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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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