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Eponyms in Dermatology
by Heather Kiraly Orkwis, DO. (Updated July 2015*)

Aleppo/Baghdad/Delhi boil = Conradi-Hnermann syndrome Hutchinsons sign = pigmenta-

lesion of cutaneous leishmaniasis = XLD chondrodysplasia punctata (EBP tion of proximal nail fold, suggestive of
gene) melanoma
Asboe-Hansen sign = extension of
intact blister when pressure is applied to Crowes sign = axillary or inguinal Janeway lesions = non-painful
roof; seen in pemphigus vulgaris freckling seen in neurofibromatosis hemorrhagic macules or nodules of
palms and soles, seen in infective
Auspitzs sign = punctate bleeding Darier disease = Keratosis follicu- endocarditis
points within lesion upon scratching; laris (ATP2A2)
seen in psoriasis Kasabach-Merritt syndrome =
Dariers sign = urtication following consumptive coagulopathy within a
Bateman purpura = actinic (solar) rubbing of macule/papule in mastocyto- kaposiform hemangioendothelioma or
purpura sis (urticaria pigmentosa) tufted angioma
Bazex syndrome, acquired = Dennie-Morgan lines = crescentic Klippel-Trenaunay syndrome
acrokeratosis paraneoplastica creases of lower eyelids due to stagna- = angio-osteohypertrophy syndrome;
Bazex syndrome (BazexDu- tion of venous blood, seen in atopic port-wine stain, soft tissue and bony
prChristol Syndrome), XLD = dermatitis hypertrophy, venous and lymphatic
follicular atrophoderma, multiple BCCs, malformations
Degos disease = malignant atrophic
hypotrichosis, localized hypohidrosis papulosis Kopliks spots = small, white spots
Bazins disease = erythema indura- on erythematous buccal mucosa, seen
Dercum disease = adiposis dolo-
tum, associated with TB in early measles
rosa; mostly obese menopausal women,
Becker nevus/melanosis = large, consisting of multiple exquisitely tender Kveim-Sitzbach test = skin test
unilateral hyperpigmented patch, with lipomas with human sarcoid tissue injected into a
secondary hypetrichosis often hairy on patient suspected of having sarcoidosis;
Favre-Racouchot = open comedo-
upper extremity or chest positive results are a sarcoid granuloma
nes and solar elastosis
at the site
Beckwith-Wiedemann syndrome
Gianotti-Crosti syndrome = papu-
= exomphalos-macroglossia-gigantism Kyrles disease = chronic general-
lar acrodermatitis of childhood
syndrome (p57/KIP2) ized dermatosis with papules with central
Goltz syndrome = focal dermal keratotic plugs (DM, renal disease)
Behcets disease = triad of apthous
ulcers, genital ulcers, ocular inflamma- hypoplasia (PORCN) Leser-Trlat sign = abrupt onset of
tion (+HLA-B51; Silk Road Disease) Gorlin syndrome = nevoid basal cell multiple seborrheic keratoses, associ-
carcinoma syndrome (patched-1) ated with internal malignancy
Bloch-Sulzberger disease =iIn-
continentia Pigmenti (NEMO; X-linked Gottrons papules = erythematous Lichtenbergs figures = branching
dominant) eruption over knuckles, elbows, knees, pattern of cutaneous marks pathogno-
seen in dermatomyositis monic for lightning injury
Bockharts impetigo = follicular
impetigo Graham-Little-Piccardi-Lasseur Lofgren syndrome = erythema
syndrome = variant of LPP: cicatricial nodosum, bihilar lymphadenopathy
Bournevilles disease = Tuberous (sarcoidosis)
Sclerosis (Epiloia) (TSC1, TSC2) alopecia of scalp, non-scarring alopecia
of axilla and groin, follicular lichen planus Louis-Bar syndrome = ataxia telan-
Bowens disease = squamous cell eruption giectasia mutated (ATM) gene
carcinoma in situ
Grovers disease = transient acan- Lovibonds angle = 160 angle
Buruli ulcer = M. ulcerans (named tholytic dermatosis between proximal nail fold and the nail
after Buruli region of Nile River, Africa)
Hailey-Hailey disease = familial plate
Buschke-Lowenstein tumor = benign pemphigus (ATP2C1) Lyells syndrome = toxic epidermal
verrucous carcinoma of glans penis and
Hecks disease = oral focal epithelial necrolysis (TEN)
prepuce (HPV 6, 11)
hyperplasia (HPV 13, 32) Madelungs disease = benign sym-
Buschke-Ollendorff syndrome =
Griscelli syndrome = pigmentary metric lipomatosis (Launois-Bensuade
dermatofibrosis lenticularis disseminata,
dilution, T- and B-cell immunodeficiency, syndrome, horse-collar appearance)
osteopoikilosis (LEMD3)
recurrent infection, progressive CNS Maffucci syndrome = superficial
Calabar swellings = localized an- deterioration (myosin VA)
gioedema in tissue from migrating loiasis and deep venous malformations, en-
chondromas, chondrosarcoma (PTHR1)
Hermansky-Pudlak syndrome
Carney Complex = NAME syn- = pigment dilution, bleeding diathesis,
drome, LAMB syndrome (PRKAR1A) Majocchis disease = purpura an-
Heather Orkwis, DO, is lysosomal membrane defect (HPS1) nularis telangiectoides
a PGY4 dermatology Carvajal syndrome = left sided car- Howel-Evans syndrome =
diomyopathy, woolly hair, keratoderma Majocchi granuloma = deep der-
resident at St. Joseph non-transgradiens PPK, esophageal matophyte infection of hair follicle
(desmoplakin)
Mercy Hospital in Ann carcinoma (TOC)
Cobb syndrome = cutaneomeningo- Mal de Meleda = keratoderma pal-
Arbor, Michigan. Hughes triad = antiphosholipid an- moplantaris transgrediens (SLURP1)
spinal angiomatosis
tibody syndrome (fetal loss, thrombosis,
Civatte bodies = degenerated, thrombocytopenia) Marfan syndrome = tall stature,
apoptotic keratinocytes seen in lichen arachno-dactyly, ectopia lentis, progres-
planus Hutchinson-Gilford syndrome = sive aneurysmal dilation of ascending
progeria (lamin A) aorta, CHF (fibrillin 1)
DRirections
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 boards fodder
Eponyms in Dermatology
by Heather Kiraly Orkwis, DO. (Updated July 2015*)

Marjolins ulcer = aggressive SCC Ollendorfs sign = secondary Shulmans syndrome = eosino-
arising in site of chronic injury or burn syphilis papule tender to touch with blunt philic fasciitis (dry river bed)
probe
McCune-Albright Syndrome = Sjgren-Larsson syndrome =
Albright syndrome; Coast of Maine Osler-Weber-Rendu syndrome = ichthyosis with erythroderma, spastic
caf-au-lait macule(s), polyostotic fibrous Hereditary Hemorrhagic Telangiectasia di-tetraplegia with scissor gait, mental
dysplasia, precocious puberty (GNAS1) syndrome (HHT1, HHT2) retardation, atypical retinitis pigmentosa
(FALDH)
Milroys disease = congenital lower Papillon-Lefvre syndrome = pal-
limb lymphedema (FLT4) moplantar keratoderma with periodonto- Sneddons syndrome = livedo
sis (cathepsin C) reticularis, HTN, CVA associated with
Montgomery syndrome = xan- antiphospholipid antibodies
thoma disseminatum Parry-Romberg syndrome =
acquired progressive hemifacial atrophy Sturge-Weber syndrome =enceph-
Mucha Habermann disease = (morphea variant) alotrigeminal angiomatosis
pityriasis lichenoides et varioliformis
acuta (PLEVA) Refsum syndrome = phytanic acid Tyndall effect = blue tinging of
storage disease (PAHX, PEX7) subcutaneous lesions due to short wave-
Muckle-Wells syndrome = length colors (blue/violet) scattering
recurrent fevers and urticaria, progres- Richner-Hanhart syndrome =
sive deafness, secondary amyloidosis Tyrosinemia type II (tyrosinase amino- Urbach-Wiethe disease = lipoid
(cryopyrin) transferase) proteinosis (ECM1)
Muir-Torre syndrome = DNA mis- Ritters disease = staphylococcal Vohwinkel syndrome = PPK mu-
match repair defect, sebaceous tumors, scalded skin syndrome tilans, keratoderma hereditaria mutilans
adenocarci-noma of the colon (MLH1, (connexin 26, loricrin)
MSH2) Russells sign = dorsal hand with
dry skin and calluses, seen with bulimia/ Von Recklinghausen disease =
Naxos disease = right sided cardio- purging neurofibromatosis I (neurofibromin)
myopathy, woolly hair, non-epidermolytic
PPK (plakoglobin) Schnitzlers syndrome = nonpru- Wells syndrome = eosinophilic
ritic urticaria, arthralgias, IgM monoclonal cellulitis, flame figures on dermatopa-
Netherton syndrome = ichthyosis protein thology
linearis circumflexa (SPINK5)
Senear-Usher syndrome = Zinsser-Engman-Cole syndrome
Nikolskys sign = normal epidermis pemphigus erythematosus; variant of P. = Dyskeratosis Congenita (dyskerin)
easily separated when pressed firmly foliaceous confined to seborrheic sites
with a sliding motion, seen in pemphigus
vulgaris, staphylococcal scalded skin Sezary syndrome = generalized
syndrome exfoliative erythroderma (part of CTCL)

References
1. Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology. 3rd ed. China: Elsevier publishing; 2012.
2. Spitz JL. Genodermatoses: A clinical guide to genetic skin disorders. Philadelphia, Pa: Lippincott Williams & Wilkins; 2005.

*Reviewed and updated July 2015 by: Alina Goldenberg, MD, Emily deGolian, MD, Elise Herro, MD, and
Sharon Jacob, MD.

www.aad.org/DIR Fall 2012 p. 2 DRirections

in
esidency