Dermatology Pearls in Paradise for the American Board of Dermatology certification and recertification exams

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Dermatology Pearls in Paradise for the American
Board of Dermatology certification and
recertification exams

• Brendan Thomas, M.D.
• Board-certified dermatologist
• Dreyer Medical Group
• Aurora, Illinois
• info_at_brendanthomas.us

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Objectives

• 30 high-yield pearls
• What I did to prepare

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• For every pearl at the bottom of the ocean,
  there is a ton of whale shit.
• Ernest Lee, M.D.

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1. Ipilimumab

• Mechanism of action
• Blocks CTLA-4 receptor on cytotoxic T
  lymphocytes, preventing their inhibition,
  allowing them to destroy melanoma cells
• Adverse effects include
• Facial swelling
• Colitis
• Also known as CD152
• Normally CTLA-4 stimulation inhibits T-cells

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2. Vemurafenib

• Mechanism of action
• B-Raf protein inhibitor
• Indication
• Treatment of BRAF V600E mutation positive
  unresectable or metastatic melanoma
• Also known as PLX4032 and by the brand name
  Zelboraf
• Substitution of valine (V) with glutamic acid
  (E) at codon 600

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3. Dimethylaminopropylamine

• DMAPA is used in the formation of cocamidopropyl
  betaine
• Cocamidopropyl betaine is found in liquid soaps
  and shampoos
• DMAPA remains in products as contaminant
• DMAPA is important cause of eyelid dermatitis

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4. Angiopoietin receptors

• Tie-1 and Tie-2 are cell-surface receptors
• Angiopoietins bind these receptors and promote
  angiogenesis
• There are four angiopoietins
• Serum Ang2 levels are elevated in patients with
  angiosarcoma
• Mutations that lead to dysfunction of the Tie-2
  receptor are associated with vascular
  malformations

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5. Viral-associated trichodysplasia

• Closely linked with organ transplantation,
  immunosuppression, and pre-B cell leukemia
• Follicular spiny papules erupt on face
• Likely due to a polyomavirus
• Treat with valganciclovir
• Cyclosporin often cited
• A dsDNA virus and member of papovavirus family

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6. Merkel cell carcinoma

• Merkel cell polyomavirus (MCPyV)
• Wide local excision
• Sentinel lymph node biopsy in all cases
• Adjuvant radiotherapy for lesions on head and
  neck with locoregional spread

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7. Coding excision size

• Malignant lesions are excised with margins
• Diameter of resulting surgical defect (lesion
  margins) termed excised diameter
• Excised diameter used to select proper CPT code
  to bill excision
• A 6mm BCC on back excised with 4mm margins would
  result in a 1.4cm excised diameter (11602)

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8. Disseminated infundibulofolliculitis

• Young dark-skinned patients
• Uniform 12mm papules on neck and upper trunk
• Figure 33-31 in Andrews 10th edition on page 776
• Treat with topical steroids, PUVA or isotretinoin

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9. Syringomas

• Occur not just on face but also neck, chest,
  axillae, upper arms, and periumbilically
• Figure 111.22B in Bolongias 2nd edition on page
  1704
• Figure 29-36 in Andrews 10th edition on page 664
• Associated with Down syndrome, NicolauBalus
  syndrome, and Brooke-Spiegler syndrome

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10. Lupus miliaris disseminatus faciei

• Red-to-yellow papules on central face and around
  eyelids
• Histology shows single superficial BB-like nodule
• Central caseation necrosis
• http//img.medscape.com/pi/emed/ckb/dermatology/10
  48885-1070740-268.jpg

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11. Defects in keratin 5

• Epidermolysis bullosa simplex
• DowlingDegos' disease
• Olmsted syndrome
• For list of cutaneous conditions caused by
  mutations in keratins see http//en.wikipedia.org
  /wiki/List_of_cutaneous_conditions_caused_by_mutat
  ions_in_keratins
• For more lists (some good, some not so good) of
  board-related fodder see http//en.wikipedia.org/
  wiki/List_of_cutaneous_conditionsSee_also
• May be due to mutation in gene encoding keratin
  5 and/or 14

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12. Migrating conditions

• Creeping eruption (Cutaneous larva
  migrans) 2cm/day
• Larva migrans profundus (Gnathostomiasis) 1cm/hour
  
• Larva currens
• 10cm/day
• Erythema gyratum repens
• 1cm/day

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13. SchöpfSchulzPassarge syndrome

• Ectodermal dysplasia
• Diffuse symmetric palmoplantar keratoderma
• Hypodontia
• Multiple eyelid apocrine hidrocystomas
• Syringofibroadenomas on extremities
• Poromas

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14. Erythrodontia

• Congenital erythropoietic porphyria
• Hepatoerythropoietic porphyria

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15. Paraneoplastic pemphigus

• Target antigens desmoglein 1, desmoglein 3,
  BPAG1, plectin, desmoplakin 1, desmoplakin 2,
  envoplakin, periplakin, unknown antigen (170kDa)
• Notably not BPAG2
• Do not confuse the various BP antigen synonyms
  due to exam stress
• BPAG1 (BP230)
• BPAG2 (BP180, type XVII collagen)

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16. Eruptive xanthomas

• Arise on buttocks and extensor surfaces of
  extremities
• Hypertriglyceridemia
• 1 4 5

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17. Voriconazole

• Azole antifungal
• Prophylaxis against Aspergillus infection
• Accelerates development of SCCs
• Photosensitivity and premature photodamage
• Skin cancer most frequent malignancy in organ
  transplant recipients (95 NMSC SCC gt BCC)
• UVA-induced like most medication-related
  photosensitivity

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18. Trench conditions

• Trench fever
• Bartonella quintana
• Pediculus humanus corporis
• Trench mouth
• Mixed population of bacteria
• Prevotella intermedia, Fusobacterium, Treponema
  and Selenomonas spp.,
• Trench foot

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19. Defects in p genes

• p53
• LiFraumeni syndrome
• Actinic keratoses
• Squamous cell carcinoma
• p57
• BeckwithWiedemann syndrome
• p63
• HayWells syndrome (AEC syndrome)
• EEC syndrome
• RappHodgkin syndrome

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20. Vismodegib

• Mechanism of action
• Antagonizes membrane bound smoothened receptor
  leading to less activity of GLI transcription
  factor and ultimately decreased expression of
  tumor mediating genes
• Adverse effects include
• Muscle spasms
• Alopecia
• Used to treat locally advanced or metastatic
  BCCs
• Inactivating PTCH mutations and activating SMO
  mutations cause most BCC's. Normal pathway SSH
  binds PTCH, together they inhibit SMO leading to
  less GLI transcription factor going to nucleus
  (net result decreased expression of tumor
  mediating genes).

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21. Beckers nevus

• Associated with a smooth muscle hamartoma
• Transient induration/elevation upon rubbing in
  80 (pseudo-Darier's sign)
• May occur on the forehead, cheek, chest,
  shoulder, forearm, wrist, abdomen, buttock, and
  shin

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22. Cold panniculitis

• Cheeks of toddlers and young children
• Results from contact with popsicle or ice bag
• No treatment necessary

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23. Extracellular matrix protein-1

• ECM-1 mutated in lipoid proteinosis
• Targeted by autoantibodies in lichen sclerosus
• Also know as UrbachWiethe disease
• Beaded papules on eyelid margin not the only
  finding. First clinical sign is weak cry due to
  deposition of hyaline-like material in laryngeal
  mucosa. Hoarseness remains throughout life.
  Ice-pick-like acneiform scars occur on face.

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24. Collagen types in cartilage

• II (2)
• IX (9)
• X (10)
• XI (11)
• XII (12)
• XX (20)
• XXVII (27)
• Autoantibodies to collagen type 2, as well as 9
  and 11, have been reported to cause relapsing
  polychondritis. This targeting of cartilage
  results in the red ears (sparing the lobes),
  arthritis, aortic aneurysms, and tracheal
  collapse.

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25. Clofazimine

• Mechanism of action
• Unknown
• Indication
• Multibacillary leprosy (gt5 lesions)
• Adverse effects include
• Orangepink skin and body fluid discoloration
• Ichthyosis
• Fatal enteropathy
• Due to crystal deposition in the small bowel
  mucosa

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26. GNAS1 gene

• Encodes G protein a-subunit that regulates
  adenylate cyclase activity
• Normal function is to negatively regulate bone
  formation
• Mutated in several conditions including
• Progressive osseous heteroplasia
• Plate-like osteoma cutis
• Albright hereditary osteodystrophy
• McCuneAlbright syndrome

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27. WHIM syndrome

• Warts
• Hypogammaglobulinemia
• Infections
• Myelokathexis
• Mutated chemokine receptor CXCR4 gene
• Namely recurrent bacterial infections such as
  sinusitis, cellulitis, periodontitis, and
  meningitis
• Chronic peripheral neutropenia due to retention
  of neutrophils in the bone marrow
• Autosomal dominant

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28. Paraphenylenediamine (PPD)

• Common contact allergen found in
• Black hair dye, scuba gear, henna
• Cross-reacts with
• Para-aminobenzoic acid (PABA), azo dyes,
  sulfonamides/sulfonylureas, thiazides, ester
  anesthetics
• Neutrophilic and eosinophilic dermatitis
• Not found in pure henna
• PPD PASTE

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29. Omalizumab

• Mechanism of action
• Humanized monoclonal IgG1 antibody that binds to
  IgE preventing interaction with receptor (Fc?RI)
  on mast cells and basophils
• Has been used to treat urticaria, atopic
  dermatitis, and bullous pemphigoid
• IgG not only circulating auto-antibody isotype
  in bullous pemphigoid, but also IgE

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30. Oral fibroma

• Most common tumor of the oral cavity
• Located along bite line of the buccal mucosa
• Cured by conservative surgical excision

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What I did to prepare for the certification
(board) exam

• Note taking and memorization
• Clinical images
• Pathology
• Practice questions

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Google GTD
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Google pomodoro technique
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Note taking and memorization (I)

• Leading up to month prior to exam
• Read through and took notes from Bolongias
  entire 2nd edition (ISBN 1416029990)
• Added missing high-yield facts to my notes from
  Dermatology Quick Glance (ISBN 0071415262)
• Spent time each week reviewing, re-reviewing,
  and memorizing above notes

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Note taking and memorization (II)

• During one month prior to exam
• Skimmed Genodermatoses (i.e. "Spitz" ISBN
  0781740886) and added to my notes when needed
• Reviewed notes from 2012 Florida Dermatology
  Dermatopathology Board Review Course
• Re-reviewed my own notes

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Clinical images

• During one month prior to exam reviewed all
  images in
• Andrews 10th and 11th editions
• Bolongias 2nd edition
• Color Atlas of Dermatology 1st and 2nd editions
  (i.e. "Callen" ISBN 0721637566 and 0721682561)

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Pathology

• During one month prior to exam
• Watched all the PowerPoint lectures by Dr.
  Elston found on the Dermatopathology Requisites
  in Dermatology website
• Reviewed all the online DLCS study slide sets
• Note I also attended two review courses, the
  Barron Board Review and the Florida Dermatology
  Dermatopathology Board Review Course, both of
  which had slide reviews
• http//www.requisitesindermatology.com/dermatopat
  hology_ppt.php
• http//dermpathlab.com/residents/slide-study-set-
  program

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Practice questions

• During one month prior
• Reviewed all ETAS practice questions
• Reviewed all quizzes in McGraw-Hill Specialty
  Board Review Dermatology A Pictorial Review
  (ISBN 0071597271)
• Reviewed all questions in the ASDS Primer in
  Dermatologic Surgery A Study Companion
• http//dermatologyinreview.com/Galderma
• http//www.asds.net/primerbook.aspx

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What I did NOT do to prepare

• Galderma Preboard Seminar review course
• Journal review

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• Questions?

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References

• Books (ISBN)
• Andrews' Diseases of the Skin Clinical
  Dermatology (1437703143)
• Dermatology (0723435715)
• Hurwitz Clinical Pediatric Dermatology A
  Textbook of Skin Disorders of Childhood and
  Adolescence (1437704123)
• Papers (PMID)
• A review of radiotherapy for merkel cell
  carcinoma of the head and neck (23213534)
• Clofazimine a review of its medical uses and
  mechanisms of action (7829710)
• Cutaneous toxic effects associated with
  vemurafenib and inhibition of the BRAF pathway
  (22431713)
• Disseminate and recurrent infundibulofolliculitis
  response to isotretinoin (15303788)
• Eyelid dermatitis contact allergy to
  3-(dimethylamino)propylamine (9134437)
• Human papillomavirus typing of verrucae in a
  patient with WHIM syndrome (20713842)
• Neutrophilic and eosinophilic dermatitis caused
  by contact allergic reaction to
  paraphenylenediamine in hair dye (23165836)
• Observations on angiopoietin 2 in patients with
  angiosarcoma (15149523)
• Routine omission of sentinel lymph node biopsy
  for merkel cell carcinoma lt 1 cm is not
  justified (19933899)
• Schöpf-Schulz-Passarge syndrome further
  delineation of the phenotype and genetic
  considerations (19002348)
• Skin cancer in solid organ transplant recipients
  advances in therapy and management (21763561)
• Successful treatment of bullous pemphigoid with
  omalizumab (23165827)
• The genesis of Zelboraf Targeting mutant B-Raf
  in melanoma (23027900)