Skin Diseases in Pregnancy
Normal skin changes associated with pregnancy are darkening of linea alba (linea nigra), melasma (see Section 13), and striae distensae (Fig. 15-1).
Pruritus occurring in pregnancy may be due to a flare of preexisting dermatosis or a pregnancy-specific dermatosis.
Pregnancy-specific dermatoses associated with fetal risk are cholestasis in pregnancy, pustular psoriasis of pregnancy (impetigo herpetiformis), and pemphigoid gestationis.
Pregnancy-specific dermatoses not associated with fetal risk are polymorphic eruption of pregnancy and prurigo gestationis.
An algorithm of an approach to a pregnant patient with a pruritus is shown in Fig. 15-2.
Figure 15-1. Striae distensae in a pregnant woman (36 weeks of gestation).
Figure 15-2. Algorithm of approach to a pregnant patient with pruritus. AEP, atopic eruption of pregnancy; PEP, polymorphic eruption of pregnancy; PG, pemphigoid gestationis; CP, cholestasis of pregnancy.
Cholestasis of Pregnancy (CP) ICD-9: 646.7 ICD-10: K83.1
Occurs in the third trimester.
Leading symptoms: pruritus, either localized (palms) or generalized. Most severe during the night.
Cutaneous lesions invariably absent, but excoriations in severe cases.
Elevation of serum bile acids.
Fetal risks include prematurity, intrapartal distress, and fetal death.
Treatment: ursodeoxycholic acid, plasmapheresis.
Pemphigoid Gestationis ICD-9: 646.8 ICD-10: 026.4
Pemphigoid gestationis is a pruritic polymorphic inflammatory dermatosis of pregnancy and the postpartum period. It is an autoimmune process with circulating complement-fixing IgG antibodies in the serum. The condition is described in Section 6.
Polymorphic Eruption of Pregnancy (PEP) ICD-9: 709.8 ICD-10: 99.740
PEP is a distinct pruritic eruption of pregnancy that usually begins in the third trimester, most often in primigravidae (76%). Common, estimated to be 1 in 120–240 pregnancies.
There is no increased risk of fetal morbidity or mortality.
The etiology and pathogenesis are not understood.
Average time of onset is 36 weeks of gestation, usually 1–2 weeks before delivery. However, symptoms and signs can start in the postpartum period.
Severe pruritus develops on the abdomen, often in the striae distensae. Skin lesions consist of erythematous papules, 1–3 mm, quickly coalescing into urticarial plaques (Fig. 15-3) with polycyclic shape and arrangement; blanched halos around the periphery of lesions. Target lesions. Tiny vesicles, 2 mm, but bullae are absent. Although pruritus is the chief symptom, excoriations are infrequent. Affected are the abdomen, buttocks, thighs (Fig. 15-3), upper inner arms, and lower back.
The face, breasts, palms, and soles are rarely involved. The periumbilical area is usually spared. There are no mucous membrane lesions.
Differential diagnosis includes all pruritic abdominal rashes in pregnancy (Fig. 15-2), drug reaction, allergic contact dermatitis, and metabolic pruritus.
Laboratory findings including histopathology and immunohistopathology are noncontributory.
The majority of women do not have a recurrence in the postpartum period, with subsequent pregnancies, or with the use of oral contraceptives. If a recurrence occurs, it is usually much milder.
Management: high-potency topical steroids that often can be tapered off, oral prednisone in doses of 10–40 mg/d relieves symptoms in 24 h. Oral antihistamines are ineffective.
Synonyms: PEP, toxemic rash of pregnancy, late-onset prurigo of pregnancy.
Figure 15-3. Polymorphic eruption of pregnancy [previously called pruritic urticarial papules and plaques of pregnancy (PUPPP)] Urticarial papules are present on both thighs where they coalesce to urticarial plaques. Similar papules and urticarial lesions are present within striae distensae on the abdomen of this pregnant woman at 35 weeks of gestation. Lesions were extremely pruritic, causing sleepless nights and great stress, yet there are no excoriations.
Prurigo of Pregnancy and Atopic Eruption of Pregnancy (AEP) ICD-9: 698–2JJ 782.1
Prurigo of pregnancy is now reclassified as part of the AEP spectrum.
AEP consists of flares of atopic dermatitis (also in patients who previously did not have AD); present either with eczematous or prurigo lesions (see Section 2).
The cardinal symptom is pruritus.
Pustular Psoriasis in Pregnancy ICD-9: 696.7 ICD-10: L40.1
Previously called impetigo herpetiformis.
Clinically and histopathologically indistinguishable from pustular psoriasis of von Zumbusch
Burning, smarting, not itching.
May have hypocalcemia and decreased vitamin D levels.
See “Pustular Psoriasis” in Section 3.
Skin Manifestations of Obesity
Obesity is widely recognized as an epidemic in the Western world.
Obesity is responsible for changes in skin barrier function, sebaceous glands and sebum production, sweat glands, lymphatics, collagen structure and function, wound healing, micro- and macrocirculation, and subcutaneous fat.
Obesity is implicated in a wide spectrum of dermatologic diseases, including acanthosis nigricans (Section 5), acrochordons, keratosis pilaris (Section 4), hyperandrogenlsm and hirsutism (Section 33), striae distensae, adipositas dolorosa and fat redistribution, lymphedema, chronic venous insufficiency, (Section 17) and plantar hyperkeratosis (Section 4).
Cellulitis, skin infections (Section 25), hidradenitis suppurativa (Section 1), psoriasis (Section 3), insulin resistance syndrome, and tophaceous gout (p. 400).
Skin Diseases Associated with Diabetes Mellitus*
Acanthosis nigricans (p. 87) and lipodystrophy.
Associated with insulin resistance in diabetes mellitus. Insulin-like epidermal growth factors may cause epidermal hyperplasia.
Adverse cutaneous drug reactions in diabetes (see Section 23).
Insulin: local reactions—lipodystrophy with decreased adipose tissue at the sites of subcutaneous injection; Arthus-like reaction with urticarial lesion at site of injection.
Systemic insulin allergy: Urticaria, serum sickness–like reactions.
Oral hypoglycemic agents: Exanthematous eruptions, urticaria, erythema multiforme, photosensitivity.
Calciphylaxis (p. 429)
Cutaneous perforating disorders
Rare conditions in which horny plugs perforate into the dermis or dermal debris is eliminated through the epidermis. Not always associated with diabetes (p. 432).
Diabetic bullae (bullosis diabeticorum) (p. 382).
Diabetic dermopathy (p. 384).
Eruptive xanthomas (p. 394).
Granuloma annulare (p. 375).
Infections (see Sections 25 and 26).
Poorly controlled diabetes associated with increased incidence of primary (furuncles, carbuncles) and secondary Staphylococcus aureus infections (paronychia, wound/ulcer infection), cellulitis (S. aureus, group A streptococcus), erythrasma, dermatophytoses (tinea pedis, onychomycosis), candidiasis (mucosal and cutaneous), mucormycosis with necrotizing nasopharyngeal infections.
Necrobiosis lipoidica (p. 385).
Peripheral neuropathy (diabetic foot) (p. 383).
Peripheral vascular disease (see Section 17)
Small-vessel vasculopathy (microangiopathy): Involves arterioles, venules, and capillaries. Characterized by basement membrane thickening and endothelial cell proliferation. Presents clinically as acral erysipelas-like erythema, ± ulceration.
Large-vessel vasculopathy: Incidence greatly increased in diabetes. Ischemia is most often symptomatic on lower legs and feet with gangrene and ulceration. Predisposes to infections.
Synonym: Scleredema adultorum of Buschke. Need not be associated with diabetes. Onset correlates with duration of diabetes and with the presence of microangiopathy. Skin findings: poorly demarcated scleroderma-like induration of the skin and subcutaneous tissue of the upper back, neck, proximal extremities. Rapid onset and progression.
Scleroderma-like syndrome. Scleroderma-like thickening of skin and limited joint mobility (“prayer sign”).
*Figures in parentheses indicate page numbers where these conditions are dealt with.
Diabetic Bullae ICD-9: 694.8 ICD-10: E14.650
Large, intact bullae arise spontaneously on the lower legs, feet, dorsa of the hands, and fingers on noninflamed bases (Fig. 15-4).
When ruptured, oozing bright red erosions result but heal after several weeks.
Localization on dorsa of hand and fingers suggests porphyria cutanea tarda, but abnormalities of porphyrin metabolism are not found.
Neither trauma nor an immunologic mechanism has been implicated. Histologically, bullae show intra- or subepidermal clefting without acantholysis.
Figure 15-4. Diabetic bulla A large, intact bulla is seen on the pretibial skin on the right lower leg. The patient had many of the vascular complications of diabetes mellitus, i.e., renal failure, retinopathy, and atherosclerosis obliterans resulting in amputation of the left big toe.
"Diabetic Foot” and Diabetic Neuropathy ICD-9: 713.5 ICD-10:G63.2
Peripheral neuropathy is responsible for the “diabetic foot.”
Other factors are angiopathy, atherosclerosis, and infection and most often they are combined.
Diabetic neuropathy is combined motor and sensory. Motor neuropathy leads to weakness and muscle wasting distally.
Autonomic neuropathy accompanies sensory neuropathy and leads to anhidrosis, which may not be confined to the distal extremities.
Sensory neuropathy predisposes to neurotropic ulcers over bony prominences of feet, usually on the great toe and sole (Fig. 15-5).
Ulcers are surrounded by a ring of callus and may extend to interlying joint and bone, leading to osteomyelitis.
Figure 15-5. Diabetic, neuropathic ulcer on the sole A large ulcer overlying the second left metacarpophalangeal joint. The patient, a 60-year-old male with diabetes mellitus of 25 years’ duration, has significant sensory neuropathy of the feet and lower legs as well as peripheral vascular disease, which resulted in the amputation of the fourth and fifth toes.
Diabetic Dermopathy ICD-9: 709.8 ° UCD-10: E14:560
Circumscribed, atrophic, slightly depressed lesions on the anterior lower legs that are asymptomatic (Fig. 15-6).
They arise in crops and gradually resolve, but new lesions appear and occasionally may ulcerate.
The pathogenic significance of diabetic dermopathy remains to be established, but it is often accompanied by microangiopathy.
Figure 15-6. Diabetic dermopathy A crusted erosion at the site of traumatic injury and many old pink depressed areas and scars are seen on the anterior leg of a 56-year-old male with diabetes mellitus. Identical findings were on the other leg.
Necrobiosis Lipoidica ICD-9: 709.3 ICD-10: E14.640
Necrobiosis lipoidica (NL) is a cutaneous disorder often, but not always, associated with diabetes mellitus.
Young adults, early middle age, but not uncommon in juvenile diabetics. Female:male ratio: 3:1 in both diabetic and nondiabetic forms.
Incidence: From 0.3% to 3% of diabetic individuals. One-third of patients have clinical diabetes, one-third have abnormal glucose tolerance only, one-third have normal glucose tolerance.
The severity of NL is not related to the severity of diabetes. Control of the diabetes has no effect on the course of NL.
Slowly evolving and enlarging over months, persisting for years. Cosmetic disfigurement; pain in lesions that develop ulcers.
Lesion starts as brownish-red or skin-colored papule that slowly evolves into a well-demarcated waxy plaque of variable size (Fig. 15-7A). The sharply defined and slightly elevated border retains a brownish-red color, whereas the center becomes depressed and acquires a yellow-orange hue. Through the shiny and atrophic epidermis, multiple telangiectasias of variable size are seen. Larger lesions formed by centrifugal enlargement with elevated erythematous border (Fig. 15-7B) or merging of smaller lesions acquire a serpiginous or polycyclic configuration. Ulceration may occur within the plaques, and healed ulcers result in depressed scars. Burned-out lesions are tan with telangiectasia.
Usually one to three lesions; >80% occur on the shin; at times symmetric. Less commonly on feet, arms, trunk, or face and scalp; rarely may be generalized.
Dermatopathology: Sclerosis, obliteration of the bundle pattern of collagen → necrobiosis, surrounded by concomitant granulomatous infiltration in lower dermis. Microangiopathy.
The lesions are so distinctive that biopsy confirmation is not necessary; however, biopsy may be required in early stages to rule out granuloma annulare (which frequently coexists with NL), sarcoidosis, or xanthoma.
Glucocorticoids. Topical: Under occlusion is helpful; however, ulcerations may occur when NL is occluded. Intralesional : triamcinolone, 5 mg/mL, into active lesions or lesion margins usually arrests extension of plaques of NL. Ulcération: Most ulcerations within NL lesions heal with local wound care; if not, excision of entire lesion with grafting may be required.
Figure 15-7. Necrobiosis lipoidica diabeticorum (A) A large, symmetric plaque with active tan-pink, yellow, well-demarcated, raised, firm border and a yellow center in the pretibial region of a 28-year-old diabetic female. The central parts of the lesion are depressed with atrophic changes of epidermal thinning and telangiectasia against yellow background. (B) Same lesion several months later showing progression with a granulomatous, more elevated and reddish border.
Cushing Syndrome and Hypercorticism ICD-9: 255.0 ICD-10:E24
Cushing syndrome (CS) is characterized by truncal obesity, moon face, abdominal striae, hypertension, decreased carbohydrate tolerance, protein catabolism, psychiatric disturbances, and amenorrhea and hirsutism in females.
It is associated with excess adrenocorticosteroids of endogenous or exogenous source.
Cushing disease refers to CS associated with pituitary adrenocorticotropic hormone (ACTH)-producing adenoma. CS medicamentosum refers to CS caused by exogenous administration of glucocorticoids.
Skin lesions: A plethoric obese person with a “classic” habitus that results from the redistribution of fat: moon facies (Fig. 15-8), “buffalo” hump, truncal obesity, and thin arms. Purple striae, mostly on the abdomen and trunk; atrophic skin with easy bruising and telangiectasia. Facial hypertrichosis with pigmented hairs and often increased lanugo hairs on the face and arms; androgenetic alopecia in females. Acne of recent onset (without comedones) or flaring of existing acne.
General symptoms consist of fatigue and muscle weakness, hypertension, personality changes, amenorrhea in females, polyuria, and polydipsia.
Workup includes determination of blood glucose, serum potassium, and free cortisol in 24-h urine. Abnormal dexamethasone suppression test with failure to suppress endogenous cortisol secretion when dexamethasone is administered. Elevated ACTH. CT scan of the abdomen and the pituitary. Assessment of osteoporosis.
Management consists of elimination of exogenous glucocorticoids or the detection and correction of underlying endogenous cause.
Figure 15-8. Cushing syndrome Plethoric moon facies with erythema and telangiectases of cheeks and forehead; the face and neck and supraclavicular areas (not depicted here) show increased deposition of fat.
Graves Disease and Hyperthyroidism ICD-9: 242.0 ICD-10: E05.0
Graves disease (GD) is a disorder with three major manifestations: hyperthyroidism with diffuse goiter, ophthalmopathy, and dermopathy. These often do not occur together, may not occur at all, and run courses that are independent of each other.
Ophthalmopathy. GD ophthalmopathy has two components, spastic (stare, lid lag, lid retraction) and mechanical [proptosis (Fig. 15-9A), ophthalmoplegia, congestive oculopathy, chemosis, conjunctivitis, periorbital swelling, and potential complications of corneal ulceration, optic neuritis, optic atrophy]. Exophthalmic ophthalmoplegia: ocular muscle weakness with inward gaze, convergence, strabismus, and diplopia.
Acropachy, which represents diaphyseal proliferation of the periosteum and clubbing of fingers (Fig. 15-9B).
Dermopathy (pretibial myxedema): Early lesions—bilateral, asymmetric, firm, nonpitting nodules and plaques that are pink, skin-colored, or purple (Fig. 15-9C). Late lesions—confluence of early lesions, which symmetrically involve the pretibial regions and may, in extreme cases, result in grotesque involvement of entire lower legs and dorsa of feet; smooth surface with orange peel–like appearance, later becomes verrucous.
Note. Dermopathy may also occur after treatment of hyperthyroidism.
Thyroid: Diffuse toxic goiter, asymmetric, lobular. Asymmetric and lobular thyroid enlargement, often with the presence of a bruit.
Management: Thyrotoxicosis—Antithyroid agents. Ablation of thyroid tissue, surgically or by radioactive iodine. Ophthalmopathy—-Symptomatic treatment in mild cases. Severe cases: prednisone 100–120 mg/d initially, tapering to 5 mg/d. Orbital radiation. Orbital decompression. Dermopathy—Topical glucocorticoid under plastic occlusion. Low-dose oral glucocorticoids (prednisone, 5 mg/d). Intralesional triamcinolone 3–5 mg/mL for smaller lesions.
Hypothyroidism and Myxedema ICD-9: 244.0–244.9 ICD-10: E03.9
Myxedema results from insufficient production of thyroid hormones and can be caused by multiple disturbances.
Hypothyroidism may be thyroprivic (e.g., congenital, primary idiopathic, postablative); goitrous (e.g., heritable biosynthetic defects, maternally transmitted, iodine deficiency, drug-induced or chronic thyroiditis); trophoprivic(e.g., pituitary); or hypothalamic [e.g., infection (encephalitis), neoplasm].
Early symptoms of myxedema are fatigue, lethargy, cold intolerance, constipation, stiffness and cramping of muscles, carpal tunnel syndrome, menorrhagia, slowing of intellectual and motor activity, decline in appetite, increase in weight, and deepening of voice.
There is a dull, expressionless facies (Fig. 15-10), with puffiness of eyelids. Skin appears swollen, cool, waxy, dry, coarse, and pale with increased skin creases.
The hair is dry, coarse, and brittle. Thinning of the scalp, beard (Fig. 15-10), and sexual areas. Eyebrows: alopecia of the lateral one-third. Nails brittle and slow growing.
Large, smooth, red, and clumsy tongue.
Workup includes thyroid function tests, thyroid-stimulating hormone, scintigraphic imaging, and serum cholesterol (↑).
Management is by replacement therapy.
Figure 15-9. Graves disease (A) Proptosis, lid retraction, and telangiectasia and hemorrhage in the bulbar conjunctiva. (B) Thyroid acropachy (osteoarthropathy) with clubbing of fingers. (C) The pink- and skin-colored papules, nodules, and plaques in the pretibial region are called dermopathy (formerly pretibial myxedema).
Figure 15-10. Myxedema Dry, pale skin; thinning of the lateral eyebrows; puffiness of the face and eyelids; increased number of skin creases; dull, expressionless, beardless facies.
Addison Disease ICD-9: 255.41 ICD-10: E27.1
Addison disease is a syndrome resulting from adrenocortical insufficiency.
It is insidious and is characterized by progressive generalized brown hyperpigmentation, slowly progressive weakness, fatigue, anorexia, nausea, and, frequently, GI symptoms (vomiting and diarrhea).
Suggestive laboratory changes include low serum sodium, high serum potassium, and elevation of the blood urea nitrogen. The diagnosis is confirmed by specific tests of adrenal insufficiency.
Skin: the patient may appear completely normal except for a generalized brown hyperpigmentation: (1) in areas where pigmentation normally occurs either habitually or UV induced: around the eyes, face, dorsa of hands (Fig. 15-11A), nipples, in the linea nigra (abdomen), axillae, and anogenital areas in males and females and (2) in new areas: gingival or buccal mucosa, creases of palms (Fig. 15-11B), bony prominences. Also in new scars following surgery.
This disease should be managed by an endocrinologist.
Figure 15-11. Addison disease (A) Hyperpigmentation representing an accentuation of normal pigmentation of the hand of a patient with Addison disease. (B) Note accentuated pigmentation in the palmar creases.
Metabolic and Nutritional Conditions
Xanthomas ICD-9: 272.2 ICD-10: E78.5
Cutaneous xanthomas are yellow-brown, pinkish, or orange macules, papules, plaques, nodules, or infiltrations in tendons.
Histologically, there are accumulations of xanthoma cells—macrophages containing droplets of lipids.
Xanthomas may be symptoms of a general metabolic disease, a generalized histiocytosis, or a local fat phagocytosing storage process.
The classification of metabolic xanthomas is based on this principle: (1) xanthomas due to hyperlipidemia and (2) normolipidemic xanthomas.
The cause of xanthomas in the first group may be a primary hyperlipidemia, mostly genetically determined (Table 15-1), or secondary hyperlipidemia, associated with certain internal diseases such as biliary cirrhosis, diabetes mellitus, chronic renal failure, alcoholism, hyperthyroidism, and monoclonal gammopathy, or with intake of certain drugs such as beta-blockers and estrogens.
Some of the xanthomas are associated with high plasma low-density lipoprotein (LDL)-cholesterol levels, and therefore with a serious risk of atheromatosis and myocardial infarction. For that reason, laboratory investigation of plasma lipid levels is always necessary. In some cases, an apoprotein deficiency is present.
Table 15-2 shows correlations of clinical xanthoma type and lipoprotein disturbances.
TABLE 15-1 CLASSIFICATION OF GENETIC HYPERLIPIDEMIAS
TABLE 15-2 CLINICAL PRESENTATIONS OF XANTHOMAS
Xanthelasma ICD-9: 374.51 ICD-10: H02.6
Most common of all xanthomas. In most cases, an isolated finding unrelated to hyperlipidemia.
Occurs in individuals >50 years; however, when in children or young adults, it is associated with familial hypercholesterolemia (FH) or familial dysbetalipoproteinemia (FD).
Skin lesions are asymptomatic. Soft, polygonal yellow-orange papules and plaques localized to upper and lower eyelids (Fig. 15-12) and around inner canthus. Slow enlargement from tiny spots over months to years.
Cholesterol should be estimated in plasma; if enhanced, screening for type of hyperlipidemia (FH or FD). If due to hyperlipidemia, complication with atherosclerotic cardiovascular disease may be expected.
Laser, excision, electrodesiccation, or topical application of trichloroacetic acid. Recurrences are not uncommon.
Synonyms: Xanthelasma palpebrarum, periocular xanthoma.
Figure 15-12. Xanthelasma Multiple creamy-orange, slightly elevated dermal papules on the eyelids of a normolipemic individual.
Xanthoma Tendineum ICD-9: 272.2 ICD-10: E78.500
These subcutaneous tumors are yellow or skin colored and move with the extensor tendons (Fig. 15-13).
They are a symptom of FH that presents as type IIa hyperlipidemia.
This condition is autosomal recessive with a different phenotype in the heterozygote and homozygote.
In the homozygote, the xanthomata appear in early childhood and the cardiovascular complications in early adolescence; the elevation of the LDL content of the plasma is extreme. These patients rarely attain ages above 20 years.
Management: A diet low in cholesterol and saturated fats, supplemented by cholestyramine or statins. In extreme cases, measures such as portacaval shunt or liver transplantation have to be considered.
Synonym: Tendinous xanthoma.
Figure 15-13. Tendinous xanthoma Large subcutaneous tumor adherent to the Achilles tendon.
Xanthoma Tuberosum ICD-9: 374.51 ICD-10: E78.230
This condition comprises yellowish nodules (Fig. 15-14) located especially on the elbows and knees by confluence of concomitant eruptive xanthomas.
They are to be found in patients with FD, familial hypertriglyceridemia with chylomicronemia (type V) and FH (Table 15-2).
In homozygous patients with FH, the tuberous xanthomas are flatter and skin colored. They are not accompanied by eruptive xanthomas (see below).
Management Treatment of the underlying condition.
Synonym: Tuberous xanthoma.
Figure 15-14. Tuberous xanthoma Flat-topped, yellow, firm nodule.
Eruptive Xanthoma ICD-9: 272.2 ICD-10: E78.2
These discrete inflammatory-type papules “erupt” suddenly and in showers, appearing typically on the buttocks, elbows, lower arms (Fig. 15-15), and knees.
A sign of FHT, FD, the very rare familial lipoprotein lipase deficiency (Table 15-2), and diabetes out of control.
Papules are dome shaped, discrete, initially red, then yellow center with red halo (Fig. 15-15).
Lesions may be scattered, discrete, in a localized region [e.g., elbows, knees (Fig. 15-15), buttocks] or appear as “tight” clusters that become confluent to form nodular “tuberoeruptive” xanthomas.
Management: React very favorably to a low-calorie and low-fat diet.
Figure 15-15. Papular eruptive xanthomas (A) Multiple, discrete, red-to-yellow papules becoming confluent on the knees of an individual with uncontrolled diabetes mellitus; lesions were also present on both elbows and buttocks. (B) Higher magnification of xanthomas on the trunk of another patient.
Xanthoma Striatum Palmare ICD-9: 272.2 ICD-10: E78.260
This condition is characterized by yellow-orange, flat or elevated infiltrations of the volar creases of palms and fingers (Fig. 15-16).
Pathognomonic for FD (type III) (Table 15-2). Next to xanthoma striatum palmare, FD also presents with tuberous xanthoma (Fig. 15-16) and xanthelasma palpebrarum (Fig. 15-12).
Patients with FD are prone to atherosclerotic cardiovascular disease, especially ischemia of the legs and coronary vessels.
Management: Patients with FD react very favorably to a diet low in fats and carbohydrates. If necessary, this may be supplemented with statins, fibrates, or nicotinic acid.
Figure 15-16. Xanthoma striatum palmare The palmar creases particularly over the interphalangeal joints, are yellow, often a very subtle lesion noticeable only upon close examination.
Normolipemic Plane Xanthoma
Xanthoma planum is a normolipemic xanthoma that consists of diffuse orange-yellow pigmentation and slight elevations of the skin (Fig. 15-17). There is a recognizable border.
These lesions can be idiopathic or secondary to leukemia, but the most common association is with multiple myeloma.
The lesions may precede the onset of multiple myeloma by many years.
Figure 15-17. Plane xanthoma Yellowish-red, slightly elevated plaques on the neck, noticeable mainly because of the accentuation of the skin texture in a normolipemic patient with lymphoma. Plane xanthomas occur most commonly on the upper trunk and neck and most commonly occur in individuals with myeloma.
Scurvy ICD-9: 267° ICD-10: E54
Scurvy is an acute or chronic disease caused by dietary deficiency of ascorbic acid (vitamin C).
Scurvy occurs in infants or children on a diet consisting of only processed milk or in edentulous adult persons who do not eat salads and uncooked vegetables.
Precipitating factors: Pregnancy, lactation, and thyrotoxicosis; most common in alcoholism.
Symptoms of scurvy occur after 1–3 months of vitamin C uptake. Lassitude, weakness, arthralgia, and myalgia.
Skin lesions: Petechiae, follicular hyperkeratosis with perifollicular hemorrhage, especially on the lower legs (Fig. 15-18A). Hair becomes fragmented and buried in these perifolliculár hyperkeratotic papules (corkscrew hairs); also, extensive ecchymoses (Fig. 15-18B), which can be generalized. Nails: splinter hemorrhages.
Gingiva: swollen, purple, spongy, and bleeds easily. Loosening and loss of teeth.
Hemorrhage occurring into periosteum of long bones and into joints → painful swellings and, in children, epiphyseal separation. Sternum sinks inward: scorbutic rosary (elevation at rib margins). Retrobulbar, subarachnoid, intracerebral hemorrhage can cause death.
Laboratory: Normocytic, normochromic anemia. Folate deficiency, resulting in macrocytic anemia. Positive capillary fragility test. Serum ascorbic acid level zero. X-ray findings are diagnostic.
Unless treated, scurvy is fatal. On treatment, spontaneous bleeding ceases within 24 h, muscle and bone pain fade quickly, bleeding from gums stops in 2–3 days.
Management: Ascorbic acid 100 mg three to five times daily until 4 g is given; then 100 mg/d is curative in days to weeks.
Figure 15-18. Scurvy (A) Perifollicular purpura on the leg. The follicles are often plugged by keratin (perifollicular hyperkeratosis). This eruption occurred in a 46-year-old alcoholic, homeless male, who also had bleeding gums and loose teeth. (B) These extensive ecchymoses occurred in an edentulous 65-year-old male who lived alone and whose food intake consisted mainly of biscuits soaked in water.
Acquired Zinc Deficiency and Acrodermatitis Enteropathica ICD-9: 269.9 ICD-10: E60
Acquired zinc deficiency (AZD) occurs in older individuals due to dietary deficiency or failure of intestinal absorption of zinc (malabsorption, alcoholism, prolonged parenteral nutrition).
Acrodermatitis enteropathica is a genetic disorder of zinc absorption. Autosomal recessive trait. It occurs in infants, bottle-fed with bovine milk, days to few weeks or in breast-fed infants, soon after weaning.
Skin Findings: Identical in AZD and AE. Patches and plaques of dry, scaly, sharply marginated and brightly red, eczematous dermatitis evolving into vesiculobullous, pustular, erosive, and crusted lesions (Figs. 15-19 and 15-20A). Initially occur in the perioral and anogenital areas. Later, scalp, hands and feet, flexural regions, trunk. Fingertips glistening, erythematous, with fissures and secondary paronychia. Perlèche. Lesions become secondarily infected with Candida albicans, S. aureus. Impaired wound healing.
Diffuse alopecia, graying of hair. Paronychia, nail ridging, and loss of nails.
Red, glossy tongue; superficial aphthous-like erosions; secondary oral candidiasis.
Photophobia; irritable, depressed mood. Children with AE whine and cry constantly. Failure of growth.
Anemia, low serum/plasma zinc levels; reduced urinary zinc excretion.
After zinc replacement, severely infected and erosive skin lesions heal within 1–2 weeks (Fig. 15-20B), diarrhea ceases, and irritability and depression of mood improve within 24 h.
Management Dietary or IV supplementation with zinc salts in two to three times the required daily amount restores normal zinc status in days to weeks.
Figure 15-19. Acquired zinc deficiency Well-demarcated, psoriasiform and eczematous-like plaques with scaling and erosions overlying the sacrum, intergluteal cleft, buttocks, and hip in a 60-year-old alcoholic female whose diet had consisted of pickles and cheap wine. She also had a similar eruption around the mouth, perlèche, atrophic glossitis, and had glistening, shiny, oozing fingertips.
Figure 15-20. Acrodermatitis enteropathica (A) Sharply demarcated, symmetric, partially erosive, scaly, and crusted plaques on the face of an infant after weaning. Similar lesions were also found in the perigenital and perianal regions and on the fingertips. The child was highly irritable, whining, and crying and had diarrhea. (B) Within 24 h after zinc replacement, the irritability and diarrhea ceased and the infant’s mood improved; and after 10 days (shown here), the perioral and perigenital lesions had healed.
Pellagra ICD-9: 265.2 ICD-10: E52
Pellagra arises from a diet deficient in niacin or tryptophan, or both. Tryptophan is converted in the body to niacin. A predominantly maize-based diet is usually implicated.
Pellagra is characterized by the three Ds: dermatitis, diarrhea, and dementia. Skin changes are determined by exposure to sunlight and pressure.
The disorder begins with a symmetric itching and smarting erythema on the dorsa of the hands, neck, and face. Vesicles and bullae may erupt and break, so that crusting occurs and lesions become scaly (Fig. 15-21A). Later, skin becomes indurated, lichenified, rough, covered by dark scales and crusts; there are cracks and fissures and a sharp demarcation from normal skin (Fig. 15-21B).
Distribution: dorsa of hands and fingers (“gauntlet”) (Fig. 15-21B), band-like around the neck (“Casal necklace”) (Fig. 15-21A), dorsa of feet up to malleoli with sparing of the heel, and butterfly region of the face.
Diagnosis is verified by detection of decreased levels of urinary metabolites.
100–300 mg niacinamide orally plus other B vitamins lead to complete resolution.
Figure 15-21. Pellagra (A) Scaly crusted band-like plaque on the neck (“Casal necklace”). (B) “Gauntlet” of pellagra; indurated, lichenified, pigmented, and scaly skin on the dorsa of the hands. Note sharp demarcation to lower arm.
Gout ICD-9: 274 ICD-10: M10
A clinical syndrome occurring in a group of diseases characterized by the deposition of monosodium urate crystals in synovial fluid and joints.
Acute gouty arthritis usually occurs in middle age and usually affects a single joint in the lower extremities, usually the first metatarsophalangeal joint. Can also affect fingers (Fig. 15-22A).
Intercritical gout describes the interval between attacks of gout. With time attacks tend to be polyarticular.
In chronic tophaceous gout, patients rarely have asymptomatic periods. Urate crystals are found in soft tissues, cartilage (Fig. 15-22B), and tendons.
Gout may occur with and without hyperuricemia, renal disease, and nephrolithiasis.
Figure 15-22. Acute gouty arthritis affecting (A) the distal interphalangeal joint of the fifth digit. (B) Gouty tophi on helix.