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At times, the surface is verrucous, in which case the color may be brownish, and the lesions may be mistaken for warts. This chronic granulomatous disease is endemic in Brazil and also occurs in Argentina and Venezuela. Extrafaciallesions Flushing may involve the ears, lateral facial contours, neck, upper chest, and scalp. Other patients, mostly women, have papules and nodules of the scalp and extremities, with sparing of the mucous membranes, in a. Telangiectasia or dermal mucinosis in an acral papular pattern may be the predominant findings in some cases. VanRijenM,etal: Mupirocin ointment for preventing Staphylococcus aureus infections in nasal carriers. Paradoxically, patients at this stage also have hyperreactive skin and frequently, inflammatory, often pruritic skin diseases. In about 30% of women and in 15% of men, skin manifestations appear in the form of erythema nodosum over the shins and sometimes over the thighs, hips, and buttocks. T-cell receptor gene rearrangement studies that indicate clonality are also supportive but do not alone predict an aggressive course. Acrodermatitis enteropathica has also been reported to flare as a bullous eruption with each pregnancy. The transition zones of the cervix and anus are at highest risk for the development of cancer. In severe or recurrent cases, complement deficiency, especially of the late (C5, C6, C7, or C8) components, should be investigated. These differ from the leiomyoma by dense cellularity, nuclear pleomorphism, numerous mitotic figures, and disarray of the smooth muscle bundles. Physical stimuli may produce urticarial reactions and represent up to 35% of cases of chronic urticaria. These treatments will be a welcome addition with the potential to provide care without the concerns associated with systemic drugs. Dermatophytomas present as yellow streaks within the nail and may respond to unroofing and curettage. Corticosteroid use is generally restricted to unique circumstances, such as impetigo herpetiformis when expeditious delivery is not possible. They present in infancy along the midline of the face, scalp, neck, or back as soft, compressible masses that may transilluminate or enlarge with crying. The distribution of the lesions on 563 Porokeratosis 27 Genodermatoses and Congenital Anomalies. Specific exanthems associated with bacterial or viral infections are discussed in Chapters 14 and 19. Topical corticosteroids, retinoids, calcipotriene, and macrolactams are of some benefit. However, the eruption may persist for many months as a result of fresh, recurrent outbreaks. Pseudomonas hot foot syndrome was reported in a group of 40 children who developed painful, erythematous plantar. Mites Scabies Sarcoptes scabiei, the itch mite, is an oval, ventrally flattened mite with dorsal spines. Many cases of inverted follicular keratosis represent irritated seborrheic keratoses.

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Furunculosis A furuncle, or boil, is an acute, round, tender, circumscribed, perifollicular staphylococcal abscess that generally ends in central suppuration. Bullae are often arranged in rosettes or an annular array, the so-called string of pearls configuration. Lesions often are only slightly raised, but a deep, firm infiltration is palpable. DepsP,etal: Clinical and histological features of leprosy and human immunodeficiency virus co-infection in Brazil. Histologically, the distinctive lesions are sharply demarcated and composed of collagenous stroma showing acid mucopolysaccharides infiltrated by plump mesenchymal cells with oval nuclei. It is frequently associated with a streptococcal infection, and in children, this is by far the most common precipitant. Organisms include Penicillium, Acremonium, Trichoderma, Sce dosporium, and Paecilomyces. Fatal or disseminated infections occurred in 74% of immunosuppressed persons, compared with 6. Epithelioidsarcoma Epithelioid sarcoma occurs chiefly in young adults, with onset usually from ages 20 to 40. Erythema, plaquelike edema, and wheals such as those seen in solar urticaria can be seen. Reticular telangiectatic erythema may occur overlying implantable cardioverter-defibrillators. Retinoids are teratogens, and birth defects may occur with excess vitamin A supplementation during pregnancy. The lesions are arranged in nummular or discoid groups, where they persist unchanged for months and cause no symptoms. Percentage of patients clearing with each drug 194 Published data allow for some comparisons of biologic agents, but the endpoints of some trials differ. Culture on Sabouraud glucose agar shows a growth of creamy, grayish, moist colonies in about 4 days. Diethylcarbamazine, in increasing doses over a 14-day period, is the treatment of choice. The application of a hydrocolloid dressing over an isolated mastocytoma in an infant may reduce the flushing it produces. Mucocutaneous findings include koilonychia, glossitis, angular cheilitis, pruritus, and telogen effluvium, diffuse hair loss. They are nonflagellate oval organisms about 3 mm in diameter, known as Leishman-Donovan bodies. In about 5% of affected persons, serious eye lesions arise late in the disease, gradually leading to blindness. Patients may also have dysmorphic facial appearance, clubbing of the fingers, mild mental retardation, and papilledema. Graded compression stockings and elevation, standard treatments for venous insufficiency, are most effective in this condition. Large, pyramidal, junctional melanocytes are often noted overlying the lesion, and a superficial shave biopsy may be mistaken for a melanocytic lesion. Typically, there is a dense lymphoid infiltrate around the eccrine sweat glands and ducts. Onycholysis and subungual debris may be present, indicating involvement of the nail bed. In adults, the scalp and face are usually spared, but in infants, lesions are usually present over the entire cutaneous surface. Treatment Systemic antibiotics combined with topical therapy are recommended for patients with impetigo contagiosa.

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In about one third of patients with chronic idiopathic urticaria, autoantibodies bind to high-affinity IgE receptors. Haim-Munk syndrome is autosomal recessive with periodontal disease, keratoderma, and onychogryphosis, linked to cathepsin C gene mutations. In areas where parasitic disease is common, eosinophilia is an inexpensive screening test with a fair yield. Progressive accumulation of ceramide in affected tissues results in the complications. These patients may be managed with avoidance of dietary triggers and by sipping iced water to break the flush. Skin findings may include acne fulminans, acne conglobata, pustular psoriasis, hidradenitis suppurativa, dissecting cellulitis of the scalp, Sweet syndrome, Sneddon-Wilkinson disease, and palmoplantar pustulosis. It usually follows drainage of peritoneal abscess, lung abscess, or chronic empyema. HochedezP,etal: Management of travelers with fever and exanthema, notably dengue and chikungunya infections. If there is a palpable dermal component, or if on stretching the lesion there is a pearly quality, a biopsy should be considered. About 75% of patients slowly recover, leaving 25% with some residual motor deficit. Beneath the crusts, exuberant granulations are covered with a seropurulent exudate, which oozes out of small sinuses that extend down to indolent subcutaneous abscesses. Histologically, they demonstrate numerous elongated encapsulated neurofibromas, often embedded in diffuse neurofibroma that involves the dermis and subcutaneous fat. Xerostomia may produce difficulty in speech and eating, increased tooth decay, thrush, and decreased taste (hypogeusia). Scabies is usually contracted by close personal contact, although it may also be transmitted by contaminated linens and clothing. Actinic keratoses are usually erythematous, more sharply rough, and slightly scaly. In this manner, the infection persists and gradually spreads, at times extending deep into the follicles. Topical anticandidal preparations are usually effective, but recurrence is common. Systemic reactions may occur through either large amounts of venom or a previously sensitizing exposure from which anaphylaxis may result, and systemic treatment with epinephrine, antihistamines, or corticosteroids may be needed. Failure to respond within this period should result in reconsideration of the diagnosis, assessment for compliance, and concern about drug resistance. As a result of friction (skin rubbing skin), heat, and moisture, the affected fold becomes erythematous, macerated, and secondarily infected. Minimal credible data exist regarding other possible aggravating dietary factors, but some reports have implicated excess calories, excess protein, high fat (especially saturated and -6 polyunsaturated fatty acids), excess zinc, and excess iron. IkedaT,etal: Disseminated cryptococcosis-induced skin ulcers in a patient with autoimmune hepatitis. Without treatment, erythema migrans and the secondary lesions fade in a median of 28 days, although some may be present for months. Spread of infection in the hospital setting is frequently traced to the hands of a health care worker. OyamaN,KanekoF: Trichilemmal carcinoma arising in seborrheic keratosis: a case report and published work review. BakryO,etal: Adams-Oliver syndrome: a case with isolated aplasia cutis congenita and skeletal defects. Numerous genes have been identified as possibly conferring susceptibility to infection with M. It also upregulates expression of the genes encoding Dsg3 and periplakin; increases measurable levels of E-cadherin, Dsg1, and Dsg3; and interferes with phosphorylation of these adhesion molecules. Candidal paronychia is frequently seen in diabetic patients, and part of the treatment is bringing the diabetes under control.

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The lesions may erupt at any time during pregnancy and usually resolve with delivery. There is a decrease in linoleic acid and an increase in palmitoleic and oleic acids. This is the first component of the syndrome to appear, becoming apparent between ages 5 and 15. Large, hypertrophic, hyperemic nodular masses are centered over the distal half of the nose. It may occur as an autosomal dominant or recessive trait, as a sporadic condition, as an isolated finding or in association with congenital facial and limb abnormalities, or with severe neurologic defects. Incontinentia pigmenti is an important cause of neonatal seizures and encephalopathy. Bone changes of the anterior chest wall on nuclear scans are the most specific diagnostic findings. The treatment of pseudoporphyria is physical sun protection and discontinuance of any inciting medication. The diagnosis of chikungunya virus infection is made by detecting virus-specific IgM in the serum. The dermatitis produced by elevated glucagon levels from islet cell tumors of the pancreas (necrolytic migratory erythema) and a similar dermatosis seen in hepatitis C infection and other forms of hepatic insufficiency (necrolytic acral erythema, pseudoglucagonoma) probably also represent nutritional deficiency dermatoses. The chest wall and mandible are the most common sites for musculoskeletal complaints in adults; the long bones, particularly the tibia, predominate in children. Eighty percent of patients have solitary lesions, with half of these occurring on the head and neck. Histologic findings are spongiosis and neutrophils scattered throughout the epidermis, individual necrotic keratinocytes, perivascular and interstitial infiltrates composed of lymphocytes and neutrophils, and edema of the papillary dermis. The diagnosis is confirmed by finding rubella-specific IgM in oral fluids or the serum. If they are seronegative but had exposures as previously outlined, treatment would be as for early syphilis, with benzathine penicillin, 2. Biopsy shows a patchy lymphohistiocytic and plasma cell infiltrate in the fascia and subfascial muscle, with massive thickening of the fascia and deep subcutaneous septa. Treatment the treatment of choice for all endemic treponematoses is benzathine penicillin G, 1. The outlying vesicles or bullae, which are usually not grouped, resemble varicella and 372 the maxillary or mandibular branches of the trigeminal nerve. Because most of the lymph channels running from the vulva drain into the nodes around the lower part of the rectum, an inflammatory reaction in these nodes results in secondary involvement of the rectal wall. Skin lesions may present as verrucous lesions, ulcers, papulonodular lesions, or crusted papules with umbilication. In girls, masculinization occurs, with the prominent cutaneous signs of excess androgen production. This may be followed by 40 mg/mL triamcinolone by intralesional injection every 3 weeks. MitjaO,etal: Short report: challenges in recognition and diagnosis of yaws in children in Papua New Guinea. RatzingerG,etal: Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. Autosomal recessive neonatal progeroid syndrome is characterized by neartotal absence of fat from birth, with sparing of the sacral and gluteal areas. Congenital erythropoietic porphyria presents soon after birth with the appearance of red urine (noticeable on diapers).

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Hamartin and tuberin associate physically in vivo, suggesting that they function in the same complex rather than in separate pathways. Purpuric macules evolve to bullous lesions that rapidly ulcerate, especially below the knees. Some of these have not been subjected to controlled clinical trials, as is true of most topical treatments. Bed rest is of great value and may be all that is required in mild cases, especially in children. Women are affected three times more often than men, with peak age of onset between the third and fifth decades. Lichenscrofulosorum Lichen scrofulosorum consists of groups of indolent, minute, keratotic discrete papules scattered over the trunk. Hypoparathyroidism may also occur in DiGeorge syndrome, or with parathyroid infiltration or their inadvertent surgical removal during thyroid surgery. Pilosebaceous unit abnormalities are not typically thought to be part of the pathogenesis of this condition; however, some evidence points to abnormalities being present, especially in the patient with the glandular type. Capillaryaneurysms these flesh-colored solitary lesions, resembling an intradermal nevus, may suddenly grow larger and darker and become blue-black or black as a result of thrombosis. The diagnostic hemophagocytic cells may occasionally be identified in skin biopsies. Children with aplastic crisis caused by parvovirus B19 usually do not have a rash. The adverse effects of prolonged retinoid therapy make their use for long-term maintenance therapy difficult. Systemic toxicity usually resolves spontaneously with supportive care within 1 or 2 days. The individual lesions comprising the affected region are erythematous papules and plaques with fine scale. Hormonal therapy Hormonal interventions in women may be beneficial even in the absence of abnormal laboratory tests. CardosoJ,etal: Cellular neurothekeoma with perineural extension: a potential diagnostic pitfall. Tuberculoid leprosy is characterized by asymmetric nerve involvement localized to the skin lesions. Histologically, skin lesions show perivascular and perineural edema and large numbers of lymphocytes. An effective vaccine has not been developed; the only preventive strategy for travelers is to avoid mosquito bites. Most of these children can be managed conservatively, although Crotalidae antivenin, antibiotics, and fasciotomy may be needed. Psoriasiform syphilid has infiltrated copper-colored papules, often arranged in a figurate pattern. After a time, firm subcutaneous nodules, pea-sized or larger, develop on various sites of the body. In more darkly pigmented persons, hypopigmentation is prominent and may be purely macular. The use of bacitracin ointment inside the nares twice daily throughout the course of isotretinoin therapy eliminates, or greatly reduces, the risk of inducing nasal carriage of S. If the infection originally involved one finger, eventually all the fingers and the dorsum of the hand, the palm, or both may become infected, with the erythema appearing and disappearing; or extension may take place by continuity. Although these usually resolve by 1 year of age, lesions may persist for many years. Infantile perineal protrusion refers to a pyramidal soft tissue swelling covered by red or rose-colored skin along the median perineal raphe (skin between posterior fourchette and anus). Such a sore begins a few days after exposure, since the incubation period for chancroid is short, and later the sore may transform into an indurated syphilitic lesion. In men with scrotal lesions, similar lesions rarely will be found on the shaft of the penis, termed idiopathic calcinosis cutis of the penis. Generalized osteoporosis, arterial and venous thrombosis, and mental retardation are features of homocystinuria not found in Marfan syndrome.

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However, muscle inflammation often is present but not symptomatic, and the term hypomyopathic is preferred. Children may be frightened by such a device, so a cotton-tipped swab is an option for them. The lesions are sometimes umbilicated and appear in crops, resembling smallpox or chickenpox. Cutaneous eruptions take the form of erythema nodosum, erythema multiforme, photosensitivity, and scarlatiniform eruptions. Genitalleiomyomas these lesions are located on the scrotum, on the labia majora, or rarely on the nipples. WeaverJ,etal: Simple non-staining method to demonstrate urate crystals in formalin-fixed, paraffin-embedded skin biopsies. A unilateral injury to the innervation of the face, or a stroke, may lead to unilateral localized seborrheic dermatitis. A granulomatous inflammatory infiltrate is frequently present, consisting of lymphocytes, epithelioid cells, and Langerhans giant cells. Voriconazole has also been used alone for patients with less serious blastomycosis. Prior varicella does not always protect the immunosuppressed host from multiple episodes. Other indications are gram-negative folliculitis, inflammatory rosacea, pyoderma faciale, acne fulminans, and acne conglobata. ShemerA,etal: Treatment of tinea capitis-griseofulvin versus fluconazole-a comparative study. There is a small but real risk of developing congestive heart failure associated with the use of itraconazole. The clinical finding of erythema migrans is the most sensitive evidence of early infection. In the United States, leishmaniasis is largely restricted to southern Texas, although rare reports of human cutaneous disease have occurred as far north as Pennsylvania, and visceral leishmaniasis in immunosuppressed humans is being recognized as an emerging infection in areas not previously thought to be endemic for the disease. There may be an association between the rhizomelic variety and maternal autoimmunity and connective tissue disease. Nerve involvement is detected by enlargement of peripheral nerves and lesional loss of sensation. As noted, several of these antibodies identify specific clinical subsets of patients. Additionally, papular lesions located primarily on the hands have been described as rheumatoid papules. When used as a single agent, however, in both children and adults, the efficacy is low (30%), comparable with placebo. In food-cobalamin malabsorption syndrome, the body is unable to release vitamin B12 from food or intestinal transport proteins, especially with accompanying achlorhydria. Transmission to patients through orogenital sex has been proposed, but the virus may also reach the vulvar mucosa hematogenously. The Spanish pine caterpillar, Thaumetopoea pityocampa, causes both dermatitis and anaphylactoid symptoms. As the lesions spread peripherally and the skin clears centrally, large circles are formed by fusion of the spreading lesions to produce gyrate patterns. All-trans-retinoic acid causes terminal differentiation of some leukemic clones and is used to treat promyelocytic leukemia. The lesions are twice as common in men; 70% of diabetic men over age 60 have diabetic dermopathy. Histologically, the nodules contain bilharzial ova undergoing degeneration, with calcification and a surrounding cellular reaction of histiocytes, eosinophils, and occasional giant cells. Fractional photothermolysis has been used in a variety of skin types for both rubra and alba types of striae. Clinically, these cannot be absolutely differentiated, so a biopsy should not be performed. Nail plate thinning, longitudinal ridging, splitting, and nail bed hyperkeratosis may be seen. Most neonatal acne patients remit by age 1 year, although occasionally cases extend into childhood and through puberty.

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Initially, the skin over the nodules is red, smooth, slightly elevated, and shiny. The best method, however, is toluidine blue staining of semithin, plastic-embedded sections. When invasive, infections characteristically are acute, rapidly developing, and often fatal. ZhangJ,etal: Association between vaccination for herpes zoster and risk of herpes zoster infection among older patients with selected immune-mediated diseases. Therefore, excisional biopsy of the typical keratoacanthoma of less than 2 cm in diameter should be considered in most cases. HovnanianA: Netherton syndrome: skin inflammation and allergy by loss of protease inhibition. Over time, fibrosis ensues with coalescence of firm papules into keloidal plaques, as on the neck (acne keloidalis nuchae. Clinical features Classic Kaposi sarcoma the early lesions appear most often on the toes or soles as reddish, violaceous, or bluish black macules and patches that spread and coalesce to form nodules or plaques. Degranulation of dermal eosinophils produces the flame figures seen in histologic sections. A generalized pruritic eruption has been attributed to the larvae of the carpet beetle, Anthrenus verbasci. KapoorS: Vitamin C for attenuating postherpetic neuralgia pain: an emerging treatment alternative. Ultrastructurally, the fibroblasts demonstrate defective synthesis of collagen, deposited as fibrillogranular material. Untreated scarring is severe and can lead to adhesions, vestibular bands, and even vaginal stenosis. Multiple leiomyomas are inherited in an autosomal dominant manner as part of Reed syndrome. The nose is blocked, often with bloodstained mucus, and a copious discharge of mucus runs down over the lips. There is minimal or no loss of sensation over the lesions, no nerve thickening, and no change in sweating. These may be associated with tender nodules (subcutaneous and intramuscular hemorrhage) and subperiosteal hemorrhage, leading to pseudoparalysis in children. The standard treatment is systemic corticosteroids, with approximately 1 mg/kg/day of oral prednisone. These patients respond to discontinuation of the implicated medication or to treatment of the underlying condition. Certain diseases in this group have specific causes (erythema marginatum of rheumatic fever, carrier state of chronic granulomatous disease, erythema migrans of Lyme borreliosis) and are discussed in the relevant chapters. The advancing border is usually slightly raised, warm, red to bluish red, and free of any scale. Cutaneous schistosomal granulomas most frequently involve the genitalia, perineum, and buttocks. The initial dose of clindamycin is 150 mg three times daily, reduced gradually as control is achieved. Skin lesions are common, and in about one quarter of male patients, a dermatologist makes the diagnosis. Some patients may have overlapping features of Proteus syndrome: multiple lipomas, epidermal nevi, cerebriform lesions of the plantar surfaces, vascular malformations, macrodactyly, hemihypertrophy, exostoses, and scoliosis. Infrequently, only the nail may be involved for months, with later appearance of the band on the skin, or the nail may remain the sole area of involvement throughout the course of the disease. For infants with skin involvement, the prognosis is generally good, with either spontaneous remission or spontaneous transformation into benign ganglioneuromas expected. These same gene mutations are found in sporadic seborrheic keratoses, which, not surprisingly, have the same histology. The condition is often clinically misdiagnosed as a malignancy, and the histologic appearance of the characteristic granules allows diagnosis. Sweetsyndrome(acutefebrileneutrophilic dermatosis) Since its first description in 1964 by Dr.

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Primary cutaneous disease also occurs in healthy individuals in the form of a draining abscess or lymphangitic nodules following a cutaneous injury. Skin lesions resolve rapidly, but the neurologic damage may be permanent; thus the importance of early diagnosis. Gradually, the entire nail becomes brittle and separated from its bed as a result of the piling up of subungual keratin. Anthralin Anthralin is effective but is irritating and stains skin, clothing, and bedding. Flattening and cessation of itching are reliably achieved by this approach and in some cases may even be achieved with topical corticosteroids. Liposarcomas may be well differentiated; subtypes include the adipocytic, sclerosing, inflammatory, spindle cell, and dedifferentiate variants. Diagnosis is best made by demonstration of ova in smears taken from the anal region early in the morning before the patient bathes or defecates. The virus is shed into the saliva, so contact with oral secretions is the most common route of transmission. Phagedenic ulcerations and pseudoelephantiasis of the scrotum, penis, or labia are sometimes encountered. Unfortunately, ticks often attach in areas where they are not noticed, allowing them to engorge and transmit disease. Al-JamaliJ,etal: Gorham-Stout syndrome of the facial bones: a review of pathogenesis and treatment modalities and report of a case with a rare cutaneous manifestations. The wood tick (Dermacentor andersoni) is an important disease vector in western states. Their bodies consist of cephalothorax and abdomen, in contrast to insects, which have three body segments. The tumor is mucinous and deeply infiltrative but does not demonstrate nuclear atypia or mitosis. Such patients have a clear history of inoculation and present with a small primary nodule and subsequent secondary nodules along the draining lymphatics, creating a picture similar to sporotrichosis. Involvement of the small intestine may cause extreme dilation of the duodenum and jejunum, producing a characteristic radiographic picture of persistently dilated intestinal loops long after the barium has passed through. The oropharyngeal form may occur after ingestion of infected and inadequately cooked meat. Similarly, cutaneous lesions can be very psoriasiform, and if they develop in a person with known psoriasis, lesions can be mistaken for a flare of that disease. Acid maltose lozenges are less expensive and remain useful for symptomatic relief. It consists of outbreaks of unilateral or segmental papules and plaques that may be few in number or solitary. Rarely reported neurologic sequelae seem to be restricted to the eye, including uveitis, choroiditis, chorioretinitis, and optic atrophy. Larvacurrens Intestinal infections with Strongyloides stercoralis may be associated with a perianal larva migrans syndrome called larva currens because of the rapidity of larval migration (currens means "running" or "racing"). In predisposed persons with Hailey-Hailey disease, skin trauma, bacterial or fungal infection, and dermatoses may trigger lesions. This IgM develops rapidly, but 50% of sera drawn on the first day of the rash are negative. Skin tightness and induration begin on the neck and/or face, spreading symmetrically to involve the arms, shoulders, back, and chest. For patients who continue to have bleeding episodes that require blood transfusions, octreotide, a somatostatin analog known to decrease splanchnic blood flow, may be effective. Treatment Whereas minimal disease heals spontaneously in the majority of patients with histoplasmosis, moderate to severe disease requires therapy. Telangiectases also appear later on the butterfly area of the face, inside the helix and over the backs of the ears, in the roof of the mouth, in the necklace area, in the flexures, and over the dorsa of the hands and feet. Both office and central laboratories can be used to isolate fungi, but false-negative results are common in both settings. Aspergillus fumigatus is the most common cause of disseminated aspergillosis with cutaneous involvement.