ETAS_MCQ_11 disorder of hair and nails


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ETAS_MCQ_11 disorder of hair and nails

  1. 1. Disorder of hair and nails1) Fungal finger onycholysis usually results from:A. Fusarium sppB. Trichophyton rubrumC. Candida albicansCorrect ChoiceD. Scopulariopsis brevicaulisE. Trichophyton interdigitaleIt is widely accepted that C. albicans acts as colonizer that has found an ideal environment.2) The visible portion of the nail matrix is called the:A. HyponychiumB. EponychiumC. LunulaCorrect ChoiceD. Nail plateE. CuticleThe nail unit is comprised of six major components including the nail matrix, nail platic, cuticle, nailbed, anchoring portion, and the framing portion (proximal/lateral/distal folds). The lunula is acomponent of the distal matrix. It is grayish white because of the nature of the keratinization of itsepithelium3) Steatocystoma multiplex and natal teeth are associated with which of the following conditions?A. Rubenstein-Taybi syndromeB. Nail-patella syndromeC. Jackson-Sertole pachyonychia congenita (Type 2) Correct ChoiceD. Jadassohn-Lewandowsky pachyonychia congenita (Type 1)E. Coffin-Siris syndromeJackson-Sertole pachyonychia congenita (Type 2) is characterized by thickening of the nailbed andplate, steatocystoma multiplex, and natal teeth4) Atrophy of the isthmus is a characteristic histologic finding of which of the following conditions?A. Folliculitis DecalvansB. Parry-Romberg syndromeC. Discoid lupus erythematosusD. Lichen planopilaris Correct ChoiceE. Acne Keloidalis NuchaeLichen planopilaris is characterized by perifollicular lymphocytic infiltration with atrophy of theisthmus
  2. 2. 5) A patient with diffuse severe sudden hair loss developing 3 months after hospitalization forsepticemia likely has which of the following conditions?A. Uncombable hair syndromeB. Catagen effluviumC. Loose anagen syndromeD. Anagen effluviumE. Telogen effluvium Correct ChoiceTelogen effluvium typically ensues stressful events after a 2-3 month period6) Primary total dystrophic onychomycosis is due to:A. Trichophyton rubrumB. Candida sppCorrect ChoiceC. FusariumD. Scytalidium dimidiatumE. Scopulariopsis brevicaulisThe main characteristic of this immunologic disorder the simultaneous involvement of all the tissuesof the nail unit7) An 18 year-old girl who was hospitalized last month after a serious car accident is noted to havewhite transverse grooves on her fingernails and toenails. The most likely diagnosis is:A. Half-and-half nailsB. Twenty-nail dystrophyC. Terry’s nailsD. Mees’ linesE. Beau’s lines Correct ChoiceBeau’s lines are transverse grooves in the nails. They are caused by a generalized systemiccondition which disrupts nail formation. Conditions may include infection, myocardial infection,neurologic events and cytotoxic medications.8) Papular atrichia is caused by mutations in which gene?A. PatchedB. WinglessC. Fox foreheadD. Hairless Correct ChoiceE. Distal-less homeboboxHairless gene mutations cause papular atrichia.9) This nail finding is characteristically associated with what disorder?
  3. 3. A. Congenital heart failureB. HyperthyroidismC. CirrhosisD. Chronic renal failureCorrect ChoiceE. AnemiaLindsay’s nail is characteristically associated with chronic renal failure10) Koilonychia is most commonly seen in association with:A. Progressive systemic sclerosisB. Renal diseaseC. Impaired iron metabolismCorrect ChoiceD. Pulmonary diseaseE. Alopecia areataIn koilonychia (spoon nails), the nail is a concave shape with raised lateraledges. Iron-deficiency anemia and Plummer-Vinson syndrome have been associated withkoilonychia. Involvement of only the first three fingernails is suggestive of an occupational cause. Inearly infancy, koilonychia is a normal finding. Koilonychia is also seen in hemochromatosis, anautosomal recessive condition associated with increased intestinal iron absorption and increasediron deposition leading to metallic-grey hyperpigmentation of the skin, hepatomegaly, diabetes,hypogonadism, polyarthritis, and cardiac abnormalities11) What condition most likely describes a hair defect with ruffled cuticle in 4-year old female withblond hair?A. Loose anagen hair syndromeCorrect ChoiceB. Pili annulatiC. Pili tortiD. Wooly hairE. MonilethrixLoose anagen hair syndrom occurs primarily in young, females with blond hair. The defect results inearly keratinization of the inner root sheath which leads to poor anchoring. Examination of hairshaft reveals ruffled proximal cuticle12) How long should last the wash-out period before performing mycologic investigations in aclinically recurrent onychomycosis treated by terbinafine, itraconazole or fluconazole:A. Four monthsB. Two monthsC. Three monthsCorrect ChoiceD. Six monthsE. One monthThis is an interesting point, because we know that these systemic antifungals are retained in thekeratin of the toenails up to six months after the treatment has been stopped.Therefore this answer could have been debatable were it not for the presence of fungi in the nail
  4. 4. bed (which is the primary location of the disease) and where the scraping should be properlyperformed for mycologic testing after removal of a piece of nail as proximal as possible13) Which of the following is NOT a cause of red lunulae?A. EndocarditisCorrect ChoiceB. Carbon monoxide poisoningC. Rheumatoid arthritisD. ImuranE. PsoriasisEndocarditis is not a cause of red lunulae. Alopecia areata, psoriasis, prednisone for connectivetissue diseases, carbon monoxide poisoning, cardiac failure, SLE, RA, COPD, CO2, cirrhosis, imuranand radiation are the causes of this condition14) The "ragged cuticle" seen in dermatomyositis is also known as:A. EmperipolesisB. Plummers signC. ventral pterygiumD. Candy-cane nailsE. Samitz signCorrect ChoiceCuticular fraying from dermatomyositis is called the Samitz sign or "ragged cuticle". The otheroptions are not associated with dermatomyositis. A ventral pterygium can be seen in systemicsclerosis. There is no such sign as Plummers sign. Candy-cane nails are seen in Dariers disease.Emperipolesis is seen in Rosai-Dorfman disease and is not a nail finding15) Acute paronychia is most commonly caused by which of the following organisms?A. Trichophyton rubrumB. Staphylococcus aureus Correct ChoiceC. Candida tropicalisD. Candida albicansE. Pseudomonas aeruginosaStaphylococcus aureus is the most common cause of acute paronychia16) The combination of poliosis, uveitis, deafness, and vitiligo are characteristic of which of thefollowing conditions?A. Vogt-Koyanagi-Harada syndrome Correct ChoiceB. Cornelia de Lange SyndromeC. PiebaldismD. Waardernburg syndromeE. Cronkhite-Canada syndromeVogt-Koyanagi-Harada syndrome is characterized by poliosis, uveitis, deafness, and Vitiligo
  5. 5. 17) A patient with curly hair that straightened after puberty, enamel hypoplasia, dental pits, andincreased bone density likely has a mutation in which of the following genes?A. SPINK 5B. Distal-less homeobox-3 gene (DLX3) Correct ChoiceC. SLURP 1D. Bone morphogenetic protein type 2E. Vascular-endothelial growth factor receptor 3Distal-less homeobox-3 gene (DLX3) mutations cause trichodentoosseous syndrome, which ischaracterized by curly hair that straightens in the 2nd-3rd decades, enamel hypoplasia, dental pits,and increased bone density18) Argininosuccinic aciduria is characteristically associated with this hair finding –name thecondition pictured below:A. TrichoschisisB. MonilethrixC. Pili trianguli et canaliculiD. Trichorrhexis nodosa Correct ChoiceE. Trichorrhexis invaginataTrichorrhexis nodosa is a finding in argininosuccinic aciduria19) Signs of virilization include all of the following EXCEPT:A. Increased muscle mass in the limb girdlesB. ClitoromegalyC. Temporal baldingD. Centripetal obesityCorrect ChoiceE. AcneCentripetal obesity is a sign of cortisol excess not of virilization. In addition to the those listedabove, irregular or absent menses and deepening of the voice are signs of virilization20) Distal subungual onychomycosis is most often caused by:A. Epidermophyton floccosumB. Trichophyton megniniiC. Trichophyton mentagrophytesD. Trichophyton rubrumCorrect ChoiceE. Trichophyton schoenleiniiTrichophyton rubrum is the most common organism involved in distal subungual onychomycosis.21) Yellow nails can be seen in each of the following syndromes except:
  6. 6. A. BronchiectasisB. HyperlipidemiaCorrect ChoiceC. Thyroid diseaseD. PenicillamineE. Tobacco useYellow discoloration of the nail plate can be associated with bronchiectasis or underlying pulmonarydisease such as COPD. Other associated conditions include lymphedema, rheumatoid arthritis,thyroid disease, malignancies, penicillamine, and chronic sinusitis or bronchitis. Hyperlipidemia isnot associated with nail changes22) Lindsays nails (distal nail normal, proximal nail white) is characteristic of:A. Plummer-Vinson syndromeB. LEOPARD syndromeC. HemochromatosisD. Chronic renal failureCorrect ChoiceE. Ectodermal dysplasiaLindsays nails are characteristic of chronic renal failure and indicate nailbed edema. The remainingoptions are causes of koilonychia, a spooning defect of the nail23) A woman with hypopigmented in lines of Blaschko and scarring alopecia likely suffers which ofthe following conditions?A. Chondrodysplasis punctataB. Anhidrotic Ectodermal DysplasiaC. Bloch-Sulzberger Syndrome Correct ChoiceD. Rothmund-Thomson SyndromeE. Focal Dermal HypoplasiaBloch-Sulzberger Syndrome (incontinentia pigmenti)24) The most common cause for superficial type onychomycosis is:A. Epidermophyton floccosumB. Scopulariopsis brevicalisC. Trichophyton rubrumD. Fusarium oxysporumE. Trichophyton mentagrophytesCorrect ChoiceT. mentagrophytes is the most common cause of superficial type onychomycosis. Other causesinclude: Fusarium, Acremonium and Aspergillus spp25) Congenital generalized hypertrichosis is associated with which of the following symptoms?A. Methimazole teratogenicity
  7. 7. B. Gingival fibromatosis Correct ChoiceC. Androgen-secreting ovarian tumorsD. Pituitary insufficiencyE. DistichiasisGingival fibromatosis. Congenital Generalized Hypertrichosis with Gingival Fibromatosis is a geneticcondition with hypertrichosis on the face and upper body and gingival hyperplasia and fibromatosis26) A significantly increased number of "club hairs" on a pull test is indicative of:A. Anagen effluviumB. Alopecia areataC. Angrogenetic alopeciaD. Telogen effluviumCorrect ChoiceE. Normal hair anatomyTelogen hairs are characteristically club shaped. An increased number of telogen hairs on hair pullwould be suggestive of telogen effluvium. Anagen hairs have a curled appearance at the root. Whilea small number of telogen hairs are normal, a significantly increased number would not be normalhair anatomy27) Trichobezoar is is a potentially life-threatening complication of:A. Discoid lupusB. Anhidrotic ectodermal dysplasiaC. Alopecia areataD. Lichen planopilarisE. Trichotillomania Correct ChoiceNEEDS EXPLANATIONS28) Which of the following is an example of an apparent leukonychia?A. Mees linesB. Terrys linesC. OnychoschiziaD. Hutchinsons linesE. Muehrckes linesCorrect ChoiceAn apparent leukonychia disappears with pressure and is a problem with nail bed, not nail plate.Apparent leukonychia may be indicative of overall health compromise. Muehrckes lines are pairedwhite bands and are a type of apparent leukonychia29) Endonyx onychomycosis is due to:A. Trichophyton soudanenseCorrect ChoiceB. Trichophyton rubrum
  8. 8. C. Scytalidium dimidiatumD. ScopuliaropsisE. Trichophyton mentagrophytesEndonyx onychomycosis may also be observed with T. violaceum. This is the equivalent of endothrixinfection of the hair associated with tinea capitis30) A patient with yellow nail syndrome should be evaluated for systemic disease involving whichorgan system?A. HematologicB. HepaticC. Pulmonary Correct ChoiceD. CardiovascularE. RenalYellow nail syndrome is characterized by slowly growing, yellow nails that are excessively curved.The lunulae and cuticles are absent. It has been associated with a array of pulmonary diseasesincluding tuberculosis, asthma, pleural effusion, bronchiectasis, sinusitis, bronchitis, and COPD31) The defect associated with the hair finding shown in the image is:A. hHB6/hHB1Correct ChoiceB. hHB8/hHB3C. hHB10/hHB5D. hHB9/hHB4E. hHB7/hHB2hHB6/hHB1 keratins are associated with Monilethrix. Other findings include: short, brittle hair,extensor keratosis pilaris, mental retardation, syndactyly, cataracts, and nail/teeth abnormalities32) Which of the following syndromes does not have Dorsal pterygium as a feature:A. Lesch-Nyhan syndromeB. Lamellar ichthyosisCorrect ChoiceC. Cicatricial pemphigoidD. Chronic GVHDE. Lichen planusDorsal pterygium (scarring of the proximal nail fold) is caused by lichen planus, acrosclerosis,onychotillomania, Lesch-Nyhan syndrome, chronic GVHD, SJS/TEN and cicatricial pemphigoid. All ofthe listed items are correct except lamellar ichthyosis. It does not have this nail finding.33) Which part of the matrix gives rise to the dorsal nail plate?A. Distal matrixB. Dorsal matrixC. Ventral matrix
  9. 9. D. Lateral matrixE. Proximal matrix Correct ChoiceThe proximal matrix gives rise to the dorsal nail plate, while the distal matrix gives rise to theventral nail plate34) Yellow nail syndrome can be associated with:A. D-penicillamine use in patients with rheumatoid arthritisCorrect ChoiceB. Alopecia areataC. Wilson’s diseaseD. DoxorubicinE. Minocycline use in acne patientsAnswer: A. D-penicillamine use in patients with rheumatoid arthritis.Yellow nail syndrome has been described in patients with rheumatoid arthritis receiving thiolcompounds including D-penicillamine and bucillamine. Reversal of the condition has been describedafter discontinuation of the drug35) A patient with cirrhosis has noticed that the proximal aspect of his nails have become white.This characteristic finding is called:A. Muehrckes linesB. Mees linesC. Terrys linesCorrect ChoiceD. Beaus linesE. Half-and-half nailsTerry’s lines are described as an abnormal white appearance of the nail except for the most distalportion. It has been described in association with cirrhosis, congestive heart failure, and diabetesmellitus36) A defect in which part of the nail cause a true leukonychia?A. LunulaB. Nail plateCorrect ChoiceC. HyponychiumD. Nail bedE. CuticleTrue leukonychia is caused by an acute stress to the nail and manifests in the nail plate. Examplesinclude Terrys nails and Mees lines. Apparent leukonychia, on the other hand, are a result ofproblems in the nail bed and are indicative of overall health compromise. Examples of apparentleukonychia include Lindsays and Muehrckes nail37) Tay’s syndrome is characterized by this hair condition pictured below – identify the condition:A. MonilethrixB. TrichoschisisCorrect Choice
  10. 10. C. Trichorrhexis nodosaD. Trichorrhexis invaginataE. Pili trianguli et canaliculiTay’s syndrome is also known as trichothiodystrophy which is characterized by trichoschisis or cleanfracture of the hair shaft38) A patient with wooly hair, diffuse palmoplantar keratoderma, and right ventriculararrhythmogenic cardiomyopathy likely has a mutation in which of the following genes?A. HHB6B. PlakophilinC. SPINK 5D. Plakoglobin Correct ChoiceE. SLURP 1Naxos syndrome is caused by mutations in the plakoglobin gene. Its clinical features include woolyhair, diffuse palmoplantar keratoderma, and right ventricular arrhythmogenic cardiomyopathy39) Black nail is caused by:A. Proteus mirabilisCorrect ChoiceB. Staphylococcus aureusC. Hortaea werneckiiD. Pseudomonas spp.E. Trichophyton mentagrophytesNail infections with Proteus mirabilis. T. mentag causes white discoloration of the nail andPseudomonas will cause a green or yellow/green discoloration. Hortaea werneckii causes tineanigra. S. aureus does not cause black nails40) The combination of a low-set hairline and synophrys is seen in which of the followingconditions?A. Noonan SyndromeB. Turner SyndromeC. Waardenburgs syndromeD. Cornelia de Lange Syndrome Correct ChoiceE. Werner syndromeExplanation41) Pili trianguli et canaliculi is the characteristic hair fiding in which of the following disoders?A. MonilethrixB. Naxos syndromeC. Anhidrotic ectodermal dysplasia
  11. 11. D. Pili annulatiE. Uncombable hair syndrome Correct ChoicePili trianguli et canaliculi, characterized by a triangular hair shaft with a central longitudinal groove,is the finding seen in uncombable hair syndrome42) The most common cultured agent in chronic paronychia associated with a black hue of thelateral edge of the nail plate is:A. Trichophyton rubrumB. gram-negative bacteriaC. Scytalidium dimidiatumD. Scopulariopsis brevicaulisE. Candida spp.Correct ChoiceChronic paronychia is likely a condition that represents a contact reaction to allergens or irritants. Itoften is superinfected with candida. This underlies the importance of treatment with both anantifungal and a topical steroid43) Cutaneous signs of cortisol excess include all of the following EXCEPT:A. Atrophic/fragile skinB. Dorsocervical/Supraclavicular fat padsC. Striae distensaeD. PlethoraE. Peripheral obesityCorrect ChoiceAll of the listed options are signs of cortisol excess except peripheral obesity. The most commonpattern of obesity in patients with cortisol excess is centripetal or central obesity44) Which genetic defect could be expected in an individual with these nail findings in addition toshort stature, capillary malformations, beaked nose, mental retardation, and cryptorchidism?A. ectodysplasinB. hHB1/hHB6C. GNASD. CREBBPCorrect ChoiceE. ERCC6Rubenstein-Taybi syndrome is a genetic disorder caused by mutations in the gene encoding CREBbinding protein. This protein acts as a nuclear protein involved in coactivating cAMP regulated geneexpression. Clinically, patients have capillary capillary malformations, beaked nose, mentalretardation, cryptorchidism, and broad thumbs45) Which autoantibody is associated with these cuticular changes, shawl sign, and overallfavorable prognosis?A. Scl-70B. High titered ANA with speckled pattern
  12. 12. C. Mi-2Correct ChoiceD. RoE. Jo-1Dermatomyositis has many different cutaneous manifestations including heliotrope rash, Gottronspapules and sign, mechanics hand and poikiloderma atrophicans vasculare. Antibodies to Mi-2 indermatomyositis are associated with cuticular changes, shawl sign and a overall favorable prognosis46) The most common nail infection that causes white superficial/proximal white subungalonychomycosis is:A. Trichophyton rubrumCorrect ChoiceB. Trichophyton mengatrophytesC. Scatylidium hyalinumD. Aspergillus sppE. Hendersonula toruloideaT. rubrum is the most common cause of white superficial or proximal white subungalonychomycosis. This can also be caused by aspergillus. T. mentag causes superficial onychomycosisand less commonly distal subungal type. Hendersonula and Scatylidium cause lateral invasion withparonychia47) Acquired progressive kinking of hair is a complication of which of the following medications?A. SlfonamidesB. AntimetabolitesC. AntimalarialsD. DapsoneE. Retinoids Correct ChoiceRetinoids can cause acquired progressive kinking of hair48) Follicular atrophoderma is a feature of which of the following conditions?A. Papular atrichiaB. Tricho-rhino-phalangeal syndromeC. Menkes kinky hair syndromeD. Tricho-dento-osseous syndromeE. Bazex syndrome Correct ChoiceBazex syndrome is characterized by follicular atrophoderma, hypotrichosis, hypohidrosis, andmutiple BCCs of face.49) Proximal white subungual onychomycosis with polydactylous involvement needs at least onelaboratory test:A. HIVCorrect ChoiceB. T-helper CD4 lymphocytes
  13. 13. C. Blood countD. Sedimentation rateE. Liver enzymesThis type of onychomycosis has a prognostic value: It means that the CD4 lymphocytes are below450.50) Yellow nail syndrome is associated with which of the following?A. Diabetes mellitusB. Dermatophyte infectionC. Multiple myelomaD. Lymphedema and bronchiectasis Correct ChoiceE. PanhypopituitarismLymphedema and bronchiectasis are associated with yellow nail syndrome51) This hair disorder and mutations in ATP7A (MNK) gene are found in what genodermatosis?A. Crandalls syndromeB. Bjornstad syndromeC. TrichothiodystrophyD. Menkes kinky hair syndrome Correct ChoiceE. Bazexs follicular atrophodermaMenkes kinky hair syndrome is characterized by pili torti and mutations in APT7A (MNK) gene.Bjornstad syndrome, Crandall’s syndrome and Bazex’s follicular atrophoderma are associated withpili torti but are not associated with this mutation52) Which of the following conditions with hair abnormalities is caused by mutations in a DNAhelicases?A. Anhidrotic Ectodermal DysplasiaB. KID SyndromeC. Rothmund-Thomson Syndrome Correct ChoiceD. Hidrotic Ectodermal DysplasiaE. Incontinentia PigmentiExplanation53) Pathognomonic nails changes in HOOD syndrome or Hereditary osteoonychodysplasia areA. Pterygium inversumB. Triangular lunulaeCorrect ChoiceC. Yellow nailsD. Red and white longitudinal banding
  14. 14. E. Pincer nailsIn the HOOD syndrome (Hereditary osteoonychodysplasia) or the Nail-Patella syndrome, patientshave different degrees of nail dysplasia. In general, the thumbs are most severely involved and naildisease decreases in severity from the second to the fifth digits. Characteristic nail changes includelongitudinal ridging, koilonychia, and splitting. Toenails are rarely involved. Pathognomonic nailchange is the V shaped triangular lunulae with the distal peak in the midline54) What is the mode of inheritance of this disorder of the hair shaft?A. SporadicB. Autosomal recessiveC. X-linked recessiveD. Autosomal dominantCorrect ChoiceE. X-linked dominantMonilethrix is an autosomal dominant condition with variable penetrance. The hair shaft has anelliptical or beaded hair appearance55) Triangular lunulae are:A. associated with a renal cell carcinomaB. associated with hyperplastic patellaeC. part of the syndrome caused by a LMX1b mutationCorrect ChoiceD. associated with a mutation of SERCA2E. associated with a mutation of ATP2C1Triangular lunulae are part of the Nail-Patella syndrome. Findings in this syndrome include LMX1bmutation, hypoplastic or absent patellae, nephropathy, a "Lester Iris" - hyperpigmentation of thepupillary margin, cataracts, heterochromia irides and glaucoma. ATP2C1 and SERCA2 are associatedwith Hailey-Hailey and Darier-White disease respectively.56) Fraying of the cuticles is a sign of which of the following conditions?A. Lead poisoningB. Lupus erythematosusC. Dermatomyositis Correct ChoiceD. Systemic sclerosisE. HypothyroidismDermatomyositis can be associated with fraying of the cuticles (Samitz sign).57) Frontal bossing, saddle nose, hypoplastic midface, peg shaped or conical teeth andhypopigmented short sparse scalp and body hair are prominent features of which of the followingA. Hidrotic ectodermal dysplasiaB. Arginosuccinic aciduriaC. Monilethrix
  15. 15. D. Christ Siemens Touraine syndromeCorrect ChoiceE. Pachyonychia congenitaChrsit Siemens Touraine syndrome also known as Hypohidrotic ectodermal dysplaisa or anhidroticectodermal dysplasia is X-linked recessive disroder that presents in infancy to early childhood.Features include hypo-anhidrosis with increased body temperature, hypopigmented sparse scalpand body hair, frontal bossing, saddle nose, hypoplastic midface, abnormal ears, hypo-anodontia,and increased bronchopulmonary inections. Cloustons syndrome or hidrotic ectodermal dysplasia isAD and associated with palmoplantar keratoderma, nail dystrophy, short sparse scalp hair, andtufting of the terminal phalanges. Arginosuccinic aciduria is associated with seizures, trichorrhexisnodosa, failure to thrive, and hyperammonemia. Monelothrix is associated with beaded hairs andkeratosis pilaris. Pachyonychia congenita is associated with nail dystrophy and focal palmoplantarkeratoderma58) Which of the following syndromes does NOT have the finding shown in the image as part of thespectrum?A. CrandallCorrect ChoiceB. Argininosuccinic aciduriaC. CitrullinemiaD. Menkes Kinky HairE. NethertonCrandall syndrome has pili torti as its hair finding. The other options all have trichorrhexis nodosa asone of the hair findings in the syndrome. Trichorrhexis nodosa is the fraying of hair ends so that theresemble a "broom-stick" or "paint brush"59) Which of the following is caused by mutations in gap junction proteins?A. Hidrotic ectodermal dysplasiaCorrect ChoiceB. Anhidrotic ectodermal dysplasiaC. Netherton syndromeD. Naxos syndromeE. Rothmund-Thompson syndromeHidrotic ectodermal dysplasia is caused by mutations in connexin 30, which is a gap junction protein60) Proximal white subungual onychomycosis associated with paronychia is never due to:A. Fusarium sppB. Candida spp.Correct ChoiceC. Scopulariopsis brevicaulis sppD. Acremonium sppE. Aspergillus spp.All of the molds indicated, here, may be responsible for this condition, but principally 2,3,461) Muehrckes nails are most often associated withA. Hyperalbuminemia
  16. 16. B. HyperthyroidismC. HypoalbuminemiaCorrect ChoiceD. HypothyroidismE. None of these answers are correctMuehrckes nails are most associated with hypoalbuminemia. They represent transverse doublewhite lines that are the abnormality of the vascular bed, probably a localized edematous statesecondary to the hypoalbuminemia62) Anonychia is not a feature of which of the following syndromes?A. Hidrotic ectodermal dysplasiaCorrect ChoiceB. DOOR syndromeC. Nail-Patella syndromeD. Coffin-Siris syndromeE. COIF syndromeHidrotic ectodermal dysplasia features hyperconvex nails, micronychia and nail dystrophy. All of theother listed options can cause anonychia. COIF syndrome is Conginital Onychodysplasia of the IndexFinger and DOOR syndrome is Deafness, Congenital Onychodystrophy Recessive Form. Coffin-Sirissyndrome is characterized by: nail onychodysplasia or aplasia (usually of the fifth finger or toe),coarse facies including bushy eyebrows, scant scalp hair, full lips, and microcephaly, mental/growthdeficiency, short distal phalanges, and other abnormalities63) A 4 year-old boy has a single circular alopecic atrophic patch on the vertex of the scalp. Thismost likely is due to which of the following conditions?A. Triangular AlopeciaB. Aplasia cutis congenita Correct ChoiceC. Congenital syphilisD. Keratosis Follicularis Spinulosa DecalvansE. Morphea en coup de sabreAplasia cutis congenita often affects the vertex of the scalp and heals with a circular atrophicalopecic scar64) Splinter hemorrhage of the nail can be seen with which of the following parasitic infections?A. GnathostomiasisB. DracunculiasisC. Trichinosis Correct ChoiceD. ScabiesE. SparganosisTrichinosis can cause splinter hemorrhage of the nails.65) What is the gene defect that causes red hair?
  17. 17. A. Melanocortin 1 receptorCorrect ChoiceB. P proteinC. Tyrosine aminotransferaseD. TyrosinaseE. Tyrosinase-related protein 1Defects in melanocortin 1 receptor lead to the phenotypic appearance of red hair. Individuals withred hair have increased ratio of pheomelanin to eumelanin and are at increased risk for melanoma66) Chronic paronychia is most commonly caused by which of the following organisms?A. Staphylococcus aureusB. Pseudomonas aeruginosaC. Candida albicans Correct ChoiceD. Candida tropicalisE. Trichophyton rubrumCandida albicans is the most common pathogen associated with chronic paronychia67) The common culprit of proximal white subungual onychomycosis is :A. Fusarium sppB. Candida albicansC. Scydalidium dimidiatumD. Trichophyton rubrumCorrect ChoiceE. Trichophyton interdigitaleT. rubrum is responsible for more than 95% of these cases.68) The syndrome including mental and physical retardation, convulsions, episodicunconsciousness, liver enlargement, skin lesions, and dry and brittle hair showing trichorrhexisnodosa microscopically and fluorescing red. is:A. CitrullinemiaB. Argininosuccinic aciduriaCorrect ChoiceC. Crandalls syndromeD. Bjornstads syndromeE. Bazex Follicular AtrophodermaArgininosuccinic aciduria has the features listed including trichorrhexis nodosa and red fluorescenceof the hair. Bazexs follicular atrophoderma and Crandall syndrome both have findings of pili tortiand citrullinemia has trichorrhexis nodosa. None of the other options have red fluorescence as afeature69) The epithelium that lies on the volar surface of the digit is the:A. Hyponychium Correct Choice
  18. 18. B. EponychiumC. Nail plateD. Nail bedE. Nail matrixThe epithelium that lies on the volar surface of the digit is the hyponychium. The nail bed is theactual nail made up of tightly packed onychocytes. The nail matrix is the epithelium which startsmid distal phalanx which generated the nail plate. It keratinizes without a granular layer anddetermines the thickness of the nail plate. The nail bed is the thin epithelium immeduately beneaththe nail plate70) Triangular lunulae are seen in what disorder?A. Gorlin’s syndromeB. Dyskeratosis congentiaC. Nail-patella syndrome Correct ChoiceD. Neurofibromatosis IIE. Papillon-Lefevre syndromeTriangular lunulae are seen in nail-patella syndrome which is characterized by mutation in LMX1b71) Which of the following are characteristics of Trichorhinophalangeal syndrome?A. Brittle hair, short stature, cerebellar ataxiaB. Shortened phalanges, sparse hair, bulbous noseCorrect ChoiceC. Kinky hair, frontal bossing, small widely spaced teeth with poor enamelD. Sparse fine hair, Short extremities, ImmunodeficiencyE. Palmoplantar keratoderma, trichorrhexis nodosa, sinus infectionsCharacteristic features of trichorhinophalangeal syndrome are shortened phalanges, sparse hair,and bulbous nose72) Blue lunulae are associated with each of the following except:A. Wilson’s diseaseB. PhenolphthaleinC. ArgyriaD. QuinacrineE. Cardiac failureCorrect ChoiceBlue lunulae have been reported in association with argyria, Wilson’s disease, hereditary acrolabialtelangectasia, paronychia, phenolphthalein, quinacrine, topical bichloride, and mercury exposure73) The best test for Cushing syndrome is:A. Plasma LHB. Plasma DHEAC. Plasma prolactinD. Overnight dexamethasone suppresion testCorrect Choice
  19. 19. E. Plasma testosteroneThe overnight dexamethasone suppression test is the test of choice for diagnosis of Cushingsyndrome. In this test, a dose of dexamethasone is given at 11pm and the plasma cortisol level isdrawn the following morning. If the Cushing syndrome is from an adrenal tumor or an ectopic ACTHproducing tumor, there is no change in cortisol secretion. A normal response is suppression of thecortisol level by the extra dexamethasone. If the Cushings is from a pituitary tumor, at low doses ofdexamethasone there are no changes in cortisol levels while at high doses of dexamethasone therewill be a normal suppression. The other listed items are not used for diagnosis of Cushingsyndrome.74) Which part of the nail complex results in nail pits?A. Proximal nail foldB. Nail bedC. Proximal nail matrixCorrect ChoiceD. Nail plateE. Dorsal nail matrixPitting of the nails are punctate depressions that migrate distally and result from defect in theproximal nail matrix. It may be seen in conditions such as psoriasis, eczema and alopecia areata75) Spotted red lunulae are absent in which of the following conditions?A. Rheumatoid arthritisB. Systemic lupus erythematosusC. Keratosis follicularisCorrect ChoiceD. Lichen planusE. Alopecia areataKeratosis follicularis has nail findings of v-shaped nicking of the distal nail, subungualhyperkeratosis and red/white alternating longitudinal bands. In addition to those listed above,psoriasis can also cause spotted red lunulae76) A patient with sparse hair, a pear-shaped broad nose and cone-shaped epiphyses likely sufferswhich of the following conditions?A. Hay-Wells SyndromeB. Focal Dermal Hypoplasia (Goltz syndrome)C. Trichodentoosseous SyndromeD. Tricho-rhino-phalangeal Syndrome Correct ChoiceE. Ectrodactyly-Ectodermal dysplasia-Clefting (EEC)Tricho-rhino-phalangeal Syndrome is characterized by sparse hair, a pear-shaped broad nose andcone-shaped epiphyses77) A patient with diffuse hair loss developing after a thallium scan likely has which of the followingconditions?A. Loose anagen syndrome
  20. 20. B. Telogen effluviumC. Anagen effluvium Correct ChoiceD. Uncombable hair syndromeE. Catagen effluviumAnagen effluvium can result from infusions of thallium78) Yellow nail syndrome has been associated with use of which of the following treatments forRheumatoid Arthritis?A. InfliximabB. D-penicillamineCorrect ChoiceC. AdalimumabD. MethotrexateE. EtanerceptD-penicillamine has been associated with the development of yellow nail syndrome in patiens withrheumatoid arthritis. The other options are used to treat RA, but have not been associated withdevelopment of this syndrome79) Blue lunulae are characteristic of which disease?A. ClubbingB. Rubenstein-Taybi syndromeC. Yellow nail syndromeD. Wilsons disease Correct ChoiceE. Hypertrophic pulmonary osteoarthropathyWilsons disease is associated with characteristic blue lunulae80) Which genodermatosis characterized by broad thumbs and this nail disorder pictured below?A. Rubenstein-Taybi syndrome Correct ChoiceB. Proteus syndromeC. Tuberous sclerosisD. Rothmund-ThompsonE. Pachyonychia congenitaRubensteom-Taybi syndrome is characterized by broad thumbs and broad nails or brachyonychia.This syndrome is characterized by mutations in CREB-binding protein81) What is the common cause of the “one hand-two-foot” syndrome?A. Trichophyton rubrumCorrect ChoiceB. Trichophyton mentagrophytes ( var. Interdigitale)C. Candida albicans
  21. 21. D. Scytalidium dimidiatumE. Scopulariopsis brevicaulisThis condition is due to T. rubrum in 90% of the cases , T. interdigitale and Scytalidium dimidiatumshare the 10% left82) Regarding androgens in women, which of the following statements is NOT correct?A. Eyebrows, eyelashes and vellus hairs are androgen-dependentCorrect ChoiceB. There are no differences in eyelashes, eyebrows and vellus hair-bearing areas in men and womenC. Testosterone binds the androgen receptorD. The hair follicle requires conversion of testosterone to dihydrotestosterone for expression ofandrogen actionE. Dihydrotestosterone binds the androgen receptorOnly testosterone and dihydrotestosterone bind the androgen receptor, Eyebrows, eyelashes andvellus hairs are NOT androgen-dependent, thus there are no differences between these areas inmen and women, and the hair follicle requires conversion of testosterone to dihydrotestosterone forexpression of androgen action83) Ventral pterygium pictured below is characteristically associated with what disorder?A. Chronic GVHDB. Cicatricial pemphigoidC. SJS/TEND. Lichen planusE. Systemic sclerosisCorrect ChoiceVentral pterygium is most characteristically associated with systemic sclerosis. All other choices areassociated with dorsal pterygium84) Arsenical poisoning is associated with what nail finding?A. Oil spotsB. OnycholysisC. Muehrckes linesD. Mees lines Correct ChoiceE. HapalonychiaArsenical poisoning is characteristically associated with Mees lines85) The hair finding characterized by an invagination of the distal hair shaft into the cup formed bythe proximal hair shaft is:A. Pili tortiB. Pili triangulatiC. TrichoschisisD. Trichorrhexis invaginataCorrect Choice
  22. 22. E. Trichorrhexis nodosaThe above description is that of trichorrhexis invaginata, which is characteristic of Nethertonsyndrome. Other findings include: ichthyosis linearis circumflexa and atopy. It is caused by themutation of the serine protease inhibitor, SPINK586) Psoriatic onycholysis is caused by psoriasis in which of the following nail subunits?A. Proximal nail foldB. HyponychiumC. Nail plateD. Nail bed Correct ChoiceE. Nail matrixNail bed involvement by psoriasis often causes onycholysis87) A patient with synophrys, dystopia canthorum, and heterochromia irides likely has which of thefollowing hair abnormalities?A. Trichorrhexis invaginataB. Scarring alopeciaC. White forelock Correct ChoiceD. Diffuse thinningE. Trichorrhexis nodosaWhite forelock is a common feature of Waardenburg syndrome88) Mees lines are:A. double white transverse lines from abnormal vascular bedB. local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail foldsC. transverse white lines that affect all nails, grow out with nail growthCorrect ChoiceD. vertical black lines on a single or multiple nailsE. brownish macules beneath the nail plateMees lines are transverse white lines that affect all nails, grow out with nail growth. They are seenin arsenic poisoning, rheumatic fever, congenital heart failure, leprosy and with significant systemicdisease. Brownish macules beneath the nail plate are oil spots, seen in psoriasis. Onychophosis isthe local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail folds. Thedouble white transverse lines from abnormal vascular bed are Muehrckes lines which are caused bya nephrotic syndrome, low albumin, liver disease or malnutrition89) Highest graft survival in hair transplantation is achieved through the use of:A. MicrograftsB. Follicular unitsCorrect ChoiceC. Single hairsD. Minigrafts
  23. 23. E. 4-5 mm plugsSeager et. al examined the survival rate of single hair grafts and compared them to follicular unitgrafts in a single patient. They found that there was a much higher survival rate in the follicular unitgrafts. They hypothesized that the extra tissue surrounding the follicular unit grafts protected themfrom crush injury90) This characteristic nail finding is seen in what disorder?A. Dariers diseaseCorrect ChoiceB. Coffin-Siris syndromeC. Lichen planusD. COIF syndromeE. Pachyonychia congenitaAlternating red and white longitudinal bands on the nail are characteristic of Darier’s disease. Othernail findings include subungual hyperkeratosis and distal v-shaped nicking91) A commonly known cause of splinter hemorrhages in the nail is endocarditis. Which of thefollowing would be unlikely to cause splinter hemorrhages?A. TrichinosisB. VasculitisC. Rheumatoid arthritisCorrect ChoiceD. TraumaE. PsoriasisRheumatoid arthritis is not a cause of splinter hemorrhages of the nail. Endocarditis is the mostcommonly thought of cause of splinter hemorrhages. Trichinosis, trauma, psoriasis and vasculitisalso can be causes of this also and should be considered when splinter hemorrhages are seen92) The combination of ankyloblepharon, ectodermal dysplasia, and cleft palate with wiry sparsehair is characteristic of which of the following syndromes?A. Tricho-rhino-phalangeal SyndromeB. Focal Dermal Hypoplasia (Goltz syndrome)C. Trichodentoosseous SyndromeD. Ectrodactyly-Ectodermal dysplasia-Clefting (EEC)E. Hay-Wells Syndrome Correct ChoiceHay-Wells Syndrome, also known as AEC (Ankyloblepharon-Ectodermal dysplasia-Clefting) ischaracterized by wiry, sparse hair or alopecia, ankyloblepharon, PPK, partial anhidrosis, cleft lip,palate, absent, and dystrophic nails93) Distal subungual onycholysis associated with paronychia is due to:A. Scytalidium dimidiatumCorrect ChoiceB. Fusarium sppC. Trichophyton mentagrophytes
  24. 24. D. Candida sppE. Trichophyton rubrumThis mold is the only one responsible for paronychia in DLSO and is more often observed in fingerthan in toenails94) A subungual hematoma covering 50% of the nailbed should be managed by which of thefollowing?A. Trephination of the nail plateB. Needle aspiration of the hematomaC. No treatmentD. Pressure dressingE. Removal of the nail plate Correct ChoiceRemoval of the nail plate is necessary for management of hematomas covering more than 25% ofthe nailbed95) Terrys nails are seen in which of the following conditions?A. Pulmonary fibrosisB. Bladder carcinomaC. Renal failureD. Aerodigestive carcinomaE. DiabetesCorrect ChoiceTerrys nails are described as all but the distal 2mm of each nail evenly white due to a defect in thenail bed. It can be seen in cirrhosis, congenital heart failure and diabetes96) A patient with a history of gastrointestinal polyposis presents with alopecia, generalizedpigmentation, and nail dystrophy. Which of the following is the most likely diagnosis?A. Cronkhite-Canada Syndrome Correct ChoiceB. Cowden syndromeC. Peutz-Jegher syndromeD. Dyskeratosis congenitaE. Gardner syndromeCronkhite-Canada Syndrome is a sporadic gastrointestinal polyposis syndrome characterized byalopecia, generalized pigmentation, and nail dystrophy97) What is the most common hair finding in Nethertons syndrome?A. Trichorrhexis invaginataCorrect ChoiceB. Pili tortiC. MonilethrixD. Trichoschisis
  25. 25. E. Trichorrhexis nodosaNetherton syndrome is an an autosomal recessive genodermatosis that is caused by a mutation inSPINK5. The characteristic findings include trichorrhexis invaginata, ichthyosis linearis circumflexa,and food allergy. Infants may present with erythroderma98) Transverse white bands on one or two nails is caused by:A. trauma to the matrixCorrect ChoiceB. psoriasisC. systemic lupus erythematosisD. alopecia areataE. lichen planusLongitudinal leukonychia or transverse white bands are caused by trauma to the nail matrix. AA,psoriasis, SLE and LP all cause spotted red lunulae, not transverse white bands99) Mutations in c-kit are associated with which of the following conditions?A. Piebaldism Correct ChoiceB. Waardenburg syndromeC. Vogt-koyanagi-haradaD. Tuberous sclerosisE. Cornelia de Lange SyndromePiebaldism is caused by mutations in c-kit100) Which of the following medication is a cause of hirsutism without virilization?A. DiazoxideCorrect ChoiceB. DinitrochlorobenzeneC. DapsoneD. DiazepamE. DantrolenePhenytoin, diazoxide, cyclosporine and hexachlorobenzene all can cause increased hair growth inpatients. In addition, oral (and topical) minoxidil can cause hirsutism. The other listed medicationsdo not cause hirsutism101) What nutritional deficiency can lead to this nail finding?A. Vitamin B12B. ThiamineC. IronCorrect ChoiceD. MagnesiumE. Vitamin CIron deficiency can lead to spoon nail deformity or koilonychias
  26. 26. 102) A 42 year old woman presents with the complaint of excess hair growth on her face. She hasnormal menses and has recently had her "annual" exam and the note relates normal sized ovaries.What is the most logical next step?A. Refer her to endocrinologyB. Order a CT of the abdomenC. Send a 21-hydroxylase enzyme deficiency testD. Check plasma levels of androstenedione and testosteroneCorrect ChoiceE. Biopsy from the most affected areaWomen with idiopathic hirsutism will have evidence of androgen excess but with normal menses,normal-sized ovaries and no evidence of tumors of adrenal or ovary and normal adrenal function.They will often have slight elevations of plasma androstenedione and testosterone. Check the bloodlevels of the plasma steroids would be a logical first step103) Ferritin levels need to be, at minimum, above which of the serum levels to treat iron-deficiency related telogen effluvium?A. 30ng/dLB. 20ng/dLC. 60ng/dLD. 10ng/dLE. 40ng/dLCorrect ChoiceThe therapeutic target for iron deficiency related telogen effluvium is greater than 40mg/dL. Othercauses of telogen effluvium include thyroid dysfunction, as well as such medications as beta-blockers, antihyperlipidemic drugs, and NSAIDs104) Plummers nails are:A. defective keratinization on the proximal matrixB. caused by B12 deficiencyC. hereditary or from shoe traumaD. caused by onycholysis from hypothyroidismCorrect ChoiceE. caused by cirrhosisPlummers nails are caused by onycholysis from hypothyroidism. Pincer nails are hereditary or fromshow trauma, usually 1st toenails. Nail pitting is caused by defective keratinization on the proximalmatrix. B12 deficiency can cause nail pigmentation105) What determines the thickness of the nail plate?A. Nail plateB. HyponychiumC. Proximal nail foldD. Nail bed
  27. 27. E. Nail matrixCorrect ChoiceThe nail matrix determines the thickness of the nail plate106) A patient presents with 20 nails with absent cuticles and lunulae, slow growth, dystrophicshape and a yellow hue. Which of the following findings is/are associated?A. pleural effusionsCorrect ChoiceB. cirrhosisC. pulmonary fibrosisD. chest painE. upper extremity edemaYellow Nail syndrome has been associated with lower extremity edema, bronchiectasis and pleuraleffusions. The other listed findings are not part of Yellow Nail syndrome107) A rapid onset of hair growth with or without accompanying virilization can occur in which ofthe following conditions EXCEPT:A. Ovarian cystCorrect ChoiceB. Adrenal carcinomaC. ArrhenoblastomaD. Krukenburg tumor of the ovaryE. Adrenal adenomaAll of these options except an ovarian cyst can cause a rapid onset of hair growth with or withoutaccompanying virilization. An Arrhenoblastoma is a tumor of the ovary that secretes testosterone108) Deposition of mucin in the hair follicle can be associated with which disease?A. Keratosis Follicularis Spinulosa DecalvansB. Adams-Oliver SyndromeC. Mycosis fungoides Correct ChoiceD. Perifolliculitis Capitis Abscedens et SuffodiensE. Acne Keloidalis NuchaeMycosis fungoides can be associated with follicular mucinosis109) A 32 year-old woman presents with the complaint shown in the image. This finding began oneyear ago and has slowly worsened. Her female relatives do not have similar hair patterns. SerumDHEA returns at 8544 ng/ml and serum testosterone at 3 ng/ml. What is the most likely diagnosisfor cause of her hirsutism:A. Poryphria cutanea tardaB. Erythropoietic protoporphyriaC. An androgen producing tumorCorrect ChoiceD. Stein-Leventhal syndromeE. Acute intermittent porphyria
  28. 28. DHEA > 8000 ng/ml and testosterone > 2 ng/ml are suggestive of a secreting neoplasm. Porphyriacutanea tarda and Stein-Leventhal syndrome do present with hirsutism, but will not have theelevated levels of DHEA and testosterone in the serum. Acute intermittent porphyria andErythropoietic protoporphyria do not have hypertrichosis110) Plummers nails are associated with which of the following disorders?A. DermatomyositisB. Systemic sclerosisC. Lupus erythematosusD. Lead poisoningE. Hypothyroidism Correct ChoicePlummers nails are onycholysis due to hypothyroidism111) This hair finding is caused by mutations in what gene?A. SERCA2B. Keratin 16C. DyskerinD. NEMOE. SPINK 5Correct ChoiceMutations in SPINK 5, a serine protease inhibitor leads to Netherton’s syndrome which ischaracterized by trichorrhexis invaginata or bamboo hair112) Air spaces in the hair shaft lead to this condition pictured below – name the condition:A. Wooly hairB. TrichoschisisC. Pohl Pinkus constrictionD. Pili annulatiCorrect ChoiceE. Pili trianguli et canaliculiPili anulati is caused by air spaces in the hair shaft.113) Thin hair with premature graying is characteristic of which of the following syndromes?A. Cornelia de Lange SyndromeB. Noonan SyndromeC. Werner syndrome Correct ChoiceD. Waardenburgs syndromeE. Turner SyndromeExplanation114) Mutations in hair keratins hHB6 and hHB1 cause this characteristic finding pictured below –name the condition:
  29. 29. A. TrichoschisisB. TrichoptilosisC. MonilethrixCorrect ChoiceD. Hair castsE. Tiger-tail hairMutations in the hair keratins, hHB6 and hHB 1 cause monilethrix.115) A patient with psoriasis has pitting of the nails. This finding is due to involvement of whichpart of the nail unit?A. Distal matrixB. Proximal matrixCorrect ChoiceC. HyponychiumD. Proximal nail foldE. Nail bedDisease in the proximal matrix is responsible for producing pitting, onychorrhexis, and Beau’s lines.Changes in the intermediate matrix can cause leukonychia, and the distal matrix may beresponsible for focal onycholysis, thinned nail plate, and erythema of the lunula.116) Björnstad syndrome is the combination of pili torti and which of the following features?A. LentiginesB. Deafness Correct ChoiceC. HypogonadismD. White forelockE. SeizuresDeafness and pili torti are the two cardinal features of Björnstad syndrome