boards’ fodder Eponyms in Dermatology by Heather Kiraly Orkwis, DO. (Updated July 2015*) Aleppo/Baghdad/Delhi boil = Conradi-Hünermann syndrome Hutchinson’s 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 Crowe’s 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 Auspitz’s sign = punctate bleeding Darier disease = Keratosis follicu- endocarditis points within lesion upon scratching; laris (ATP2A2) seen in psoriasis Kasabach-Merritt syndrome = Darier’s 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 (Bazex–Du- tion of venous blood, seen in atopic port-wine stain, soft tissue and bony pré–Christol Syndrome), XLD = dermatitis hypertrophy, venous and lymphatic malformations follicular atrophoderma, multiple BCCs, Degos’ disease = malignant atrophic hypotrichosis, localized hypohidrosis papulosis Koplik’s spots = small, white spots on erythematous buccal mucosa, seen Bazin’s disease = erythema indura- Dercum disease = adiposis dolo- in early measles tum, associated with TB 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 Kyrle’s 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) Behcet’s disease = triad of apthous ulcers, genital ulcers, ocular inflamma- hypoplasia (PORCN) Leser-Trélat sign = abrupt onset of tion (+HLA-B51; Silk Road Disease) Gorlin syndrome = nevoid basal cell multiple seborrheic keratoses, associ- ated with internal malignancy Bloch-Sulzberger disease =iIn- carcinoma syndrome (patched-1) continentia Pigmenti (NEMO; X-linked Gottron’s papules = erythematous Lichtenberg’s figures = branching dominant) eruption over knuckles, elbows, knees, pattern of cutaneous marks pathogno- monic for lightning injury Bockhart’s impetigo = follicular seen in dermatomyositis impetigo Graham-Little-Piccardi-Lasseur Lofgren syndrome = erythema syndrome = variant of LPP: cicatricial nodosum, bihilar lymphadenopathy Bourneville’s 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- Bowen’s disease = squamous cell eruption giectasia mutated (ATM) gene carcinoma in situ Grover’s disease = transient acan- Lovibond’s 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) Lyell’s syndrome = toxic epidermal verrucous carcinoma of glans penis and necrolysis (TEN) prepuce (HPV 6, 11) Heck’s disease = oral focal epithelial hyperplasia (HPV 13, 32) Madelung’s disease = benign sym- Buschke-Ollendorff syndrome = metric lipomatosis (Launois-Bensuade dermatofibrosis lenticularis disseminata, Griscelli syndrome = pigmentary syndrome, horse-collar appearance) osteopoikilosis (LEMD3) dilution, T- and B-cell immunodeficiency, 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) Majocchi’s 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- non-transgradiens PPK, esophageal resident at St. Joseph (desmoplakin) matophyte infection of hair follicle Mercy Hospital in Ann carcinoma (TOC) Cobb syndrome = cutaneomeningo- Mal de Meleda = keratoderma pal- Arbor, Michigan. Hughes’ triad = antiphosholipid an- spinal angiomatosis moplantaris transgrediens (SLURP1) 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) irectionsin D Residency p. 1 • Fall 2012 boards’ fodder Eponyms in Dermatology by Heather Kiraly Orkwis, DO. (Updated July 2015*) Marjolin’s ulcer = aggressive SCC Ollendorf’s sign = secondary Shulman’s 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 = Sjögren-Larsson syndrome = Albright syndrome; “Coast of Maine” Osler-Weber-Rendu syndrome = ichthyosis with erythroderma, spastic café-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) Milroy’s disease = congenital lower Papillon-Lefèvre syndrome = pal- limb lymphedema (FLT4) moplantar keratoderma with periodonto- Sneddon’s 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- Ritter’s 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) Russell’s 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) Schnitzler’s syndrome = nonpru- Well’s 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 Nikolsky’s 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. irectionsin www.aad.org/DIR Fall 2012 • p. 2 D Residency.