Bolognia Reviews Flashcards

Syphilis
Terms Definitions
MCC of erythroderma in adults
psoriasis
Usually due to withdrawal of CS, cyclosporine, efalizumab, MTX
MCC of erythroderma in kids
Drug reaction #1
Psoriasis #2
MC bullous dermatosis to progress to erythroderma?
pemphigus foliaceous
Characteristic additional findings in erythroderma associated with CTCL
Painful and fissured keratoderma
Leonine facies
Diffuse alopecia
Causes of neonatal erythroderma
Non-bullous CIE
Bullous CIE
Netherton
What genes are mutated in monilethrix?
K81, K83, K86
Where does acute GVHD first appear?
Acrally
What two LP variants are associated with SCC?
Hypertrophic
Ulcerative
What is the most common location for ulcerative LP?
Palmoplantar, especially soles
What is the most common location for perforating GA?
Dorsal hand
Name the vitiligo susceptibility genes.
NALP1 (SLEV1): component of cytoplasmic inflammasomes which regulate caspase activation
AIS1-3: AIS3 from non-autoimmunity associated families
Most common location for segmental vitiligo?
Face
MC significant ocular abnormality associated with vitiligo?
uveitis
Severe form seen in Vogt-Koyonagi-Harada (uveitis, aseptic meningitis, dysacousia, poliosis,vitiligo)
What is Alezzandrini syndrome?
Unilateral whitening of scalp hair, brows, lashes
Unilateral depigmentation of skin of forehead, nose, cheek, upper lip, chin
Same side as unilateral visual changes (atrophic iris, decreased visual acuity)
Differences in childhood vitiligo vs adult
Segmental vitiligo more frequent in kids (although MC type is still vitiligo vulgaris)
Lower incidence of associated endocrinopathies
Increased incidence of autoab production
MC type of albinism in Japan?
OCA4
Besides MITF, what other gene defect is associated with Wardenburg's syndrome type 2?
SLUG: encodes a zinc finger transcription factor; involved in dev't of neural-crest derived cells
Three gene defects in wardenburg's syndrome type 4?
EDN3, EDNRB, SOX10
What is Tietz syndrome?
AD, MITF gene
Allelic to WS2
Generalized hypomelanosis of skin
Deaf-mutism
Hypoplasia of eyebrows
Light blond hair, blue eyes
Normal fundi, no photophobia or nystagmus
Systemic findings in Hermansky-Pudlak
Bleeding tendency (absence of dense bodies in plts; prolonged bleeding time)
Interstitial pulmonary fibrosis
Granulomatous colitis
Less often, RF, CM
Which Hermansky-Pudlak subtype is associated with immunodeficiency?
HPS2
AP3B1 mutation (involved in protein sorting to lysosomes and organelles)
CD1b binds AP3 complex; may account for recurrent bacterial infections
Histologic hallmark of Chediak-Higashi
giant melanosomes in melanocytes
Triad of dyskeratosis congenita
Lacy reticulated hyperpigmentation neck, upper chest
Nail dystrophy (pterygium)
Premalignant leukoplakia (lateral tongue)
Gene defect in dyschromatosis symmetrica hereditaria
DSRAD
double stranded RNA-specific adenosine deaminase
Gene defects in SCID
XLR (40%): gamma chain of IL2 receptor
AR: 20% adenosine deaminasae, 6% JAK3
AutoAb in SLE that correlate positively with activity, associated with lupus nephritis
dsDNA
AutoAb associated with neuropsychiatric SLE?
rRNP
What is typical clinical presentation of hypergammaglobulinemic purpura of Waldenstrom?
Recurring crops of burnin or stinging macula hemorrhage on LE
What is MC association in pts with hypergammaglobulinemic purpura of waldenstrom?
autoimmune CTD, especially Sjogren's
Associations with recurrent superficial thrombophlebitis
trousseau's (underlying malignancy, esp. pancreatic or lung)
Hypercoagulable state
Inflammatory ds (Behcets)
Typical skin finding with vasocclusive disease?
retiform purpura
Ddx of microvascular occlusiion
Platelet plugging
Cold-related gelling or agglutination
Vessel-invasive organism
Embolization
Systemic coagulopathies
Vascular coagulopathies
Miscellaneous (spider bite)
Cardinal physical exam findings in cryoglobulinemia?
Retiform purpuric or necrotic lesions at acral sites of cold exposure
Treatment for disseminated stronglyoides?
thiabendazole
What is most likely diagnosis in pt with h/o renal stones with sudden-onset livedo reticularis or retiform purpura?
Hyperoxaluria--oxalate embolus
Etiology of postinfectious purpura fulminans?
Acquired severe protein S deficiency
Seen in kids 2 weeks after recovering from GAS or VZV
Infection-triggered Ab interfere with protein S function
Antiphospholipid antibodies sensitivity and specificity
Anticardiolipin Ab (screening)
Detects Abs with affinity for negatively charged membrane phospholipids
Better sensitivity

Lupus anticoagulant (confirmatory)
Ab interferes with coagulation during measurement of PTT
measured with Russell viper venom test
Better specificity
What are precipitating factors associated with catastrophic antiphospholipid Ab syndrome?
Infections
Meds (sulfa containing diuretics,captopril,OCPs)
D/c anticoagulant
Surgical procedures
Clinical features of Sneddon's syndrome?
Livedo reticularis or racemosa
Labile HTN
CNS symptoms due to cerebal vascular disease (CVA, TIA)
recurrent fetal loss
raynaud's
Medication?
Ara-c erythrodysesthesia
Medication?
Toxic erythema of chemo
Ara-C ears
Petechiae due to thrombocytopenia
Medication?
Raynaud’s phenomenon with digital necrosis due to systemic bleomycin
What defect in patients with HIV is thought to increase their risk of cutaneous reactions to TMP-SMX?
acquired glutathione deficiency
Etiology?
Reaction to vitamin K injection
What toll like receptor is activated by beta-defensin?
TLR-4
alpha-defensins increase expression of which cytokines?
TNF alpha, IL1
What mediates adhesion of langerhans cells to keratinocytes?
E-cadherin
Endogenous antigens complex with MHC1 or 2?
Exogenous antigens?
Endogenous: MHC1
Exogenous: MHC2
What region of the TCR do streptococcal antigens bind-to to cause Streptococcal toxic shock syndrome?
beta variable
Th1 cytokines
IL12, TNFalpha, interferon gamma
Th1 diseases
psoriasis
ACD
tuberculoid leprosy
cutaneous (self-limited) leishmaniasis
Th2 cytokines
IL4,5,6,10
Th2 diseases
atopic dermatitis
CTCL
lepromatous leprosy
disseminated cutaneous (chronic) leishmaniasis
CD markers on Treg cells?
CD4+
CD25+
Foxp3+ (TF)
What genodermatosis is associated with foxp3 gene defect?
IPEX
Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked syndrome
Which immunoglobulin fixes complement most efficiently?
IgM
Which IgG subcalsses activate complement the best?
IgG1 and IgG3
Less effective IgG2
IgG4 does not fix complement
What is the name of the test used for assessing the presence of lupus anticoagulant activity?
Russell viper venom test
Triad in Sneddon syndrome?
livedo reticularis
labile HTN
CNS disease

May have fetal loss
Tx: coumadin
What type of T cells are involved in allergic contact dermatitis?
CD8+
What are the main markers currently used to detect B cells?
CD19,20,22
Subtype of EB with:
PPK with advancing age
Oro-esophageal involvement
Natal teeth
Clumped tonofilaments on EM
EBS-Dowling Meara
Most severe of EBS
Type of EB associated with:
Sloughing of nails
Perioral involvment with lip sparing
Junctional EB
Gene defect in hypohidrotic ectodermal dysplasia with skin fragility
plakophilin 1
Generalized erythroderma with blistering at birth
short, sparse hair
Thick, dystrophic nails
Function of gene defective in Kindler's syndrome
Kind-1
Mb-associated signaling protein linking actin cytoskeleton to ECM
EB associated with greatest risk of SCC development?
AR DEB-Hallopeau Siemens type
EB that can cause granulation tissue around the trachea leading to fatal asphyxia?
JEB-Herlitz type
Miliaria cystallina is caused by obstruction of the sweat glands at what level?
stratum corneum
Miliaria rubra: obstruction of sweat glands at what level?
malpighian layer
Diagnosis?
Eosinophilic pustular folliculitis in infancy
Sterile pustules scalp and face, spares trunk/extremities
MC eye abnormality in IP?
Strabismus
Gene defect and function in hyperIgE?
STAT3 gene
IL6 downstream mediator
MC location of pyoderma gangrenosum in infants?
Perineal area
Rigid, taut, shiny translucent skin with linear splits in the flexural folds
Diagnosis and MCC death?
Restrictive dermopathy
Pulmonary disease
Gene defects in restrictive dermopathy?
LMN-A
ZMPSTE-24 (zinc metalloproteinase involved in post-translational modification of lamin A)
3 genera under spirochaetales
Leptospira
Borrelia
Treponema
Signs of early congential syphilis
Marasmic syphilis
Typical secondary skin lesions (more bullous and erosive--pemphigus syphiliticus)
Snuffles (bloody or purulent nasal discharge)
Perioral and perianal fissures
Lymphadenitis
Skeletal involvement (pseudoparalysis)
Reduced movement secondary to pain
Pneumonitis, hepatitis, nephropathy, neurosyphilis
Signs of Late congenital syphilis
Hutchinson’s Triad
Interstitial keratitis
Hutchinson’s teeth
Neural deafness
Risk of transmission of congenital syphilis
100% when mom infected b/t conception & 7th month
<50% risk if mom infected more than 2 yrs prior to pregnancy
Reduced risk of transmission after 7th month
Treatment for primary, seconday, or early latent syphilis
Benzathine penicillin 2.4 MU IM
Alternate: doxy, TCN, E-mycin for 14 days
Treatment for late latent syphilis
Benzathine penicillin 2.4 MU IM weekly x3
Alternate: doxy, e-mycin, TCN for 28 days.
Diagnosis?
Collarette of Biett
Secondary syphilis
Growth requirement for culture of Neisseria gonorrhea
iron
Culture media for Neisseria gonorrhea?
Thayer-Martin
Cause of acute painful genital ulcers w/ inguinal adenitis & buboes w/o systemic sxs
Chancroid
Hemophilus ducreyi
Incubation period for chancroid?
3-10 days
Name of bodies in histiocytes in LGV?
Gamma-Favre bodies (Geimsa stain)
TOC for LGV?
Doxy x 3 weeks
Alternative: macrolides
Incubation period for granuloma inguinale?
long: 2-12 weeks
Cause of non-tender genital sore without lymphadenopathy?
Granuloma inguinale (donovanosis)
Calymmatobacterium granulomatis
Extragenital lesions in donovanosis?
Due to autoinoculation or dissemination
Most frequently to bone
TOC for donovanosis?
Bactrim 1 tab bid x 3 wks
or
Doxycyline 100mg bid x 3 wks
HLA association with gestational pemphigoid?
Other associations?
HLA-DR3, HLA-DR4

Graves disease
Choriocarcinoma
Hydatidiform moles
What are fetal risks in herpes gestationis?
Increased risk of prematurity and SGA (small-for-gestatational age)
10% have skin lesions, usually mild
No increase in fetal morbidity or mortality
MC pregnancy dermatosis?
Risk factors for development?
PUPPP
increased maternal weight gain
multiple gestation
higher prevalence w/ male fetus, C-section delivery
Clinical presentation of cholestasis of pregnancy?
Typically during 3rd trimester
Onset coincides with UTI in 50%
intense, generalized pruritus
typically recurs with OCP
Risk to fetus:Undefined tendency toward meconium/premature labor, fetal stress, and fetal death which may be reduced by treatment
What is maternal risk of cholestasis of pregnancy?
Vitamin K malabsorption/deficiency leading to bleeding anomalies
Tends to develop cholelithiasis, gb disease
Treatment of autoimmune progesterone dermatitis?
Tamoxifen or danazol
Inhibit ovulation by estrogen-containing preparations
Recurrent cyclic flares of dermatitis that correspond to luteal phase of the menstrual cycle
Pregnancy dermatosis with increased risk of stillbirth and placental insufficiency?
Impetigo herpetiformis
Pustular psoriasis of pregnancy
Associated w/ hypocalcemia
Medication that can provoke Darier's?
Lithium
Name the 2 non-syndromic diffuse hereditary PPKs
Vorner (epidermolytic, K9 (KI) defect)
Unna-Thost (non-epidermolytic, K1)
Mal de Meleda

Transgradient, malodorous PPK
Elbows/knees involved
Hyperhidrosis
Periorificial lesions
Thickened nails (koilonychia, subungual hyperkeratosis)
Gene defect and eponym for transgradiens and progrediens PPK?
Greither's syndrome, AD, K1
Diffuse transgradient PPK with onset in infancy
Hyperhidrosis
Hyperkeratotic plaques on elbows and knees

Histo: epidermolytic changes of granular layer (distinguishes from orthokeratosis and nl granular layer in mal de meleda)
Vohwinkel's (Keratoderma hereditaria mutilans)
Pseudoainhum
Starfish keratoses
Honeycombed palms
Variable hearing loss, ichthyosis, alopecia
Clinical features of Bart-Pumphrey syndrome?
Knuckle pads
Leukonychia
Sensorineural deafness
Variable and mild PPK
Connexin 26 (GJB2)
Syndrome with mutilating PPK, ichythosis hystrix, normal hearing?
Curth-Macklin
K1 defect
Eponym for PPK with scleroatrophy?
Huriez syndrome
Red, atrophic skin dorsal hands/feet
mild PPK
sclerodactyly
Nail changes
Almost complete absence of Langerhans cells
At risk for SCC in atrophic skin
Besides transgradient PPK and destructive periodontitis, other features in Papillon-Lefevre?
Psoriasiform lesions on extremities
Loss of teeth
Pyogenic infections
Syndrome allelic to Papillon-Lefevre?
Haim-Munk
Both due to cathepsin C defect
PPK
Periodontitis
Arachnodactyly
Atrophic nail changes
Acro-osteolysis
Eponym for mutilating PPK with periorificial plaques?
Olmsted syndrome
Keratoderma leads to flexion contractures
Mutilation w/ digit constriction +/- autoamputation
Olmsted syndrome
Clinical features of Naxos?
Diffuse NEPPK (erythematous border, non-transgradient): 1st yr of life
Woolly hair: birth
R sided cardiomyopathy: puberty
Plakoglobin gene, AR
Gene defect in focal/striate nonsyndromic PPK
Desmoglein 1 (m/c), desmocollin, desmoplakin
AD
Clinical features of Carvajal syndrome?
Striake EPPK (early infancy)
Woolly hair (birth)
Left sided cardiomyopathy (adolescence)
desmoplakin gene defect, AR
In Howell-Evans, is late or early onset PPK associated with increased risk of esophageal cancer?
late onset PPK (age 5-10)
Esophageal cancer in 5th decade
Other features of H-E: KP, dry skin, oral leukokeratosis
Inheritance and gene defect in Howell-Evans?
AD, TOC gene
Inheritance and gene defect in Richner-Hanhart?
AR, hepatic tyrosine aminotransferase
PPK associated with degeneration of elastic fibers?
acrokeratoelastoidosis of costa
Pathogenesis of acute ICD?
Damaged keratinocytes
Increased TNFa, IL6, IL1b
Upregulation of ICAM1 leading to T-cell inflammation
What electrolyte abnormality can be found with exposure to hydrofluoric acid?
hypoMg
hypoCa
What metal exposure can cause nasal septum perforation?
chromic acid
Which metal salt causes green hair discoloration?
copper
Which metal salt causes blue linear pigmentation of the tongue and gums?
inorganic mercury
(marker for systemic mercury poisoning)
Metal salt causing ulcerating granulomas and possibly hypersensitivity reactions
beryllium compounds
Hematologic effect of benzene exposure?
aplastic anemia
What is the irritant in the plant Family Alliaceae?
thiocyanates
garlic, radish
What is the irritant in the plant Family Amaryllidaceae?
calcium oxalate
daffodil, narcissus, hyacinth
What is the irritant in the plant Family Euphorbiaceae?
Phorbol esters
In croton plant
Gene defect in x-linked hypogammaglobulinemia?
BTK gene
tyrosine kinase that regulates conversion of pre-B cells to B cells
Gene defect in x-linked hypogammaglobulinemia?
BTK gene
tyrosine kinase that regulates conversion of pre-B cells to B cells
What viral infections are pts with X-linked hypogammaglobulinemia at increased susceptibility for?
hepatitis B
Enteroviral infections
Gene defect in X-linked lymphoproliferative disease?
SH2DIA
Encodes signaling lymphocytic activation molecule associated protein critical for cytotoxic T cell function
What viral infectino are pts with X-linked lymphoproliferative disease susceptible to?
Abnormal response to EBV infection
Stimulates rapidly progressive B cell lymphoma--death in 70% of affected boys
MC ocular manifestation in kids with Behcet's?
bilateral panuveitis
associated with worse prognosis
Prodrome in SJS/TEN?
fever
stinging eyes
painful swallowing
SCORTEN in SJS/TEN
Age > 40
Malignancy
Tachycardia > 120
Initial surface of epithelial detachment > 10%
Serum urea > 10
Serum glucose >14
Bicarb < 20
Skin finding in chronic rheumatic fever?
Subcutaneous nodules over bony prominences
Annular erythema of infancy
Migration rate of erythema gyratum repens?
1cm/day
Associations with erythema gyratum repens?
85% w/ neoplasm (LUNG> breast, esophagus)
pulmonary TB
icthyosis
PPK
hypereosinophilia
Meds causing photoallergic reactions
MC: thiazides
sulfonamides
sulfonylureas
phenothiazines
Iododerma
Erythematous, edematous vesiculopustules on the buttocks
Neutrophilic eccrine hidradenitis
Predisposition to develop DRESS from anticonvulsants due to?
Defect in epoxide hydroxylase or the inability to detoxify arene oxide metabolites

Cross reactivity between phenytoin, carbamazepine, and phenobarbital
Predisposition to develop DRESS from sulfonamides due to?
Slow acetylators
Also susceptibility of lymphoid cells to toxic effects of the metabolite, hydroxylamine
Meds causing DRESS
Anticonvulsants
Sulfonamides
Dapsone
Minocin
Lamotrigine
Allopurinol
Abacavir
Treatment with CS for DRESS when which organ systems involved?
Cardiac and lung
(Ineffective for liver or kidney involvement)
MCC of pseudolymphoma?
Anticonvulsants
Skin findings in serum sickness like reaction?
urticarial, EM-like with lilac center
Distinguishing features b/t serum sickness like reaction and serum sickness
SS-like: no hypocomplementemia, no immune complexes, no vasculitis, no renal involvement
Type II (cytotoxic) vs type III (immune complex) in SS
Meds associated with telogen effluvium?
Anticoagulants: heparin> warfarin
β-blockers
Lithium
Retinoids
Chemotherapeutic drug causing onycholysis?
paclitaxel
Mechanism of anagen effluvium from chemo agents?
Abrupt cessation of mitotic activity in the rapidly dividing cells of the hair matrix
Treatment for chemo-induced stomatitis?
Palifermin (keratinocyte growth factor)
Binds keratinocyte GF receptor, stimulates cells to grow and divide
Medication?
5-FU
Drug associated with necrosis of psoriasis when overdosed?
MTX
Meds involved in drug-induced psoriasis?
Terbinafine, NSAIDs (<4wks latency)
Antimalarials, ACE inhibitors (4-12 wks)
β-blockers and Lithium (>12 wks)
Fungus associated with favic chandelier?
Trichophyton schoenlenii
Pectinate bodies
Microsporum audouinii
Spiral hyphae
Trichophyton mentagrophytes
Etiology of neonatal cephalic pustulosis
Malassezia sympodialis
Risk factors and etiology for Pityrosporum folliculitis
Chronic abx use, immunosuppression, local occlusion
Excessive growth of M. furfur & M. globosa
MCC white piedra in pubic hair?
Trichosporon inkin--increased since HIV epidemic
MCC trichosporonosis?
T. asahii
serious systemic infection (fungemia, fever, skin lesions, pulmonary & renal disease)
Typical locations for white and black piedra
White: face, axillae
Black: scalp, face
Common causes of fluorescing ectothrix infection?
M. canis
M. audouinii
M. distortum
M. ferruginosum
M. gypseum (Sometimes)
Common causes of endothrix infection?
T. yaoundei
T. gourvilli
T. rubrum
T. tonsurans
T. soudanese
T. violaceum
Most sensitive stain for dermatophytes?
calcofluour white
fluorescent stain specific for glucan chains in chitin and cellulose fungal cell walls
What does chlorazol black stain?
E-chitin
Components of mycosel?
Sabaroud's dextrose agar with chloramphenicol (inhibits bacterial growth) and cycloheximide
What does cycloheximide inhibit?
Rapidly growing nonpathogenic moulds
Some pathogens: Scytalidium species, Cryptococcus neoformis, Some Candida species, Prototheca,
Yeast forms of Histoplasma and Blastomyces
Interpretation of DTM?
Dermatophytes utilize protein as a carbon source producing alkaline by-products causing the media to turn from amber to red
Nondermatophytes media to turn yellow due to acid by-products.
MC webspace involved in erosio interdigitalis blastomycetica
3rd web space between MF and RF
One of cutaneous manifestations of DM
MC endocrinopathy seen in APECED?
hypoparathyroidism
MCC mycetoma in US?
Pseudoallesheria boydii
Asexual state: Scedosporium apiosperum
White grains
Tx: miconazole
Treatment for actnomycetoma
Nocardia species: sulfonamide
Actinomyces: penicillin
Actinomadura pelletieri
Red grains
In the U.S. the Splendore-Hoeppli phenomenon is most commonly associated with what organism?
Botryomycosis: S. aureus or pseudomonas
Treatment for sporotrichosis?
SSKI (iododerma, GI upset, hypothyroidism)
Itraconazole(CHF)--NOT ketoconazole
Basidiobolomycosis
Basidiobolus ranarum
Saprophyte with worldwide distribution
Infection m/c in kids in tropical/subtropical climates
Solitary, indurated, painless, subcutaneous nodule or swelling of the thigh or buttock
RX: SSKI, ketoconazole, TMP-SMX
Surgical excision not recommended
Characteritic clinical presentation of protothecosis?
olecranon bursitis
Treatment of lobomycosis
Antifungals not effective
Surgical excision, cryosurgery
Possibly clofazimine
MC cutaneous manifestation of histoplasmosis?
Oral ulcers
MC opportunistic infections in neutropenic pts?
Systemic Candidiasis
Aspergillosis
Systemic mycosis seen most commonly in HIV?
Disseminated histoplasmosis
Coccidioidomycosis
Penicillium marneffei
Cryptococcosis
MCC of disseminated aspergillosis with cutaneous involvement?
Aspergillus fumigatus
MCC primary cutaneous aspergillosis?
Aspergillus flavus
MC fungus in burn patients?
Fusarium
Aspergillus 2nd MCC
What stains the capsule of cryptococcus?
Mucicarmine or Alcian blue
India Ink
(PAS for organism)
Fungi causing molluscum-like umbilicated papules
Cryptococcus
Histoplasmosis
Penicilliosis
Coccidioidomycosis
Treatment for zygomycosis?
Ampho B
What two organism are most commonly isolated from subcutaneous phaeohyphomycosis?
Exophiala jeanselmei
Exophiala dermatitidis
What organism most commonly causes CNS phaeohyphomycosis?
Cladosporium trichoides (Cladophialophora bantiana)
All porphyrias are AD except which?
ALA-dehydratase deficiency
Gunther's (CEP)
Factors precipitating acute porphyria attacks?
All increase increased synthesis of cytochrome P450 in liver:
alcohol, drugs, infections, fasting/diets, changes in steroid production (eg. menses, OCP’s, pregnancy)
Difference in RBC fluorescence b/t EPP and CEP
EPP: transient
CEP: stable
What are the acute porphyrias?
No skin findings: AIP, ALA-D deficiency
Skin findings: VP, HCP
Receptors expressed by mast cells?
CD34, KIT & IgG receptors (FcγRII)
Do NOT express high affinity IgE receptors (FcεRI)
What are preformed mediators in mast cells?
hepain, histamine, neutrophilic & eosinophilic chemotactic factors, trytase/chymase
MC location for mastocytoma?
distal extremities (dorsal hand near wrist)
What lab test correlates wtih systemic disease in mastocytosis?
alpha and beta tryptase
100% pts w/ levels > 75 ng/ml had proven systemic dz
Preferred test over urinary histamine / metabolites
Urinary findings in mastocytosis?
Urinary histamine
Urinary MeImAA (1,4-methylimidazole acetic acid)
Metabolite of histamine
Often persistently elevated
Correlates to extent of disease
What systemic mastocytosis pts can be treated with imatinib?
FIP1L1-PDGFRA translocation OR
Lack 816 c-KIT mutation
(Does not work for pts w/ 816 c-KIT mutation)
What embryonic week does nail appartus first appear?
9th week
Which part of epithelium of nail apparatus keratinizes with a granular layer?
Hyponychium
(Nail matrix and bed do not)
Keratins in nail matrix keratinocytes?
K 6, 16, 17
Where in nail apparatus are melanocytes?
Distal matrix and suprabasal layers
Where in nail apparatus are Langerhans cells?
Suprabasal layers of matrix
More common in proximal vs. distal matrix
Growth rate of finger and toenails?
Fingernails: 2-3mm/month
Toenail: 1mm/month

Hair:1 cm/month
Onychomadesis
What causes nail pitting?
Abnormal keratinization of proximal nail matrix leads to clusters of parakeratotic cells in dorsal nail plate

AA, psoriasis, eczema
Causes of trachyonycia?
"20 nail dystrophy"
AA, LP, Psoriasis, Eczema
Drugs associated with multiple longitudinal bands of melanonychia?
AZT
5-FU
Doxorubicin
Psoralens
Association with Muehrcke's lines?
Hypoalbuminemia (nephrotic syndrome)
chemotherapy

Paired transverse white bands
Apparent luekonychia
Onychomadesis with pseudopyogenic granuloma of the proximal nail fold is a sign of what condition
Mild and transitory peripheral nerve injury

Seen in pts w/ phalanx, metacarpal or wrist bone fractures during or after cast immobilization
Which type of dermatophyte-associated onychomycosis is highly associated with HIV-infected patients?
Proximal subungual onychomycosis

T. rubrum
What drugs may be responsible for causing nailfold pyogenic granulomas?
Retinoids
Indinavir
Lamivudine
Methotrexate
EGFR inhibitors*
Chemotherapeutic agent causing subungual hemorrhages & absesses?
Taxanes (paclitaxel and docetaxel)
What is cheilitis glandularis?
PAINFUL hypertrophy of lower lip
Dilated/inflammed salivary secretory ducts
Mucopurulent secretion can be expressed
MC intraoral sites for lymphoma?
palate and vestibule
MC type : NHL, B-cell type
MC intraoral location for melanoma?
Hard palate, maxillary gingiva
MC oral lesion in Crohns?
Linear fissures/ulcers
Pyostomatitis vegetans
Creamy-yellow “snail track” pustules & ulcers set against a background of diffusely red mucosa
M/C labial, gingival and buccal mucosa
Dorsum of tongue is SPARED
Which proteins are found in reduced levels in humans with lipodystrophies?
1) Adiponectin: plays a role in regulating insulin sensitivity and glucose and lipid homeostasis
2) leptin: decreased level results in TG accumulation, insulin resistance, diabetes, hepatic steatosis
3) glut 4: when absent, leads to insulin resistance
Potential cause of death in congenital generalized lipodystrophy?
Hypertrophic cardiomyopathy (fatal); avg age of death 32
Gene defects in congenital generalized lipodystrophy?
AGPAT2
BSCL2 (seipin)
Distinguishing features between CGL and acquired generalized lipodystrophy?
AGL: women > men, more liver complications, often preceded by systemic illness, manifests later in childhood, involves palms/soles
Gene defects in Familial partial lipodystrophy?
Progressive loss of subq fat after normal childhood; predominantly extremities

FPLD1: Kobberling (spares face), unknown
FPLD2: Dunnigan (most common), AD, LMNA gene, fat accumulation in head/neck
FPLD3: PPARG mutation encoding PPARgamma
Associated systemic diseases in acquired partial lipodystrophy?
mesangiocapillary glomerulonephritis, low C3, C3 nephritic factor, autoimmunity
Pathogenesis of acquired partial lipodystrophy?
presence of adipsin (identical to factor D, imp in alternative complement pathway)
Fat cells + C3NeF leads to unopposed activation of alternate pathway and fat lysis
How is acquired lipodystrophy from NRTI different from PI-induced lipodystrophy?
No insulin resistance seen with NRTI
Pathogenesis linked to mitochondrial damage
What is Donohue syndrome?
Look like generalized lipodystrophy
Severe insulin resistance, AN, hirsutism
Elfin facies, severe IUGR, prominent nipples, loose skin, macrophalus, early death
Insulin receptor gene mutation
Which lipodystrophy?
Kidney disease + complement dysfunction
Barraquer-Simons (acquired partial)
Which lipodystrophy?
Full body atrophy + cirrhosis + death due to variceal bleeds
Lawrence (acquired generalized)
Which lipodystrophy?
Fat loss of the extremities + compensatory acromegalic facies
Berardinelli-Seip (Congenital generalized)
Which lipodystrophy?
Myocardial infarction due to hyperlipidemia + increased omental fat
Protease inhibitor-induced
Which lipodystrophy?
Diffuse fat atrophy except palms and soles + severe acanthosis nigricans
Berardinelli-Seip (Congenital generalized)
Which lipodystrophy?
High-pitched voice + short stature + acro-osteolysis + clavicular hypoplasia
Mandibuloacral variety (familial partial)
Which lipodystrophy?
Fat loss of the extremities + NO facial fat compensation
Kobberling variety (familial partial)
MC presentation of
cutaneous histoplasmosis & coccidiomycosis
in HIV?
Ulcers on face & mouth
Distribution of scabies in HIV + patients?
Distribution in HIV is ears, face & scalp
What test should be ordered if diagnosis of reactive arthritis (Reiter's syndrome) is made?
HIV
Strong association b/t the two
Diagnosis?
Eosinophilic folliculitis
Excoriated follicular papules & pustules on forehead & trunk
HIV meds associated with alopecia?
nucleoside analogs
Which HIV protein upregulates expression of HPV?
tat protein
Which type of neoplasms are HIV + pediatric pts more at risk of compared to adults?
HIV kids: higher incidence of MALT
More smooth muscle tumors (leiomyomas and leiomyosarcomas)
What immunophilin does sirolimus bind?
FKBP12
MOA of enfuvirtide
Fusion inhibitors for HIV infection
Blocks HIV from entering target cells by inhibiting gp41 mediated viral-cell membrane fusion
Dematiaceous organism causing dermatomycoses of the soles and toe webs, paronychia, onychomycosis
Scytalidium dimidiatum
Treatment for lobomycosis?
Surgical excision
Antifungals ineffective
MC organ system affected in paracoccidiodomycosis after lung and skin?
adrenal glands
MC fungus in burn patients?
fusarium
Opportunistic organism causing white superficial onychomycosis?
scopulariopsis
Candida species implicated in oropharyngeal Candidiasis in HIV infected patients
C. dublinensis
Candida species causing chronic paronychia?
C. parapsilosis
Candida species resistant to fluconazole
C. glabrata
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