Presentation on theme: "Michael W. Wangia, M.D. Clinical Assistant Professor"— Presentation transcript:
1 Dermatology Pearls for 2014 American Board of Dermatology certifying exam Michael W. Wangia, M.D.Clinical Assistant ProfessorDermatopathology FellowUniversity of Florida Department of Dermatology
2 Objectives30 high-yield pearls What I did to prepare
3 “For every pearl at the bottom of the ocean, there is a ton of whale shit.” —Ernest Lee, M.D.
4 1. Ipilimumab Mechanism of action Blocks CTLA-4 receptor†‡ on cytotoxic T lymphocytes, preventing their inhibition, allowing them to destroy melanoma cellsAdverse effects includeFacial swellingColitis†Also known as CD152‡Normally CTLA-4 stimulation inhibits T-cells
5 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 600The most severe dermatologic adverse effects are the development of eruptive squamous cell carcinomas and keratoacanthomas.
6 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
7 4. Angiopoietin receptors Tie-1 and Tie-2 are cell-surface receptors†Angiopoietins bind these receptors and promote angiogenesisThere are four angiopoietinsSerum Ang2 levels are elevated in patients with angiosarcoma†Mutations that lead to dysfunction of the Tie-2 receptor are associated with vascular malformations
8 5. Viral-associated trichodysplasia Closely linked with organ transplantation, immunosuppression†, and pre-B cell leukemiaFollicular spiny papules erupt on faceLikely due to a polyomavirus‡Treat with valganciclovir†Cyclosporin often cited‡A dsDNA virus and member of papovavirus family
9 6. Merkel cell carcinomaMerkel cell polyomavirus (MCPyV) Wide local excision Sentinel lymph node biopsy in all cases Adjuvant radiotherapy for lesions on head and neck with locoregional spread
10 7. Coding excision sizeMalignant 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)
11 Modifiers-25-59-79Review these in the AAD website
12 8. Disseminated infundibulofolliculitis Young dark-skinned patients Uniform 1—2mm papules on neck and upper trunk Figure in Andrews’ 10th edition on page 776 Treat with topical steroids, PUVA or isotretinoin
13 9. SyringomasOccur not just on face but also neck, chest, axillae, upper arms, and periumbilically Figure B in Bolongia’s 2nd edition on page 1704 Figure in Andrews’ 10th edition on page 664 Associated with Down syndrome, Nicolau–Balus syndrome, and Brooke-Spiegler syndrome
14 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
15 11. Defects in keratin 5†‡ Epidermolysis bullosa simplex§ Dowling–Degos' diseaseOlmsted syndrome†For list of cutaneous conditions caused by mutations in keratins see:‡For more lists (some good, some not so good) of board-related fodder see:§May be due to mutation in gene encoding keratin 5 and/or 14
19 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)
20 16. Eruptive xanthomasArise on buttocks and extensor surfaces of extremities Hypertriglyceridemia
21 17. Voriconazole Azole antifungal Prophylaxis against Aspergillus infectionAccelerates development of SCC’s†Photosensitivity‡ and premature photodamage†Skin cancer most frequent malignancy in organ transplant recipients (95% NMSC | SCC > BCC)‡UVA-induced like most medication-related photosensitivity
22 18. “Trench” conditionsTrench fever Bartonella quintana Pediculus humanus corporis Trench mouth Mixed population of bacteria Prevotella intermedia, Fusobacterium, Treponema and Selenomonas spp., Trench foot
24 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 includeMuscle spasmsAlopecia†Used to treat locally advanced or metastatic BCC’s‡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).
25 21. Becker’s nevusAssociated 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 shinBe ready to see a Becker’s nevus that is not on the shoulder
26 22. Cold panniculitisCheeks of toddlers and young children Results from contact with popsicle or ice bag No treatment necessary
27 23. Extracellular matrix protein-1 ECM-1 mutated in lipoid proteinosis†‡Targeted by autoantibodies in lichen sclerosus†Also know as Urbach–Wiethe 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.
28 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.
29 25. Clofazimine Mechanism of action Unknown Indication Multibacillary leprosy (>5 lesions)Adverse effects includeOrange–pink skin and body fluid discolorationIchthyosisFatal enteropathy††Due to crystal deposition in the small bowel mucosaThe skin discoloration and ichthyosis are reversible.
30 26. GNAS1 geneEncodes G protein α-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 McCune–Albright syndrome
31 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
32 28. Paraphenylenediamine (PPD) Common contact allergen found inBlack hair dye, scuba gear, henna†Cross-reacts withPara-aminobenzoic acid (PABA), azo dyes, sulfonamides/sulfonylureas, thiazides, ester anesthetics‡Neutrophilic and eosinophilic dermatitis†Not found in pure henna‡PPD + PASTE
33 29. Omalizumab Mechanism of action Humanized monoclonal IgG1 antibody that binds to IgE preventing interaction with receptor (FcϵRI) on mast cells and basophilsHas been used to treat urticaria, atopic dermatitis, and bullous pemphigoid††IgG not only circulating auto-antibody isotype in bullous pemphigoid, but also IgEXolair is an anti-IgE approved for mod-severe allergic asthma not responding to steroids. Its finding its way off-label, in management of several IgE mediated disease including chronic urticarial, atopic derm, mastocytosis and even BP.
34 30. Oral fibromaMost common “tumor” of the oral cavity Located along bite line of the buccal mucosa Cured by conservative surgical excision
35 Miscellaneous Randoms Imatinib/Gleevec Treatment of DFSP with + PDGFR-COL1A1 mutationTreatment of hypereosinophilic syndrome with mutation in FIP1L1-PDGFR alpha mutation.
36 IPMutation? NEMO. Affects NF-KB. What is the purpose of NF-KB? - protects against TNF-induced apoptosis
37 Vascular lesions?Wiebel palade bodies. These are essentially storage granules of endothelial cells. Contain vWF and P-selectin. Play dual role in both hemostasis and inflammationBier spots are small erythematous blanching macules. Essentially benign physiologic vascular anomaly of no significance clinically. Know both malignant and benign vascular tumors and lesions.
38 FibromatosisJuvenile hyaline fibromatosis – (nodular lesions on hands, scalp and face with gingival hypertrophy and associated joint contractures). Bx shows nodular hyaline fibrosis). Genetic mutation associated? CMG2 (capillary morphogenesis protein-2). Or ANTXR2 mutations (anthrax toxin receptor 2).
40 What I did to prepareNote taking and memorization Clinical images Pathology Practice questions
41 Note taking and memorization (I) Leading up to month prior to exam Read through and took notes from Bolognia’s – put more emphasis on photos and tables entire 2nd edition (ISBN ) Read entire Andrews 2 months prior to the boards Spent 2 weeks prior to the test day My goal was to review, re-review, and memorizing above notes every two weeks
42 Note taking and memorization (II) During one month prior to exam Skimmed Genodermatoses (i.e. "Spitz" | ISBN ) and added to my notes when needed Reviewed notes from 2013 Florida Dermatology & Dermatopathology Board Review Course Re-reviewed my own notes
43 Clinical imagesDuring one month prior to exam reviewed all images in: Andrews’ 11th editions Bolognia’s 2nd edition Color Atlas of Dermatology 1st and 2nd editions (i.e. "Callen" | ISBN and ) Atlas of Clinical dermatology 4th ed Du Vivier
44 Dermatopathology During one month prior to exam† Reviewed Elston front to backWatched 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/dermatopathology_ppt.php§http://dermpathlab.com/residents/slide-study-set-program
45 Practice questions During one month prior Reviewed all ETAS practice questions†Reviewed all questions in the ASDS Primer in Dermatologic Surgery: A Study Companion‡†http://dermatologyinreview.com/Galderma‡http://www.asds.net/primerbook.aspx
46 What I did NOT do to prepare Galderma Preboard Seminar review course Quizes in McGraw-Hill Specialty Board Review Dermatology: A Pictorial Review (ISBN ) Journal review
47 **** REVIEW SCLEROTHERAPY (slerosing agents, side effects etc); HAIR TRANSPLANTATION; AND FOCUS ON TECHNIQUE AND MAJOR COMPLICATIONS.- Recommend using Bolognia and Surgery Primer
49 ReferencesBooks (ISBN): Andrews' Diseases of the Skin: Clinical Dermatology ( ) Dermatology ( ) Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence ( ) Papers (PMID): A review of radiotherapy for merkel cell carcinoma of the head and neck ( ) Clofazimine: a review of its medical uses and mechanisms of action ( ) Cutaneous toxic effects associated with vemurafenib and inhibition of the BRAF pathway ( ) Disseminate and recurrent infundibulofolliculitis: response to isotretinoin ( ) Eyelid dermatitis: contact allergy to 3-(dimethylamino)propylamine ( ) Human papillomavirus typing of verrucae in a patient with WHIM syndrome ( ) Neutrophilic and eosinophilic dermatitis caused by contact allergic reaction to paraphenylenediamine in hair dye ( ) Observations on angiopoietin 2 in patients with angiosarcoma ( ) Routine omission of sentinel lymph node biopsy for merkel cell carcinoma <= 1 cm is not justified ( ) Schöpf-Schulz-Passarge syndrome: further delineation of the phenotype and genetic considerations ( ) Skin cancer in solid organ transplant recipients: advances in therapy and management ( ) Successful treatment of bullous pemphigoid with omalizumab ( ) The genesis of Zelboraf: Targeting mutant B-Raf in melanoma ( ) Trichodysplasia of immunosuppression treated with oral valganciclovir ( ) Viral-associated trichodysplasia in patients who are immunocompromised ( ) Voriconazole-associated phototoxicity ( )