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Chronic Blistering Dermatoses Part 2

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1 Chronic Blistering Dermatoses Part 2
David M. Bracciano, D.O.

2 Pregnancy- Related Dermatoses
Intrahepatic Cholestasis of Pregnancy Polymorphic Eruption of Pregnancy Herpes (pemphigoid) gestationis purity Urticarial Papules and Plaques of Pregnancy (PUPPP) Papular Dermatitis of Pregnancy purity Folliculitis of Pregnancy

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4 Intrahepatic Cholestasis of Pregnancy
Generalized purities and jaundice No primary skin lesions, secondary excoriations Caused by cholestasis, occurs late in pregnancy, resolves after delivery 0.5% of pregnancies Tx; oral steroids

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6 Polymorphic Eruption of Pregnancy
Classification of all purity inflammatory dermatoses of pregnancy: Toxemic rash of pregnancy Pruigo annularis EM gestationis PUPPP purity Folliculitis of Pregnancy

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8 Polymorphic Eruption of Pregnancy
Pruritic inflammatory dermatoses of pregnancy occur in 1 of every 120 to 240 Treatment and prognosis is similar in subtypes

9 Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)
First reported in 1979 Erythematous papules and plaques that begin as 1-2 mm lesions within the abdominal striae Spread over the course of a few days to involve the abdomen, buttocks, thighs Upper chest, face, and mucous membranes spared

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11 PUPPP Lesions coalesce to form urticarial plaques
Intense pruritis is characteristic Primigravidas 75% of the time, usually does not recur with subsequent pregnancies Begins late in third trimester and resolves with delivery May be associated with increase weight gain

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13 PUPPP Histology: perivascular infiltrate in upper and mid dermis, epidermis normal Tx: topical or oral steroids

14 Papular Dermatitis of Pregnancy
Pruritic generalized eruption of 3-5 mm erythematous papule surmounted by a small, firm, central crust May erupt at any time during pregnancy and resolve with delivery Marked elevation of urine HCG Tx; oral steroids, may recur in subsequent pregnancies

15 Prurigo Gestationis (Besnier)
purity, excoriated papules of the proximal limbs and upper trunk Onset is weeks gestation Clears in postpartum period and does not recur Tx: topical steroids

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17 Pruritic Folliculitis of Pregnancy
2nd or 3rd trimester Small follicular pustules scattered widely over the trunk May be a type of hormonally induced acne

18 Impetigo Herpetiformis
Form of severe pustular psoriasis occurring in pregnancy Acute, usually febrile onset of grouped pustules on an erythematous base Begins in the groin, axillae, and neck Increased WBC, hypocalcemia Recurs with pregnancy, fetal death due to placental insufficiency Tx; prednisone 1mg/kg

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20 Cicatricial Pemphigoid (Benign Mucosal Pemphigoid)
Vesicles which quickly rupture, leaving erosions and ulcers with scarring Primarily occur on mucous membranes, conjunctiva (66%) and oral mucosa (90%) Oral mucosa may be the only affected site for years; desquamative gingivitis of buccal mucosa

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22 Cicatricial Pemphigoid
Tends to affect middle-aged to elderly women 2:1 female/male Ddx; oral lichen planus (biopsy and IF) Chronic disease that may lead to slowly progressive shrinkage of the ocular mucous membranes and blindness Also occurs in pharynx, esophagus, larynx, nose, penis, vagina, anal mucosa, deafness

23 Cicatricial Pemphigoid
Cutaneous lesions in 25%; tense bullae Bullae heal with or without scarring, occur on the face, scalp, neck, and inguinal region and extremities Some pts may have antibodies targeted against classic bullous pemphigoid antigens and should be classified as “mucosal predominate bullous pemphigoid” Chronic course, pts health not usually affected IgA antibodies may explain mucosal scarring tendency

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25 Cicatricial Pemphigoid
Little tendency to remission (unlike bullous pemphigoid) Subtypes include types that target basement membrane zone antigens (laminin, glycoproteins, )

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28 Cicatricial Pemphigoid
Direct IF testing C3 and IgG at the lamina lucida in 80-95% Tx: mild cases topical steroids (Temovate/Orabase), intralesional triamcinolone every 2-4 weeks Tx: Dapsone, prednisone, Azathioprine or cyclophosphamide

29 Epidermolysis Bullosa Acquisita
Antibodies to Type VII collagen Skin fragility, healing with scars Bullous eruption, scaring, milia Need to exclude all other bullous diseases: porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, and bullous drug eruption

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31 Epidermolysis Bullosa Acquisita
Tx; unsatisfactory, steroids, dapsone, colchicine, IV Immunoglobulin, Cyclosporin

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36 Dermatitis Herpetiformis
Chronic, relapsing, severely purity disease Grouped symmetrical, polymorphous, erythematous-based lesions May be papular, papulovesicular, vesiculobullous, bullous, or urticarial Itching and burning are intense Spontaneous remissions lasting a week

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38 Dermatitis Herpetiformis
Eruption usually symmetrical Scalp, nuchal area, posterior axillary folds, sacral region, buttocks, knees, forearms Pruriginous papules are a common feature Vesicles are more common than bullae; however all types of these lesions may be present in one patient Course of the disease is generally lifelong, with prolonged remissions being rare

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40 Dermatitis Herpetiformis
Very few patients with DH ever have diarrhea although DH is associated with Gluten-sensitive-enteropathy (GSE) 87% of pts with DH and IgA deposits in the skin are HLA-B8 positive (like GSE) Gluten is a protein found in cereals except for rice, oats, and corn IgA antibodies are formed in the jejunum, may deposit in the skin

41 Dermatitis Herpetiformis
Associated with; Thyroid disorders, small bowel lymphoma, non-Hodgkins lymphoma 70% of pts have abnormalities of the jejunal mucosa Gluten-free diet decreases Dapsone dose requirements after 3-4 months

42 Dermatitis Herpetiformis
Ddx: pemphigoid, EM, scabies, contact dermatitis, atopic dermatitis, eczema, insect bites, pruigo nodularis IgA in a granular pattern in the dermal papillae in normal skin is specific and pathognomonic for DH

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45 Dermatitis Herpetiformis
IgA deposits may be focal, so multiple biopsies may be needed. Deposits of the antibody are more often seen in previously involved skin or normal appearing skin adjacent to involved skin

46 Dermatitis Herpetiformis
Equal male:female Onset between 20 to 40 years Tx: Dapsone mg daily (hemolytic anemia, methemoglobinemia, check G6PD prior to tx) monitor Hct,WBCs, LFTs Tx: Sulfapyridine 0.5g QID to 2-4g/day Gluten-free diet will decrease need for meds or allow pt to go off them Celiac Society

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48 Linear IgA Bullous Dermatosis
Subepidermal blisters, a neutrophillic infiltrate, circulating IGA antibasement membrane zone antibody Deposition of IgA antibody at the dermoepidermal junction by direct IF

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51 Linear IgA Bullous Dermatosis Adult Form
Acquired autoimmune blistering disease Clinical pattern similar to dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance 50% mucous membrane involvement Oral and conjunctival lesions may be scarring No association with enteropathy or with HLA-B8 Tends to remit over several years

52 Linear IgA Bullous Dermatosis Adult Form
Linear IgA dermatosis can occur as a drug-induced disease: Self-limited, less mucosal involvement, usually does not have circulating autoantibody IgA is usually deposited in the subbasal lamina area Vanco, Lithium, amiodarone, captopril, PCN, lasix, dilantin, and others

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54 Linear IgA Bullous Dermatosis Adult Form
Histo: papillary dermal microabscess with neutrophils, subepidermal bullae may be seen with neutrophils and eosinophils Direct IF: homogeneous linear deposition of IgA is present at the BMZ Indirect IF: few will have circulating IgA autoantibody with anti-BMZ specificity Tx: Dapsone, topical steroids

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57 Linear IgA Bullous Dermatosis Childhood Form
Chronic Bullous Disease of Childhood: acquired, self-limited bullous disease Onset by 2 or 3, remits by age 13 Bullae develop on erythematous or normal appearing skin Trunk, buttocks, genitalia, and thighs Perioral and scalp lesions are common, oral lesions not uncommon Bullae arranged in a rosette or annular array “cluster of jewels”

58 Linear IgA Bullous Dermatosis Childhood Form
Histo: subepidermal bullae filled with neutrophils, eosinophils may predominate Direct IF: linear deposition of IgA at the BMZ Indirect IF: positive for circulating IgA antibodies in 50% Tx: Sulfapyridine or dapsone, topical steroids

59 Transient Acantholytic Dermatosis
Over age 50, fragile vesicles, limited extent, sparse, limited duration Rapid crusting, keratotic erosion <1cm Usually chest, shoulder Direct IF is negative Tx: topical steroids, isotretinoin

60 Nutritional Diseases Andrews Chapter 22
David M. Bracciano, D.O.

61 Nutritional Diseases Caused by insufficiency or excess of dietary essentials Common in underdeveloped countries, infants and children Often pts have features of several disorders if diet is generally restricted Alcoholism is the main cause in developed countries

62 Nutritional Diseases Postoperative pts, psychiatric pts (anorexia nervosa, bulimia), surgical or inflammatory bowel dysfunction, Crohn’s

63 Hypovitaminosis A (Phrynoderma)
Vitamin A: fat soluble found in milk, fish oil, liver, eggs, and as carotenoids in plants Common in children in developing world Developed countries found in diseases of fat malabsorption; Crohn’s, celiac, cystic fibrosis, cholestatic liver disease Vitamin A required for keratinization of mucosal surfaces

64 Hypovitaminosis A Abnormal keratinization leads to increased mortality from inflammatory disease of the gut and lung ie; diarrhea and pneumonia Phrynoderma or “toadskin” resembles keratosis pilaris. Keratotic papules over extremities and shoulders arising from pilosebaceous follicles

65 Hypovitaminosis A Eruption begins on thighs or upper arms. Spreads to shoulders, abdomen, back, and buttocks, face and neck Skin displays dryness and scaling

66 Hypovitaminosis A Ocular Findings
Major cause of blindness in children in the developing world! Earliest finding is delayed adaptation to the dark (nyctalopia) Night blindness, xeropthalmia, xerosis corneae, keratomalacia Bitot’s Spots; circumscribed areas of xerosis of the conjuctiva lateral to the cornea

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68 Hypovitaminosis A Diagnosis: based on eye findings, serum Vitamin A level. Tx: 300,000 IU Vitamin A

69 Hypervitaminosis A Skin findings similar to side effects of Retinoid therapy. Children are at greater risk. Loss of hair and coarseness, loss of eyebrows, exfoliation and pigmentation of skin, clubbing, hepatosplenomegaly, anemia, increased LFTs, pseudotumor cerebri with papilledema

70 Hypervitaminosis A Adults
Early signs are dryness of the lips and anorexia. Followed by bone and joint pains, follicular hyperkeratosis, branny desquamation of the skin, loss of scalp hair and eyebrows, dystrophy of the nails. Fatigue, myalgia, depression, anorexia, liver disease Birth defects with excess Vit A in pregnancy

71 Vitamin D Deficiency of Vitamin D causes alopecia, osteomalacia
Vitamin D overdose can cause hypercalcemia and calcinosis.

72 Vitamin E Deficiency Most common in infants of low birth weight
Peripheral edema, progressive neuromyopathy, and ophthalmoplegia

73 Vitamin K Deficiency Dietary deficiency of vitamin K, a fat soluble vitamin, does not occur in adults because it is synthesized by bacteria in the large intestine Liver disease causes deficiency Drugs: coumadin, salicylates, cholestyramine

74 Vitamin K Deficiency Decrease in the vitamin K-dependent clotting factor II, VII, IX, and X. Purpura, hemorrhage, and ecchymosis. Tx: 5 to 10 mg/day IM Vit K for 2-3 days

75 Vitamin B1 Deficiency Thiamine deficiency results in Beriberi
Edema, and peripheral neuropathy

76 Vitamin B2 Deficiency Riboflavin deficiency is seen most often in alcoholics. Phototherapy for neonatal icterus, boric acid ingestion, hypothyroidism, chlorpromazine Oral-ocular-genital Syndrome: angular chelitis, atrophic tongue, photophobia, blepharitis, confluent dermatitis of scrotum Tx: 5mg Riboflavin qd

77 Vitamin B6- Pyridoxine Deficiency: occurs in uremia and cirrhosis
Seborrheic dermatitis, glossitis, chelitis, conjunctivitis, confusion, neuropathy Excess: subepidermal vesicular dermatosis, peripheral sensory neuropathy

78 Vitamin B12 Deficiency Cyanocobalamin
Absorbed through the distal ileum after binding to gastric intrinsic factor in an acid ph. Deficiency caused by: decreased intrinsic factor, achlorhydria, malabsorption syndromes (pancreatic, sprue) Because of large body stores in adults, deficiency occurs 3 to 6 years after onset of GI disease!

79 Vitamin B12 Deficiency Glossitis, hyperpigmentation accentuated in exposed areas resembling Addison’s disease Megaloblastic anemia, weakness, paresthesias, ataxia Tx: IM B12, neuro defects may not improve

80 Folic Acid Deficiency Diffuse hyperpigmentation, glossitis, chelitis, and megaloblastic anemia

81 Scurvy Vitamin C Deficiency
Most common vitamin deficiency dxd by dermalologists Elderly alcoholics and psychiatric pts

82 Scurvy “The Four H’s” Hemorrhagic signs
Hyperkeratosis of the hair follicles Hypochondriasis Hematologic abnormalities

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84 Scurvy Perifollicular petechiae and ecchymoses, subungual, subconjunctival, intramuscular, and intraarticular hemorrhage “Corkscrew hairs”; hairshafts are curled in follicles capped by keratotic plugs Hemorrhagic gingivitis; bleeding gums, epistaxsis, anemia

85 Scurvy Dx: serum ascorbic acid level
Tx: ascorbic acid mg qd x 1 week

86 Niacin Deficiency Pellagra
Nicotinic acid, vitamin B3, niacin or its precursor tryptophan is associated with a diet entirely composed of corn, millet or sorghum Other vitamin defficiencies or malnutrition coexist Most cases are alcoholics in developed countries

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88 Pellegra Causes Carcinoid tumors, which divert tryptophan to serotonin
Intestinal parasites esp; hookworm GI diseases ie; Chron’s IV alimentation Anorexia nervosa Meds; Isoniazid, azathioprine, 5-FU, Hydantoins

89 Pelegra Chronic disease affecting GI tract, CNS, skin
“3 D’s”; diarrhea, dementia, dermatitis Dermatitis: photosensative eruption, perineal lesions, thickening and pigmentation over boney prominences, seborrheic dermatitis-like eruption on face

90 Pellegra Photosensitive eruption on face, neck, chest
(Casal’s necklace), eruption may be vesicular or bullous (wet pellegra) After several phototoxic events the skin shows hyperpigmentation, scaling, a copper hue Scrotal and perineal erosions, fissures, angular chelitis

91 Pellegra CNS and GI symptoms may occur without skin changes; apathy, muscle weakness, parasthesias, dizziness, psychosis Disease is progressive, majority of pts die in 4-5 years if untreated

92 Pellegra Diagnosis and Treatment
Diet: Animal protiens, eggs, milk, vegetables 100mg nicotinamide qid Skin lesions begin to resolve within 24 hours of tx

93 Biotin Deficiency Biotin is universally available and is produced by intestinal bacteria Deficiency is rare, can occur in short gut or malabsorption Dermatitis is perioral; pathcy, red, eroded lesions on the face and groin Candida overgrowth of lesions occurs

94 Biotin Alopecia including loss of eyebrows and eyelashes
Neuro: depression, lethargy, parasthesias Infants: hypotonia, lethagry seizures, developmental delays Inherited form: detecting organic aminoaciduria with 3-hydroxyisovaleric acid Tx: 10mg Biotin qd Skin lesions resolve rapidly, but neuro damage may be permenant

95 Zinc Deficiency Inherited or Aquired
Inherited: Acrodermatitis enteropathica Premies at risk due to inadequate body zinc stores Weaning from breast from breast milk precipitates clinical zinc deficiency Parental nutrition without adequate zinc content may contribute

96 Zinc Acquired: alcoholics, bowel disease, anorexia, AIDS
Zinc requirements increase with metabolic stress Diets containing mainly cereal grains are high in phytate, which binds zinc, Middle East, North Africa

97 Zinc Dermatitis Pustular and bullous, acral and perioral
Patchy, red, dry, scaling with exudation and crusts. Angular chelitis and stomatitis Nail dystrophy, alopecia Diarrhea, growth retardation, CNS Histo: vacuolation of the keratinocytes of the upper stratum malpighii

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99 Zinc Deficiency Diagnosis and Treatment
Characteristic skin findings, acral or perioral dermatitis Chronic diaper rash with diarrhea in an infant should lead to evaluation for zinc deficiency Diagnosis: low serum zinc, alkaline phosphatase Tx: zinc sulfate 1-2 mg/kg/day Tx: acrodermatitis enteropathica is lifelong

100 Essential Fatty Acid Defficiency
Lbw infants, bowel disease, alimentation Dermatitis similar to zinc def : xerosis, EFA’s constitute 25% of the fatty acids of the stratum corneum Widespread erythema, intertriginous weeping eruption, infection, alopecia Decrease in linoleic acid and an increase in palmitoleic and oleic acids

101 EFA Deficiency Ratio of eicosatrienoic acid to arachidonic acid of >0.4 is diagnostic Tx: Intralipid 10% IV

102 Iron Deficiency Common in menstration
Mucocutaneous; glossitis, angular chelitis, pruitus, telogen effluvium Plummer-Vinson syndrome: microcytic anemia, dysphagia, glossitis (middle aged women) thin lips, narrow mouth, koilonchia in 50% Post-cricoid esophageal web

103 Iron Deficiency Diagnosis: serum iron (Fe+)
Tx: iron sulfate 325 mg tid

104 Selenium Deficiency IV alimentation, poor soil selenium content, lbw infants Children: hypopigmentation of skin and hair (psuedoalbinism), leukonychia Cardiomyopathy, muscle pain, elevated muscle enzymes (cpk) Tx: 3 ug/kg/day selenium

105 Protein-energy Malnutrition
Spectrum of diseases: marasmus, kwashiorkor, and marasmic kwashiorkor Endemic in developing world Marasmus; def of protein and calories, children < 60% of IBW without edema Kwashiorkor; protein def, 60-80% of IBW with edema or hypoproteinemia

106 Marasmus/ Kwashiorkor
Cystic fibrosis, dietary restrictions Marasmus: skin is dry, wrinkled, loose “Monkey facies”; due to lose of buccal fat pad, no edema Kwashiorkor; edema, potbelly, hair and areas of skin are hypopigmented, hair is red, gray to white

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109 Kwashiorkor Africans call them “Red Children”
“Flag Sign”; alternating bands of pale and dark hair along a single strand correspond to periods of good and poor nutrition “Mosaic skin”; areas of hyper/hypopigmentation resemble peeling paint

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112 Carotenemia and Lycopenemia
Excessive ingestion of : carots, oranges, squash, spinach, turnips, corn, beans, butter, eggs, pumpkins, sweet potatoes, papaya (seen in Kirksville) Yellowish discoloration of skin, palms, soles, central face Carotenemia occurs in vegitarians Lyconpenemia; red foods, beets, tomatoes, chili beans (flatulence), berries leads to reddish discoloration of skin aka “K.C. Chiefs’ syndrome


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