibromatosis rarely results in contractures but tends to be locally invasive and to recur. Detailed histopathological studies, however, have demonstrated that the earliest sites of calcification are the media and/or intima of cutaneous arterioles [13]. Investigations the diagnosis of alopecia areata is straightforward in most cases and investigations are usually not needed. In advanced cases there is usually one, but occasionally more, rounded patches of alopecia over the vertex of the scalp sur rounded by crusting and a few follicular pustules. Treatment of coincident fungal infection may provide clinical benefit, although it is seldom a dermatophyte and positive cultures may merely represent colonization. In one study examining cases of infantile acne, secondary scarring affected 17% of the cases [755]. A rough and irregular nail and nail fold may result with haemorrhage in the nail fold also. Gels and acrylics, being chemically distinct entities, will not necessarily crossreact. When nickel in ear piercings has been banned, as in Denmark in 1992, there has been a substantial fall in nickel sensitization [31]. The cosmetic appearance of isolated pili torti can improve greatly with transi tion from childhood to early adulthood. Increasing the time of exposure to the perming solution up to 20 min increases the curl, but longer times do not give a further increase. Familial cases are usually attributable to infection; simultaneous occurrence in monozygotic twin sisters has been reported [131]. Although up to onethird of patients with warfarininduced skin necrosis may have partial protein C deficiency, the majority of cases appear unrelated to inherited deficiencies of protein C [10]. The site for biopsy must be carefully selected: early lesions are likely to yield more information. Proximal to and arranged perpendicu- larly to them, the subpapillary veins can be visualized. In the Canadian series, relapse following successful treatment occurred in 62% of patients. Occasionally, discrete areas of discoid lupus can mimic psoriasis and a solitary scaly plaque on a bald scalp may represent Bowen disease. Pathology In the early stages, there may be a mild dermal lymphocytic infiltrate, and in the later stages the elastic fibres in the lids fragment and decrease [2].
Presentation Lymphangioma circumscriptum may present at any age but is usually noted at birth or appears during childhood. Lymphoedema as a result of amniotic band constriction Definition and nomenclature Lymphoedema of a limb may occur as a result of amniotic band constriction. It offers the highest sensitivity and best assessment of the extent of the tumour. Erythrodermic sarcoidosis Slightly infiltrated, erythematous plaques coalesce over large areas. Asymptomatic muscle involvement is also common but symptomatic diffuse or nodular myopathy is rare. Symptoms of flushing, diarrhoea, heart failure and bronchoconstriction developing due to excessive secretion of hormones such as serotonin from carcinoid tumours (see Chapter 106) [6]. Complications Patients who are subject to frequent blushing may begin to avoid situations in work or social interactions that they associate with triggering the skin reaction. Complications and comorbidities Cranial nerve involvement may not recover, particularly in facial palsy. Anatomical features, such as an abnormally long great toe and prominent lateral nail folds, are important in some cases. Locally injected triamcinolone delivered by needle or jet injector [71] can improve the appearance, but atrophy usually remains. Subungual keratoacanthoma Definition Subungual keratoacanthoma is a rare benign but rapidly growing and aggressive tumour that is usually situated in the most distal portion of the nail bed. Some authors only advocate repeat testing postbaseline in atrisk groups such as those with diabes and patients with known familial hypertriglyceridaemia [554]. Wax phenotype is determined by a single gene pair, the wet wax allele being dominant [7]. This rarely reported condition appears to be a reaction to the traumatic introduction of foreign materials including fibreglass, vegetable matter, calcium chloride and irritant drugs into the skin. Botulinum toxin injections have been reported to be helpful for some patients with recalcitrant flushing reactions. When numerous, they appear randomly distributed upon the nail surface or have a geometric pattern. Modified decongestive lymphoedema therapy can be successful in treating head and neck lymphoedema following cancer treatment [15]. Clinical photography It is impossible to remember one patient from the next over a period of time and to provide an objective opinion on the state of their hair without some form of photographic record. There is a single reported case of coexistence of both disorders in the same infant, but that is a doubtful case because histopathological study was lacking [13]. The surface of the lymphangiomas may appear extremely warty and the lesions may be mistaken for viral warts. As well as monitoring for clinical improvement, repeat imaging may be needed to assess whether the disease is responding. Occlusion of one or several branches of an atherosclerotic calf artery may directly and irreversibly shut down the skin circulation of a wellcircumscribed area. The natural shape of the free margin of the nail is the same as the contour of the distal border of the lunula. As outlined previously, causes of hyperandrogenism should be ruled out if acne presents in this age group.
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They are asymptomatic and may resolve spontaneously [7,8]: surgery is contraindicated. The rapid response to systemic corticosteroids in one recent report would support this hypothesis [11], but this is by no means a uniform response in patients with this disorder. In scalp folliculitis, the lesions are typically distributed throughout the scalp, and are smaller, usually more numerous, non scarring and more pruritic (see later). In the penile type, surgical trauma, excessive sexual activity, sexual vacuum practices, use of constrictive elements during sexual activity, intravenous drug abuse, prolonged sexual abstinence, local or distant infection, venous obstruction due to bladder distension and pelvic tumours have all been reported [2]. Pax3 appears to prime cells for differentiation, whereas Wnt signalling allows cells to proceed along this route [13]. Other associated abnormalities include clubhand, haemangioma, cleft lip and/or cleft palate [3]. The disease is characterized by diffuse or localized pain involving adipose tissue, usually affecting those who are overweight or obese. Some nonmalignant vascular tumours may exhibit a lymphangiomalike appearance with positive staining for lymphatic markers. In rare instances, subcutaneous granuloma annulare may extend to involve deeper soft tissues and producing a destructive arthritis and limb deformity [14]. Besides venous hypertension and antiphospholipid antibodyrelated syndromes, sickle cell ulcers can show the same porcelainwhite scar of atrophie blanche. Earlier development of acne has been linked with earlier onset of puberty, which may also relate to diet/obesity and other lifestyle factors [11,12]; however; earlier recognition may also lead to earlier presentation. They are very common over the abdomen and breasts in pregnancy, and they may develop on the shoulders in young male weight lifters when their muscle mass rapidly increases [4]. The best advice to give patients who believe a lifestyle factor exacerbates their acne is almost certainly to avoid it for a while and keep a diary of how their skin reacts over a minimum period of several weeks. Mental and physi cal development may be normal but one or both may be slightly, moderately or severely retarded. Differential diagnosis Fibroma, keloid, Koenen tumours, recurring digital fibrous tumour of childhood, cutaneous horn, exostosis. Contact allergic dermatitis may be iatrogenic and deliberate, such as with the use of diphencyprone in alopecia areata treatment. Reduction of degradation by metalloproteinases would be desirable, as in other elastophagocytic disorders [24]. The pigmentation usually fades after parturition but may persist for months or years. Apert syndrome is a rare disorder associated with a hyperresponse of the epiphyses (see earlier) and sebaceous glands to androgens, which results in premature epiphyseal fusion, and acne. The benefit is most pronounced in the first 6 months of therapy and thereafter is marginal. Pre viously reported or hypothesized associations with smoking and benign prostatic hypertrophy were not confirmed [21]. The presence of obesity may aggravate a variety of other skin disorders including hidradenitis suppurativa and pilonidal sinus. Abdominal wall lymphoedema can develop following cancer treatment when ilioinguinal lymph drainage is compromised. Correlation between serum reactivity to Demodexassociated Bacillus oleronius proteins, and altered sebum levels and Demodex populations in erythematotelangiectatic rosacea patients. However, there is a category of this disease where the matrix disease remains unclear and the distal papilloma represents the identifiable entity. Blushing is not usually associated with facial sweating, but there may be accompanying palmar hyperhidrosis and tremor. However, this has been shown to be of little clinical benefit to the patient [17].
Destructive lesions resembling osteomyelitis are demonstrated on radiographs in 25% of patient [658]. Epidemiology Incidence, prevalence and ethnicity Almost all white men develop some recession of the frontal hairline at the temples during their teens. More serious side effects, for example glaucoma, hyperthermia and convulsions, can occur. Lupus panniculitis is a mostly lobular panniculitis with sclerotic septa, but, in contrast with sclerosing postirradiation panniculitis, the inflammatory infiltrate involving the lobules is mostly composed of lymphocytes and plasma cells. Phleboedema and mixed lymphovenous disease 1 Kaipainen A, Korhonen J, Mustonen T, et al. However, occasionally if the condition is associated with pregnancy or the oral contraceptive pill, the Arteriovenous malformations 103. Cutaneous amyloidosis Definition Cutaneous pigmentation is a common feature of cutaneous amyloidosis [1,2], which is discussed in detail in Chapter 58. The few exceptions to this general rule are lymphophilic tumours, such as malignant eccrine poroma, Kaposi sarcoma, lymphangiosarcoma and inflammatory breast cancer (Table 105. Stress and sleep deprivation Few studies have examined the effect of psychological stress on acne. Different preparations of botu linum A toxin have different activities, and dose schedules differ for each product; 0. Excision is usually recommended because of potential to develop a squamous or basal cell carcinoma with a lifetime risk reported between 5 and 22% [354]. In later stages, the lesions show a central area of degenerate collagen admixed with fibrinoid material and surrounded by a palisade of elongated mononuclear histiocytes. In inflammatory acne lesions, the majority of the upregulated genes are involved in inflammatory processes. Patients should be discouraged from spending too long in a chair unless it is a reclining chair. Treatment is challenging [371]; excision of the larger cysts is possible but total removal of all cysts is impractical. Epidemiology Age and gender Acne fulminans is predominantly seen in young white males aged between 13 and 22 years [620] although there have been rare cases reported in females [621]. More recent animal studies have incorporated growth factors with lymph node transfer surgical methods. Antibodies have a pathogenic role and can be used to monitor disease activity [2]. Classification of severity the Hurley staging system refers to three stages based on the presence and extent of sinus tracts and scarring (Table 92. Biotin has been used as systemic therapy, but the evidence for its efficacy is weak [8]. 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. Limited morphological classification of scarring has been described and to date there is poor consensus and clinical assessment of scars demonstrates significant variation between assessors [446]. The hallmark skin finding is naevus psiloliparus, a fatty hamartomatous malformation, of the scalp [3]. The degree of curl or tightness of the permanent wave depends both on the diameter of the roller and the size of the strand wound round the roller. Management may be best achieved through a multidisciplinary approach involving multiple specialists including dermatologists, surgeons, pain specialists, psychiatrists and psychologists. Potent topical corticosteroids may arrest progression and doxycy cline or minocycline is useful in inflammatory cases with pustules. Although originally reported in black women, lipoedematous alopecia also occurs in white women [3,4] and in men [5]. Differential diagnosis Differential diagnoses include lipoma, lymphatic malformation, lymphocele and sarcoma.
Estimates of prevalence have been difficult to calculate as it is presumed that many affected individuals do not come to medical attention. Nonmalignant acquired generalized hypertrichosis Definition Generalized hypertrichosis can occur as the consequence of sys temic disease and medications. Conjunctival pigmentation has been noted particularly in Oriental races and may be striking, especially in the lower fornix and bulbar conjunctiva. Distended dermal lymphatics can bulge on the skin surface and, with tissue organization, produce a cobblestone appearance. Squamous carcinoma of the external auditory canal Definition Malignant or in situ malignant keratinocyte neoplasm of the epidermis lining the external auditory canal. They can also rarely (<1: 10 000) be precipitated during treatment with oral isotretinoin and may be a feature of acne fulminans. Other disorders of fat tissue infiltration such as liposarcoma or lipoblastomatosis may be ruled out based on histological findings [15,16]. No specific organism has been firmly implicated in the aetiology but Staphylococcus aureus may commonly be isolated from swabs from affected skin [3]. Acquired generalized lipodystrophy associated with peripheral T cell lymphoma with cutaneous infiltration. Disease course and prognosis these lesions are benign and increase in number with age. The secretory coil contains three types of cell: large clear cells, which are the main secretory cells, small dark cells, which resemble mucussecreting cells of other organs but whose func tion is not known, and myoepithelial cells [7]. A cream made of 20% silicone oil applied under occlusion may be beneficial where it is impracticable to use silicone sheeting [38]. Pathophysiology the classical concept is that acne results from the combination of increased sebaceous gland activity with seborrhoea, abnormal follicular differentiation with increased keratinization, microbial hypercolonization of the follicular canal and increased inflammation primarily through activation of the adaptive immune system. No association with vitamin D [183] [184] Regular consumption of sweets, nuts, chocolates, and oily foods, were associated with increased acne severity No association of acne with consumption of sugary soft drinks or fatty fish. How ever, an increase in easily extracted telogen hairs may be found in active pattern hair loss. As many women wear their hair long, they are more aware of fluctuations in daily hair shedding. Folliculitis keloidalis (acne keloidalis; see Chapter 93) tends to occur in black men of African descent in their early thirties. Arthritis, glomerulonephritis, pulmonary and ocular disease, leukocytoclastic vasculitis, ocular inflammation and abdominal pain are associated. In some instances, eosinophils may be abundant [28], especially in panniculitis arising at the site of cancer vaccines [20], and in sclerosing lipogranulomata of the genitalia [40]. Complications and comorbidities Usually striae are no more than a cosmetic problem, but occasionally, if extensive, they may ulcerate or tear easily if traumatized. The eyebrows and eyelashes are lost in many cases of alopecia areata and may be the only sites affected. Because these opti cal lightening effects are caused by reflection and refraction of incident light, when such hairs are viewed by transmitted light microscopy they appear dark. The borders of the defects are generously undermined and the edges are gently reapproximated with 5/0 or 6/0 absorbable sutures. The nuclei are deeply indented, possibly due to the contractile properties of the cell. Similar blueblack pigmentation of the legs has resulted from treatment with the 4quinolone antibiotic pefloxacin [9] and the tetracycline antibiotic methacycline [10]. Prognosis the likelihood is that the lesion will continue to enlarge and suffer chronic sepsis unless treated. One study from Japan reported that spontaneous remission within 1 year occurred in 80% of patients with a small number of circumscribed patches of hair loss [7]. In one study, the incidence of lipoatrophy was lower in patients treated with ritonavirboosted atazanavir than in those who received unboosted atazanavir [14]. Of note was that a perception of bald men looking less attractive was found in more than 90% of subjects surveyed.
Anatomy and physiology of subcutaneous adipose tissue by in vivo magnetic resonance imaging and spectroscopy: relationships with sex and presence of cellulite. There have been familial cases of patients with multiple mucosal and cutaneous venous malformations. The degree of bone atrophy as established radiologically is usually much less than the clinical appearance suggests, and is severe only in some cases of early onset. The differential diagnosis of infantile acne includes neonatal acne, acne venenata infantum, chloracne and hyperandrogenism. Genital lymphoedema is much more common in men, probably because of anatomy and the dependent nature of male external genitalia. Physiology Physiological transient vasodilatation plays an integral role in thermoregulation. Arrows indicate the recommended sequence of initial choice but, on failure to respond, secondary choices can be made freely from any treatment. These materials, and hand dermatitis in general, are associated with particular occupations. The preceding inflammatory changes are the single most distinctive feature, but they are not always easy to distinguish. A diffuse dermal melanosis, having a slatyblue colour, can occur in patients with advanced melanoma and may be associated with melanuria [3,4,5]. Compression bandaging will achieve quicker results than compression hosiery but may not be tolerated if the affected tissues are very inflamed and tender. An immune complex mediated mechanism has also been postulated: IgG and IgM have been detected by direct immunofluorescence in the vessel walls of rheumatoid nodules, suggesting that a vasculitic process may be involved [7,8]. Enthusiastic reports should be assessed critically as there may be racial variation in response to treatment and some studies include both keloids and hypertrophic scars. Centrifugal lipodystrophy of the scalp presenting with an archform alopecia: a 10year followup observation. Surgery, laser vaporization [401] or excision have been used to remove large nodules and plaques. The condition is almost always sporadic, but the occurrence in two brothers sug gests a genetic factor may be important. In one large study, the frequency of these findings was livedo reticularis 24%, leg ulcers 5. Altered expression of proteoglycans may affect the threedimensional organization of collagen fibres [20]. Severe dermatitis is the most common adverse event, but the risk can be minimized by careful titration of the concentration. The tumour grows slowly, but those arising in the eyelid (from the Meibomian glands) are more likely to metastasize [368]. Proteinuria or haematuria may indicate glomerulonephritis, which may progress to endstage renal failure, particularly in those with childhood onset. Intriguing results have been reported from the use of relaxin gene therapy on Dupuytren myofibroblasts in vitro, with the potential for use in vivo [34]. Second line Patients with contraindications to cyclophosphamide can be treated with rituximab 375 mg/m2 per week for 4 weeks instead [43,44]. Other less common locations include the retroauricular and submandibular areas [28], tongue [21], mammary region [22], abdomen [7] and perineum or scrotum [29]. However, the diagnosis should prompt ongoing surveillance because it may be a first manifestation of a more generalized vasculitis. In patients with sepsis, solitary or multiple nodules and abscesses appear as a consequence of the haematogenous dissemination of bacteria. Prevalence estimates are limited but have been made in Ethiopia, Cameroon and Uganda. They may be asymptomatic or a major problem for patients with pain, bleeding and disfigurement being the commonest complications.
Panniculitis is a very uncommon complication of tophaceous gout (see Chapter 154). In miliaria rubra, the later changes include keratinization of the intraepi dermal part of the sweat duct, with leakage and then formation of a vesicle around the duct. One sympathetic grey ramus may sup ply 10 or more sensory segments, and one white ramus extends over at least five. Investigations A thorough history, with particular emphasis on precipitating or exacerbating factors, drug usage and food intake, and a detailed Prognosis 106. Eczematous painful paronychia due to cyanoacrylate nail preparations may be observed after about 3 months. However, some authors have proposed that the earliest feature in pancreatic panniculitis is a predominantly septal panniculitis resulting from enzymatic damage to endothelial cells lining septal blood vessels. Hypopigmented mycosis fungoides tends to present in darkskinned individuals: the areas of hypopigmentation are more prominent than in poikilodermatous mycosis fungoides [1,2]. The condition has also been reported in a patient receiving haemodialysis [14] and near the site of insertion of a pacemaker [15]. Meibomian cysts (usually painless), representing chronic inflammation of the meibomian glands, may appear in crops. Apart from this, the detailed cytological changes seen in the matrix epithelium under the electron microscope are essentially the same as in the epidermis [18,19]. The association with vitiligo has demonstrated a shared underlying genetic susceptibility to other autoimmune diseases [10]. Resection of excess skin and soft tissue of the thighs after massive weight loss can also cause lymphoedema. No robust studies are available to support the use of lasers or photodynamic therapy for comedonal acne. 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]. One case of acne fulminans has also been reported in a young man with androgen excess as a result of lateonset congenital adrenal hyperplasia [633]. Although prolongation of these tests would seem to predict a tendency towards bleeding, individuals with lupus anticoagulant activity very rarely bleed abnormally, but may be paradoxically predisposed to clot formation. Cellulite is nowadays among the more common concerns of patients presenting to dermatologists. In addition to an increase in circulating androgens, an increased sensitivity to normal levels of androgens or metabolism of the end organ, the dermal papilla of the hair fol licle, may well be relevant. Dose reduction or withdrawal of amiodarone can lead to complete disappearance of the pigmentation. Most shampoos contain detergents as the principal washing ingredient; detergents are synthetic petroleum products. Disease course and prognosis Prognosis both of in situ and of invasive forms is good: metastases are exceptional [98,110] and only three deaths have been reported [96,111,112]. Investigations for emphysema may be indicated, with referral to a pulmonary physician if necessary. Lymph drainage responds to exercise and movement done while wearing compression [9]. Management It is generally poorly responsive to treatment including potent topical corticosteroids [2]. Congenitalonset primary lymphoedema Historically, all cases of congenital lymphoedema were classified as Milroy disease. Sweat testing may be used as an alternative to urine testing in the setting of substance abuse [2]. It typically starts near the cuticle, extends to the nail tip and becomes progressively darker from base to tip. Epidemiology Incidence and prevalence the incidence and prevalence of this rare condition are unknown.
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