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156 CHAPTER 6: The Skin and Nails
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Kaposi sarcoma. Human herpes virus type-8 infection is the cause. Endemic disease in the Mediterranean and Africa can present with thickened scaling
skin, enlargement of the extremity, and ulceration. HIV infection greatly increases the risk of developing KS in a pattern distinct from endemic dis­ease. HIV-associated Kaposi sarcoma frequently involves the skin, mucous membranes (hard palate) and viscera (lungs and gut). The initial lesions are nonblanching, red-blue or bluish-brown papules, plaques, and nod­ules anywhere on the skin. Some lesions become spongy or compressible tumors moving centripetally from the extremities. Lymphadenopathy and lymphedema are late ndings. Iatrogenic immunosuppression in the set­ting of solid organ transplant also can be a risk factor for the development of Kaposi sarcoma.
Cutaneous T-cell lymphoma—mycosis fungoides, Sézary syndrome.
are proliferations of malignant T-cells within the dermis and epidermis.
Mycosis fungoides lesions are indurated, often scaly and atrophic patches and plaques reaching several centimeters in size. They are brown or pink and may appear eczematous. The papules and plaques progress to nodules or large masses, the tumor stage of disease. The disease is limited to the skin and may be confused with eczema and psoriasis. Sézary syndrome is a systemic disease with leukocytosis, lymphadenopathy, and skin inltration producing erythematous indurated thickening of the dermis prominently of the face and brows (leonine facies).
Metastatic cancer. The skin is the site of metastases from carcinomas or lym­phomas. Breast, lung, and colon cancers, B-cell lymphomas, and metastatic melanomas are particularly common in the skin. Any suspicious cutaneous nodule or plaque should be biopsied.
These
Clinical Vignettes and Questions 157
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CLINICAL VIGNETTES AND QUESTIONS
Case 6-1
A 54-year-old man presents with generalized erythroderma.
QUESTIONS:
1. What is the pathophysiology of generalized erythroderma?
2. What is the differential diagnosis of an adult with generalized
erythroderma?
3. What is erythema multiforme?
4. What are common causes of erythema multiforme?
Case 6-2
A 70-year-old man presents with tense bullae on his arms.
QUESTIONS:
1. What is the most likely diagnosis?
2. What condition may be confused with the correct diagnosis?
3. What is a Nikolsky sign?
A 33-year-old patient comes in with recurrent painful vesicles on the lips. Each is umbilicated on an erythematous base.
QUESTIONS:
1. What is the most likely diagnosis?
2. What is the differential diagnosis for vesicular skin lesions?
You just nished examining a 60-year-old man with history of chronic kidney disease stage 4 and severe hypoalbuminemia (secondary to nephrotic syndrome from his recent diagnosis of multiple myeloma). As you are walking out of the patients room you realize that you forgot to examine his nails. You go back to the patients room to examine his nails as they can give you some important clues regarding his condition.
QUESTION:
1. Describe Terry nails, Lindsay’s nails, Beau’s lines, Muehrcke’s
lines, and Mees’ lines and the signicance of each.
Case 6-3
Case 6-4
158 CHAPTER 6: The Skin and Nails
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Case 6-5
You are examining a 50-year-old man with a 70-pack-year smoking history and Chronic obstructive Pulmonary disease (COPD). You notice that he has clubbing. On further questioning he states that he has lost 10 pounds in the last 3 months.
QUESTIONS:
1. What condition should you be most concerned about in this
patient?
2. What is the differential diagnosis of new onset clubbing in adults?
Case 6-6
QUESTIONS:
1. What is the differential diagnosis of one or more cutaneous ulcers
associated with regional lymphadenopathy (the ulceroglandular syndrome)?
2. What is the most essential investigation to narrow this broad dif-
ferential?
3. Describe the clinical ndings for syphilis, tularemia, and anthrax.
A 76-year-old man presents with a skin lesion on his face. You nd a 2-cm at indurated pink plaque with a rolled border.
QUESTIONS:
1. What is the most likely diagnosis?
2. Describe the key features of nonmelanoma skin cancers?
While examining a 46-year-old man suspected of having infective endo­carditis, you remember that subacute bacterial endocarditis (SBE) can be associated with Janeway lesions and Osler nodes.
QUESTIONS:
1. What are Olser nodes? Describe the pathophysiology.
2. What are Janeway lesions? Describe the pathophysiology.
Case 6-7
Case 6-8
CHAPTER 7
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The Head and Neck
This chapter discusses symptoms, signs, and syndromes related to the head and neck; trauma is not covered. At least nine specialties focus on the head and neck, each developing detailed exams often utilizing specialized instru­ments. The exams described here are made with resources available to the general clinician. Symptoms, signs, and syndromes primarily of neurologic signicance are discussed in Chapter 14. By necessity, these distinctions are somewhat arbitrary. During the head and neck examination the examiner identies signicant abnormalities and identies signs of systemic disease. As always, knowing the limits of one’s expertize and the indications for spe­cialty referral are imperative.
MAJOR SYSTEMS OF THE HEAD AND NECK
The skull, facial bones, and scalp provide protection and insulation for deeper organs. The scalp and face have a rich vasculature that vasodilates on cold exposure to maintain normal temperature within vital organs. The head con­tains the organs of special sense: the eyes, ears, olfactory nerve, and taste buds. Special senses are impaired by problems in the sensory organs, cranial nerves, or brain. The tongue, pharynx, and larynx are organs of speech so structural or functional problems alter articulation. The nose, mouth, pharynx, larynx, and trachea form the upper airways. Compromised upper airways affect breathing and voice tone and/or volume. The mouth, teeth, mandible, maxilla, tongue, salivary glands, pharynx, and upper esophagus are the upper alimentary tract necessary for mastication and swallowing. Together the upper airways and digestive tract are the upper aerodigestive tract. The head and neck are highly vascular. The external carotid has rich anastomoses supplying supercial structures so ischemia is unusual. The internal carotid and vertebral arter­ies supply blood to the brain. The head and neck lymphatic network drains to regional lymph node beds. The tonsils and adenoids are lymphatic organs surrounding the upper aerodigestive tract. The neck contains the thyroid and parathyroid glands, major structures of the endocrine system.
FUNCTIONAL ANATOMY OF THE HEAD AND NECK
The Scalp and Skull. The scalp has ve layers: the skin, subcutaneous connec-
tive tissue, epicranius, a subfascial cleft with loose connective tissue, and the pericranium (Fig. 7-1). The outer three are a single thick, tough, and vascular layer, whose strength is supplied by the epicranius. The epicranius is formed by the frontalis muscle attaching to the occiput by a large central aponeurosis, the galea aponeurotica. The skin and subcutaneous tissue are tightly bound to the galea by brous bands that sharply limit the spread of blood or pus. The
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160 CHAPTER 7: The Head and Neck
Loose connective tissue
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Cutis
Galea aponeurotica
FIG. 7-1 Layers of the Scalp. For practical purposes, the cutis, subcutis, and galea aponeurotica constitute a single,
thick, tough layer with fibrous bands compartmentalizing the more superficial tissue and binding it to the galea. Between the galea and the pericranium is a potential space with a little areolar tissue. Fluid and infection spread slowly through the com­partments above the galea but spread easily t hrough the space beneath the galea and its at tached muscles (the epicranium). The pericranium is the periosteal layer that covers the bones of the skull and dips inward at the suture lines. Subperiosteal fluid is limited to the area over a single bone.
pericranium, the periosteum of the skull, dips into the sutures limiting spread of subperiosteal blood or pus to the surface of a single bone. The subfascial cleft between the pericranium and galea allows the scalp to be lifted off the skull with minimal effort, allowing blood or pus to spreads widely beneath it. A useful mnemonic is SCALP: Skin, Connective tissue, Aponeurosis, Loose connective tissue, Periosteum.
The scalp has three lymphatic drainage areas. The forehead and anterior parietal region drain to preauricular lymph nodes. The mid-parietal region drains rst to postauricular nodes and then to nodes in the posterior cervical triangle. The occipital area drains rst into nodes at the origin of the trapezius and then into the posterior cervical triangle.
Subcutis
Pericranium
Bone
The Face and Neck: Facial contour is determined by the frontal bone (form-
ing the forehead and the brows), the maxilla and zygomatic arch (forming the cheeks and inferior orbital rim), the bony and cartilaginous nose, external ears, and mandible. The mandibles articulate with the temporal bone anterior to the acoustic canal. Each ramus drops inferiorly to the angle of the jaw where the mandible turns anteriorly and medially, the two halves meet in the midline forming the chin. The upper and lower teeth contribute to the vertical facial proportions. The bony superstructure is overlaid with muscles and soft tissues, including the lips, giving the face its rounded contours. Mild facial asymmetry is common. The anterior neck is dominated by the thyroid cartilage, which is more prominent in men (the Adam’s apple), the cervical trachea, and the two sternocleidomastoid muscles arising on the mastoid process and inserting on the clavicle and manubrium. The posterior neck is enveloped in thick longitudinal muscles covering the cervical spine from the occiput to the upper back, and the fan-shaped trapezius forming the neck’s posterior lateral contour.
The Ear: The pinna, or auricle, and the external acoustic canal compose the exter-
nal ear; the middle ear consists of the tympanic membrane (TM) and tympanic
cavity with its three ossicles. The internal ear, or bony labyrinth, is composed of the cochlea (the organ of hearing) and semicircular canals (the organ for balance).
Functional Anatomy of the Head and Neck 161
External acoustic
A. External Ear B. Sectional View of Ear
Pharynx
Malleus (hammer)
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External ear. The pinna or auricle is a attened funnel with crinkled walls of yellow broelastic cartilage. It has a wide external brim narrowing internally to the external acoustic meatus. Several prominent folds have considerable indi­vidual variation (Fig. 7-2A). The helix originates as the crus coursing anteri­orly and then winding up, back, and down posteriorly forming the funnel’s brim. Above the midpoint of its posterior vertical portion, a fusiform swelling occasionally develops, the Darwinian tubercle. An inner concentric fold, the antihelix, partially surrounding an ovoid cavity, the concha, is divided into an upper and lower portion by the transverse helical crus. From the anterior brim of the funnel, below the crus a small eminence, the tragus, points back toward the lower concha. From the lower portion of the antihelix, another eminence, the antitragus, points forward to the tragus across the intertragal notch. The deep lower concha forms the external acoustic meatus. At the junction of the inferior limbs of the helix and antihelix is a pendant lobule of adipose and areolar tissue without cartilage, the earlobe.
The external acoustic meatus or canal is ~2.5 cm long, extending from the concha to the TM (Fig. 7-2B). The canal’s lateral third is walled by cartilage, the medial two-thirds runs through the temporal bone. From the concha, the canal forms a gentle S, tending inward, forward, and upward. Approximately 20 mm inside is a bony constriction, the isthmus. Ear wax produced in the cartilaginous canal acidies and protects the epithelium by suppressing bac­terial growth and capturing particles. The wax is moved to the concha by the outward migration of the canal’s epithelium.
Middle ear. The acoustic canal widens within the temporal bone’s petrous portion forming the tympanic cavity. Separating the tympanic cavity from the external canal is the TM, an ovoid biconcave disk slanting across the canal in a plane 35 degrees from vertical, its posterior superior portion is more super­cial than the anterior inferior attachment (Fig. 7-2B). The manubrium of the malleus is rmly attached to the inner TM. Viewed from outside, the attached
Darwin’s tubercle
Crus
meatus
Helix
Antihelix
Concha
FIG. 7-2 Pinna and Middle Ear Anatomy. A. Surface of pinna: The main features are depicted, but there are
Tragus
Antitragus
Earlobe
many individual variations. Darwin tubercle is only occasionally present. B. Middle ear: A vertical section through the ear. Note the flexible cartilaginous and fixed bony segment of the external acoustic meatus. The plane of the TM slants outward ~35 degrees from vertical; the conical apex points inward and upward.
Tympanic
membrane
Cartilaginous
meatus
Incus (anvil)
Stapes (stirrup)
Bony
meatus
Auditory tube (eustachian t.)
Cochlea
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portion appears as a smooth ridge forming a radius of the membrane, slant­ing upward and slightly anterior.
Inner ear. The inner ear, or labyrinth, is within the temporal bone adjacent to the middle ear. It consists of the spiral cochlea (the organ of hearing), the semi- circular canals, ampullae, utricle, and saccule (organs of balance and position sense of the head), and the acoustic nerve endings of CN-VIII. Two windows connect the middle and inner ear: the oval window contains the footplate of the stapes communicating mechanical vibrations to the inner ear via the scala vestibuli; the round window covers the origin of the scala tympani.
The Eyes: The structural and functional anatomy of the eyes is complex, but
a basic understanding facilitates interpretation of visual signs.
Orbits. The bony orbits are quadrilateral pyramids with bases facing anteriorly and apices pointing backward and medially. Their medial sides are parallel, whereas the lateral walls form a 90-degree angle (Fig. 7-3). Seven bones form each orbit. The frontal bone and lesser sphenoid wing form the orbital roof. Portions of the ethmoid, maxillary, lacrimal, and sphenoid bones form the medial wall containing the lacrimal groove for the lacrimal sac anteriorly. The zygomatic bone and greater sphenoid wing form the lateral wall. The orbital oor contains the maxillary, palatine, and zygomatic bones. Several foramens open into the orbit. At the posterior apex within the lesser sphenoid wing is the optic foramen leading into the optic canal containing the optic nerve (CN-II), ophthalmic artery, and sympathetic nerves. The superior orbital ssure between the sphenoid wings separates the roof from the lateral wall. It carries orbital branches of the middle meningeal artery, the superior ophthalmic vein, and four cranial nerves: the oculomotor (CN-III), the trochlear (CN-IV), the rst (ophthalmic) division of the trigeminal (CN-V-1), and the abducens (CN-VI).
Eyelids. The palpebral ssure is the space between the opened lids. The two angles where the lids meet are the lateral (temporal) and medial (nasal) canthi. The caruncle, a small protuberance of modied skin, lies in the medial canthus anterior to a tissue fold, the plica semilunaris. On each lid’s nasal margin is a punctum opening into the superior or inferior canaliculi which meet to form
FIG. 7-3 Relationship of the Orbits and Globes. A horizontal section through the orbits. The medial orbital walls
are parallel. When the globes are in the primary position, the parallel optic axes are parallel with the medial orbital walls. Because the orbital apices and origins of the ocular muscles are medial to the optic axes in the primary position, the lateral rectus muscles are longer than the medial and the superior and inferior recti pull medially.
Lateral canthus
Pinguecula
Medial canthus
Functional Anatomy of the Head and Neck 163
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the common canaliculus draining into the lacrimal sac. The upper lid extends to the superior rim of the bony orbit merging there with the periosteum. The skin covering the lids is the body’s thinnest skin and is readily moved and picked up. During elevation the upper eyelid invaginates between the globe and upper orbital border. The shorter lower lid extends inferiorly from the lid margin to merge with the periosteum of the inferior orbital rim. It does not infold. The lids’ tight orbital rim attachments limit uid movement into or out of the orbit. The lids contain circular bers of the orbicularis oculi muscle innervated by the facial nerve (CN-VII). The upper lid also contains vertical tendons of the levator palpebrae superioris muscle, which originates in the optic foramen and inserts into the tarsal plate. The levator palpebrae is innervated by the oculomotor nerve (CN-III). In the upper lid posterior to the levator is Müller muscle innervated from the cervical sympathetic chain. The lids are stiffened by dense transverse connective tissue plaques, the tarsal plates, which adhere posteriorly to the palpebral conjunctiva. The lids contain mei- bomian glands emptying through pinpoint openings in the lid margins. On the lid margins where the conjunctiva and skin meet is a double row of deeply pigmented eyelashes curving outward. Deep to the temporal side of the upper lid, beneath the frontal bone, lies the tear-producing lacrimal gland. Numerous accessory lacrimal glands within the conjunctiva provide baseline tear produc­tion. The epicanthal fold is a vertical semicircular skin fold over the nasal lids that partially covers the medial canthus. It is present in ~20% of white new­borns but disappears in 97% by the age of 10 years. The epicanthus must be distinguished from the horizontal skin fold in Asian patients that originates in the upper lid and variably overhangs the superior lid (Fig. 7-4).
Conjunctiva. The palpebral conjunctiva follows the inner lid from the lid margin into the superior and inferior fornices. In the fornices the conjunctiva reects covering the sclera as the bulbar conjunctiva. The conjunctiva is rmly attached to the tarsal plates but is quite loose in the fornices permitting globe movement. The larger peripheral episcleral vessels are visible through the bul­bar conjunctiva and slide over the sclera with the conjunctiva. The conjunc­tiva attaches rmly to the sclera at the corneal limbus. Arising from limbal stem cells the corneal epithelium differs from the conjunctiva. The supercial vessels of the bulbar conjunctiva are radial and tortuous (Fig. 7-33A). The deeper vessels radiate near the limbus and are not normally visible. On either
Limbus
Cilia
FIG. 7-4 External Landmarks of the Normal Right Eye.
Pilica semilunaris
Caruncle
Punctum
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side of the limbus is commonly found a raised horizontal yellow plaque, the pinguecula, caused by sun damage to conjunctival elastic tissue.
Cornea. The clear convex cornea has ve transparent avascular layers through which the anterior chamber, iris, pupil, and lens are inspected. The cornea joins the sclera at the limbus. The anterior chamber between the endothelial surface of the cornea and the iris lls with aqueous uid produced by the ciliary body. The uid drains peripherally through the anterior chamber angle at the circumferential junction of the iris and cornea. The cornea and anterior eye are best examined by slit lamp which allows visualization of individual cell layers.
Sclera. Beneath the bulbar conjunctiva the globe is covered by a tough, dense, avascular brous coat, the sclera. It is china-white except for brown melanin spots varying in number with complexion and race. Piercing the sclera is the
scleral foramen for the optic nerve, long ciliary arteries and nerves, short ciliary nerves, and venae vorticosae. The ocular muscle tendons insert into the sclera.
Iris and pupil. The iris is a muscular diaphragm of radial dilating muscle, the dilator pupillae, and a central circumferential muscle, the sphincter pupillae, surrounding the aperture for light, the pupil. These muscles control the pupil’s size regulating the amount of light passing through the lens to the retina.
Lens. Behind the iris, the exible transparent crystalline lens focuses light on the retina. The convex anterior and posterior surfaces join at the equator. The lens is suspended from its equator by the zonula ciliaris inserting into the cili- ary body (Fig. 7-5). For distant vision the eye is at rest, the ciliary muscle relax­ing increases tension on zonula attening the central lens and decreasing its refractive power. The lens accommodates to near focus by contracting the cili­ary muscle which decreases tension on the zonules allowing the elastic lens to become more spherical increasing its refractive power.
Vitreous body. The vitreous is the clear gelatinous tissue behind the lens attaching circumferentially to the peripheral pars plana and retina (vitreous base) and posteriorly to the optic nerve head, vessels, and macula. The vit­reous partially liquees with age. Traction at the vitreous base can tear the retina, allowing liquid vitreous into the subretinal space causing a retinal detachment. In ischemic retinal disease the vitreous is a scaffold for brous tissue and proliferating blood vessels which can contract tearing the retina.
Retina, choroid, and optic nerve. The posterior ocular segment is best con­sidered from the inside out. The retina loosely lines the inner globe, attaching
FIG. 7-5 Cross-Section of the Lens and Ciliary Body.
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anteriorly to the peripheral ciliary body at the pars plana. The retinal nerve ber layer coalesces to form the optic nerve which exits the globe through a lattice-like opening in the posterior sclera, the lamina cribrosa. The central reti­nal artery and vein course within the optic nerve branching onto the retina to
form superior and inferior temporal arcs around the macula. The center of the macula, the fovea, is for detailed vision. The nasal retina, supplied by the nasal vascular arcades, provides indistinct temporal peripheral vision. The retinal pigment epithelium shuttles nourishment and waste between the over­lying retinal ganglion cells and underlying choroid containing net-like blood vessels. External to the choroid is the brous sclera.
The Nose: The external nose is a pyramid joined to the face on one side
(Fig. 7-6). The root connects to the forehead and the sides join in the mid­line forming the dorsum nasi whose superior portion is the bridge of the nose. The tip of the nose is the pyramid’s apex. The triangular base is pierced on both sides by an elliptic orice, the naris (plural nares), separated in the mid­line by the columella, an extension of the nasal septum. Still hairs, the vibrissae, line the margins of the nares inhibiting foreign body inhalation. Each lateral surface ends inferiorly in a rounded eminence, the ala nasi (plural, alae nasi). The upper third of the lateral nasal walls are supported medially by the nasal bone and laterally by the nasal process of the maxilla. The lower two-thirds is supported by the greater alar cartilage and several lesser alar cartilages. The nasal passages are separated anteriorly by the cartilaginous nasal septum and posteriorly by bone, the vomer.
The nasal septum divides the nasal cavity into symmetrical air passages that begin anteriorly at the naris (Fig. 7-7), widen into a vestibule, then become a high, narrow passage ending posteriorly at an oval orice opening into the nasopharynx, the choana. A vascular network on the anterior nasal sep­tum, Kiesselbach plexus, is the site of most nosebleeds. The central septum is a vertical plane. The lateral walls contain three horizontal, parallel, down­ward curving bony plates, the superior, middle, and inferior turbinates or con- chae. The inferior turbinate’s mucous membranes are highly vascular and semi-tumescent. Vasoconstrictor drugs reduce blood ow thereby decreasing tumescence. Under each turbinate is a groove, the superior, middle, and inferior meatus. The olfactory nerve (CN-I) endings are above the superior turbinate. Superior and posterior to the superior turbinate is the opening of the sphe- noid sinus. The superior meatus contains the orices of the posterior ethmoid
FIG. 7-6 The External Nose. These diagrams show the topographic features and the skeleton. Note that the proximal
half of the nose is bone and the distal half (stippled) is cartilage.