Dermatology Pearls - Department of Dermatology

Report
Dermatology Pearls for 2014
American Board of Dermatology certifying exam
Michael W. Wangia, M.D.
Clinical Assistant Professor
Dermatopathology Fellow
University of Florida Department of Dermatology
Objectives
30 high-yield pearls
What I did to prepare
“For every pearl at the bottom of the ocean,
there is a ton of whale shit.”
—Ernest Lee, M.D.
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
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
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
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
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
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
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)
Modifiers
• -25
• -59
• -79
• Review these in the AAD website
8. Disseminated infundibulofolliculitis
Young dark-skinned patients
Uniform 1—2mm papules on neck and upper
trunk
Figure 33-31 in Andrews’ 10th edition on page
776
Treat with topical steroids, PUVA or isotretinoin
9. Syringomas
Occur not just on face but also neck, chest,
axillae, upper arms, and periumbilically
Figure 111.22B in Bolongia’s 2nd edition on page
1704
Figure 29-36 in Andrews’ 10th edition on page
664
Associated with Down syndrome, Nicolau–Balus
syndrome, and Brooke-Spiegler syndrome
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/dermat
ology/1048885-1070740-268.jpg
11. Defects in keratin 5†‡
Epidermolysis bullosa simplex§
Dowling–Degos' 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_mu
tations_in_keratins
‡For more lists (some good, some not so good) of board-related fodder see:
http://en.wikipedia.org/wiki/List_of_cutaneous_conditions#See_also
§May be due to mutation in gene encoding keratin 5 and/or 14
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
13. Schöpf–Schulz–Passarge syndrome
Ectodermal dysplasia
Diffuse symmetric palmoplantar keratoderma
Hypodontia
Multiple eyelid apocrine hidrocystomas
Syringofibroadenomas on extremities
Poromas
14. Erythrodontia
Congenital erythropoietic porphyria
Hepatoerythropoietic porphyria
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)
16. Eruptive xanthomas
Arise on buttocks and extensor surfaces of
extremities
Hypertriglyceridemia
17. Voriconazole
Azole antifungal
Prophylaxis against Aspergillus infection
Accelerates 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
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
19. Defects in p genes
p53
Li–Fraumeni syndrome
Actinic keratoses
Squamous cell carcinoma
p57
Beckwith–Wiedemann syndrome
p63
Hay–Wells syndrome (AEC syndrome)
EEC syndrome
Rapp–Hodgkin syndrome
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 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).
21. Becker’s 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
22. Cold panniculitis
Cheeks of toddlers and young children
Results from contact with popsicle or ice bag
No treatment necessary
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.
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.
25. Clofazimine
Mechanism of action
Unknown
Indication
Multibacillary leprosy (>5 lesions)
Adverse effects include
Orange–pink skin and body fluid discoloration
Ichthyosis
Fatal enteropathy†
†Due
to crystal deposition in the small bowel mucosa
26. GNAS1 gene
Encodes 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
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
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
29. Omalizumab
Mechanism of action
Humanized monoclonal IgG1 antibody that
binds to IgE preventing interaction with
receptor (FcϵRI) 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
30. Oral fibroma
Most common “tumor” of the oral cavity
Located along bite line of the buccal mucosa
Cured by conservative surgical excision
Miscellaneous Randoms
Imatinib/Gleevec
• Treatment of DFSP with + PDGFR-COL1A1
mutation
• Treatment of hypereosinophilic syndrome
with mutation in FIP1L1-PDGFR alpha
mutation.
IP
• Mutation? NEMO. Affects NF-KB. What is the
purpose of NF-KB? - protects against TNFinduced apoptosis
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 inflammation
• Bier spots are small erythematous blanching
macules. Essentially benign physiologic
vascular anomaly of no significance clinically.
Know both malignant and benign vascular
tumors and lesions.
Fibromatosis
• Juvenile 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).
What I did to prepare
Note taking and memorization
Clinical images
Pathology
Practice questions
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 1416029990)
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
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 2013 Florida
Dermatology & Dermatopathology Board
Review Course
Re-reviewed my own notes
Clinical images
During 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 0721637566 and
0721682561)
Atlas of Clinical dermatology 4th ed Du Vivier
Dermatopathology
During one month prior to exam†
Reviewed Elston front to back
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/dermatopathology_ppt.php
§http://dermpathlab.com/residents/slide-study-set-program
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
What I did NOT do to prepare
Galderma Preboard Seminar review course
Quizes in McGraw-Hill Specialty Board Review
Dermatology: A Pictorial Review (ISBN
0071597271)
Journal review
• **** REVIEW SCLEROTHERAPY (slerosing
agents, side effects etc); HAIR
TRANSPLANTATION; AND FOCUS ON
TECHNIQUE AND MAJOR COMPLICATIONS.
• - Recommend using Bolognia and Surgery
Primer
Questions?
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 <= 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)
Trichodysplasia of immunosuppression treated with oral valganciclovir (19103376)
Viral-associated trichodysplasia in patients who are immunocompromised (14726896)
Voriconazole-associated phototoxicity (21382298)
• Thank Dr. Brendan Thomas for his input
[email protected]

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