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dermatology > 4) Multifocal Eosinophilic Granuloma > Flashcards

4) Multifocal Eosinophilic Granuloma Flashcards

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Dermatology Board Prep flashcards
1
Q

Types of MEG

A
  • Porphyria
  • Ascorbic acid deficiency
  • Zinc deficiency
  • Niacin deficiency
  • Hypercorticism
  • Adrenal cortical insufficiency
  • Hyper/hypothyroidism
  • Xanthomas
  • Neurofibromas
  • Tuberculosis sclerosis
  • Venous insufficiency and related
  • DM & related
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2
Q

DM & related MEG types

A
  • Diabetic dermopathy
  • Necrobiosis lipoidica diabeticorum
  • Diabetic bullae
  • Acanthosis nigricans
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3
Q

Porphyria

A
  • 1:25,000 in US, 1:1 female to male, generally adults
  • Deficiency or mutation in uroporphyrinogen decarboxylase (UROD)
  • Hereditary 20% vs Sporadic 80%
  • Blistering photosensitivity
  • Elevated plasma and urine total porphyrins
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4
Q

Porphyria cutanea tarda (PCT)

A
  • Most common type of cutaneous porphyria while hepatoerythropoietic porphyria (HEP) is very rare with only 40 documented cases
  • Iron-related and generally presents in adulthood while
    HEP can present in children or adults depending on the degree of UROD deficiency
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5
Q

PCT classifications

A
  • Inherited or sporadic
  • Approximately 20 and 80 percent of cases, respectively
  • Based on the presence or absence of heterozygous UROD mutations
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6
Q

PCT leads to

A
  • Excess of porphyrins which are transported from the liver to the skin
  • Leads to cutaneous phototoxicity
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7
Q

Acquired susceptibility factors for porphyria

A
  • Alcohol use
  • Smoking
  • Estrogen use
  • Hepatitis C virus or HIV infection are common
  • Hemochromatosis mutations
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8
Q

Typical features of porphyria

A
  • Chronic blistering photosensitivity commonly on the backs of the hands and other sun-exposed areas
  • Can lead to infection, bullae, increased skin fragility, scarring, hyper- and hypopigmentation
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9
Q

Porphyria lesions

A
  • May be pruritic and/or painful depending on stage and degree of severity
  • Delayed cutaneous reaction to sun exposure which may result in the patient not being aware that the exposure is causing
    the skin lesions
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10
Q

Porphyria diagnosis

A
  • First line testing with measurement of plasma or urinary total porphyrins before more extensive testing
  • Biopsy is not necessary unless trying to rule out another condition
  • Once Dx is made, it is appropriate to have family members tested for UROD and/or hereditary hemochromatosis
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11
Q

Porphyria DDx

A
  • Other cutaneous porphyrias which may or may not blister
  • Epidermolysis bullosa
  • Pseudoporphyria
  • Phototoxic drug reactions and eruptions
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12
Q

Porphyria treatment

A
  • Phlebotomy or low-dose hydroxychloroquine
  • Reduction of susceptibility factors
  • Choice between phlebotomy and hydroxychloroquine depends on the degree of iron overload and other susceptibility factors
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13
Q

Ascorbic acid deficiency

A
  • Vitamin C deficiency known as “Scurvy”

- Essential dietary nutrient in humans

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14
Q

Ascorbic acid deficiency characteristics

A
  • Ecchymosis and bleeding gums, perifollicular hemorrhage, petechiae and coiled hairs
  • Symptoms are due to impaired collagen synthesis and disordered connective tissue secondary to deficiency
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15
Q

Ascorbic acid deficiency occurence

A
  • Mostly in severely malnourished individuals, drug and alcohol abusers, or those living in poverty or on diets devoid of fruits and vegetables
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16
Q

Ascorbic acid deficiency symptoms

A
  • Generally occur when the plasma concentration of ascorbic acid is less than 0.2 mg/dL
  • Most specific symptoms, as early as three months, are follicular hyperkeratosis and perifollicular hemorrhage, with petechiae and coiled hairs
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17
Q

Common symptoms associated with ascorbic acid deficiency

A
  • Ecchymosis
  • Gingivitis (with bleeding and receding gums and dental caries)
  • Sjogren’s syndrome
  • Arthralgias, edema, anemia, and impaired wound healing
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18
Q

Hemorrhagic skin lesions associated with ascorbic acid deficiency

A
  • Initially flat but may coalesce and become palpable

- Especially on the lower extremities which may resemble a systemic vasculitis

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19
Q

Musculoskeletal pain associated with ascorbic acid deficiency

A
  • May be severe

- Can be caused by hemorrhage into the muscles of periosteum

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20
Q

Generalized systemic responses of ascorbic acid deficiency

A
  • Weakness
  • Malaise
  • Joint swelling, arthralgias
  • Anorexia
  • Depression
  • Neuropathy
  • Vasomotor instability
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21
Q

Ascorbic acid deficiency imaging

A
  • Sclerotic and lucent metaphyseal bands with periosteal reaction
  • Adjacent soft-tissue edema
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22
Q

Ascorbic acid deficiency diagnosis

A
  • Clinically and usually related to a direct history of vitamin C deficiency
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23
Q

Ascorbic acid deficiency treatment

A
  • Vitamin C supplementation and reversal of the conditions that led to the deficiency
  • Adults are usually treated with 300 to 1000 mg daily for one month
  • Children recommended doses are 100 mg ascorbic acid given three times daily for one week, then once daily for several weeks until the patient is fully recovered
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24
Q

Ascorbic acid deficiency symptom resolution

A
  • Many of the constitutional symptoms improve within 24 hours of treatment
  • Bruising and gingival bleeding resolve within a few weeks
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25
Q

Zinc deficiency

A
  • Mild deficiency is characterized by impaired growth velocity
  • Severe depletion leads to growth retardation
  • Dermatologic changes occur primarily in the extremities or around body orifices
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26
Q

Zinc biological role

A
  • Forming tight bonds with certain amino acids

- Structural role in proteins

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27
Q

Zinc deficiency in chronic diseases

A
  • Malnutrition (elderly, alcoholism, etc.)
  • Malabsorption syndromes (gastric bypass, TPN, etc.)
  • Diabetics due to alteration in zinc metabolism
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28
Q

Clinical manifestations of zinc deficiency

A
  • Delayed sexual maturation, impotence, hypogonadism, oligospermia
  • Alopecia
  • Dysgeusia (impaired taste)
  • Immune dysfunction
  • Night blindness
  • Impaired wound healing and various skin lesions
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29
Q

Zinc deficiency lesions

A
  • Erythematous, vesiculobullous, and pustular lesions
30
Q

Zinc deficiency treatment

A
  • Supplementation is the mainstay of treatment
  • Ranges from approximately 4 to 14 mg/day orally
  • Good sources of zinc are in animal products such as meat, seafood, and milk while cereal contains the greatest amount of zinc for vegetarians/vegans
31
Q

Acrodermatitis enteropathica (AE)

A
  • Autosomal recessive disease in which zinc absorption is impaired
  • Characterized by diarrhea, dermatitis, alopecia, poor growth, and poor immune function
32
Q

Zinc deficiency dermatitis

A
  • Consists of hyperpigmented skin lesions on the acral surfaces of the upper and the lower extremities, as well as the face and buttocks
33
Q

Zinc deficiency onset

A
  • Symptoms usually appear in early infancy, once breast-feeding is completely discontinued
34
Q

Niacin deficiency

A
  • Causes pellagra (raw skin)
  • Commonly occurs in alcoholics
  • Characterized by a photosensitive pigmented dermatitis
  • Red tongue and many non-specific symptoms
35
Q

Niacin deficiency characteristics

A
  • Symmetric hyperpigmented rash, similar in color and distribution to a sunburn, which is present in the exposed areas of skin
  • Red tongue, diarrhea and vomiting
  • Neurologic symptoms including insomnia, anxiety, disorientation, delusions, dementia, and encephalopathy
36
Q

Niacin deficiency may be seen in

A
  • Carcinoid syndrome
  • Prolonged isoniazid use
  • Hartnup disease (due to effect on tryptophan)
37
Q

Niacin deficiency supplementation

A
  • 6 to 12 mg daily in children
  • 16 mg for adult males
  • 14 mg daily for non-pregnant adult females
38
Q

Hypercortisolism characteristic findings

A
  • Easy bruisability (loss of subcutaneous connective tissue)
  • Purple striae (fragile skin stretches due to enlarging trunk)
  • Skin atrophy (thinning stratum corneum, loss of subcutaneous fat)
  • Cutaneous fungal infections (especially tinea versicolor)
39
Q

Cushing syndrome

A
  • Hypercortisolism
40
Q

Hypercortisolism characteristic findings (continued)

A
  • Hyperpigmentation
  • Acanthosis nigrans
  • Surgical or traumatic scars
41
Q

Hyperpigmentation in hypercortisolism

A
  • Induced by increased ACTH secretion
  • Most noted in conspicuous areas such as face, neck, and hands or areas of friction or pressure such as waist, elbows, etc.
42
Q

Acanthosis nigrans in hypercortisolism

A
  • Present in the axillae and

around the neck (also seen in 40% of Type 2 DM)

43
Q

Surgical or traumatic scars in hypercortisolism

A
  • Form when plasma ACTH concentrations are markedly elevated are permanently pigmented
  • In comparison, scars incurred before ACTH hypersecretion or after it is reduced are not pigmented
44
Q

Adrenal insufficiency (Addison’s) hyperpigmentation

A
  • Excess ACTH resulting in brown pigmentation
  • Distribution similar to Cushing’s
  • Noted in conspicuous areas such as face, neck, and hands or areas of friction or pressure (waist, elbows, etc.)
  • Patchy pigmentation on inner surface of lips and buccal mucosa
  • Scars are also hyperpigmented
45
Q

Autoimmune primary adrenal insufficiency

A
  • Patchy and often bilaterally symmetrical areas of depigmented skin (vitiligo) occur in 10-20% of patients about the trunk or extremities
46
Q

Hypothyroidism clinical features

A
  • The skin is cool and pale because (decreased blood flow)
  • Epidermis has atrophied cellular layer and hyperkeratosis (dry roughness)
  • Yellowish tinge (if carotenemia)
  • Hyperpigmentation (primary hypothyroidism associated with primary adrenal failure)
  • Hair may be coarse, hair loss is common
  • Brittle nails
  • Nonpitting edema (myxedema) occurs in severe hypothyroidism
47
Q

Nonpitting edema (myxedema) in hypothyroidism

A
  • Occurs in severe hypothyroidism and may be generalized

- Results from infiltration of the skin with glycosaminoglycans with associated water retention

48
Q

Hypothyroidism clinical features (continued)

A
  • Skin is warm (increased blood flow)
  • Hyperpigmentation (ACTH secretion)
  • Thinning hair, onycholysis, softening nails
  • Vitiligo and alopecia
49
Q

Grave’s Hyperthyroidism

A
  • Pruritis and hives
  • Infiltrative dermopathy most commonly overlying the shins
  • Presents as raised, hyperpigmented, biolaceous orange peel textures papules
50
Q

Diabetic dermopathy

A
  • Approximately one-half of diabetic patients presents with multiple asymptomatic, round, dull red to pink papules or plaques predominantly located on the pretibial skin
51
Q

Diabetic dermopathy lesions

A
  • Evolve in one to two weeks to well-circumscribed, atrophic, brown macules and patches, often with fine scale
  • Older lesions show epidermal atrophy and scattered hemosiderin deposits
52
Q

Diabetic dermopathy Dx/Tx

A
  • Dx is clinical and biopsy is not routinely done
  • There is no treatment
  • Lesions may resolve spontaneously over time or persist indefinitely
53
Q

Necrobiosis Lipoidica Diabeticorum

A
  • Rare, chronic granulomatous disease (commonly involving the lower legs, especially the shins)
  • Lesions begin as red-brown or violaceous papules or nodules and progresses to yellow-brown, atrophic, telangiectatic plaques
  • Ulceration often followed minor trauma
54
Q

Necrobiosis Lipoidica Diabeticorum Dx/Tx

A
  • Clinical and punch biopsy to confirm

- Tx for nonulcerated: corticosteroid followed by intranasal therapy in case of insufficient response

55
Q

Diabetic bullae

A
  • Bullous disease of diabetes is a term used to describe the abrupt development of noninflammatory, tense, subepidermal bullae in patients with diabetes in sites of otherwise normal-appearing skin
56
Q

Diabetic bullae lesions

A
  • Most commonly occur on the feet or lower legs
  • May be up to several centimeters in diameter and are non painful
  • Bullae spontaneously resolve over the course of a few weeks
57
Q

Xanthomas

A
  • Eruptive xanthomas are 1 to 5 mm erythematous to yellow papules
  • Appear in crops and have an abrupt onset most commonly on extensor surfaces of the extremities and buttocks
58
Q

Cutaneous xanthomas characteristic histology

A
  • Lipid- laden macrophages, also known as “foam cells”
  • Eruptive type has prominent extracellular lipid deposition
  • Tuberous type have large foam cells with fibrosis
59
Q

Tuberous xanthomas

A
  • Solitary or grouped
  • Can reach sizes up to 3 cm
  • Yellow-orange or erythematous papules or nodules located over joints or extensor surfaces of the extremities (especially the elbows and knees)
60
Q

Xanthomas Tx

A
  • Pharmacologic treatment of dyslipidemia is usually indicated
  • Often leads to concomitant improvement in eruptive and tuberous xanthomas caused by hyperlipidemia
61
Q

Xanthoma resolution

A
  • Eruptive: several weeks when triglyceride levels are reduced
  • Tuberous and tendinous: slower to regress during treatment of dyslipidemia
62
Q

Neurofibromatosis

A
  • Type 1 with café-au-lait macules, freckling, Lisch nodules, and neurofibromas
  • Discrete cutaneous neurofibromas most common
  • Plexiform neurofibromas may be located superficially and associated with overgrowth of skin and soft tissues
  • 70% with NF2 have cutaneous manifestations
63
Q

The characteristic skin findings that contribute to establishing the diagnosis of neurofibromatosis

A
  • Six or more cafe-au-lait macules of greatest diameter
    >5 mm in prepubertal and >15 mm in postpubertal individuals
  • Two or more neurofibromas of any type or one plexiform neurofibroma
  • Freckling in the axillary or inguinal regions
64
Q

Tuberous sclerosis complex (TSC)

A
  • An inherited neurocutaneous disorder

- Characteristic skin lesions are seen in 81-95% of patients

65
Q

Tuberous sclerosis complex characteristics

A
  • Hypopigmented macules (ash-leaf spots) usually elliptic in shape
  • Angiofibromas (malar region of face)
  • Ungual fibromas
  • Shagreen patches (lower back)
  • Distinctive brown fibrous plaque on the forehead
66
Q

TSC brown fibrous plaque on forehead

A
  • May be the first and most readily recognized feature

- Physical examination of affected neonates and infant

67
Q

Venous insufficiency

A
  • Chronic lower extremity venous disease
  • Most common vascular disorder presenting with abnormal venous dilation (ie, telangiectasias, reticular veins, varicose veins), edema, inflammation, dermatitis, or ulceration
68
Q

Venous insufficiency pigmentation changes

A
  • Secondary to hemosiderin deposition
  • Initially most prominent at the medial ankle
  • May subsequently encroach upon the foot and lower leg
69
Q

Venous insufficiency pruritis

A
  • Dry, scaly skin that can be difficult to relieve

- Excoriations are often present secondary to scratching (can be source of infection)

70
Q

Venous insufficiency severe cases

A
  • Lipodermatosclerosis with brawny edema or even ulceration may develop
71
Q

Venous insufficiency treatment

A
  • Compression therapy along with symptomatic support
  • Leg elevation, exercise, and pharmacological therapy
  • Venous ablation and sclerotherapy

dermatology (5 decks)

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