stigmata Flashcards
DMS- cutaneous
- heliotrope, V shaped photosensitive poikiloderma, shawl sign,
- holster, flagellate, photodistributed erythema,calcinosis cutis
- hands: gottrons sign/papules( over IPJ vs LE ), nailfold telangiectasia/ragged cuticle, mechanic’s hands,Raynaud’s
- scalp poikiloderma/alopecia
ClassX: DMS
bullous ,amyopathic(26%), antisynthetase S
juvenile
antisynthetase S features, prevalence and significance?
30%,
raynaud’s, mechanics hands, ILD,
anti Jo 1 ab
juvenile DMS
calcinosis cutis, arthritis, RBBB, conduction)
DMS systemic
- myositis/Esophageal/ILD/carditis;
- malignancy 30% ovarian worldwide, NPC locally(50% of 20%), (LN,epistaxis, cranial nerve palsy, abdo masses/ascites, breast pelvic PR exam/LOW/BH)
- overlap syndrome: SLe, scleroderma,MCTD, RA/Sjogrens
scleroderma cutaneous
ivory waxy lilac indurated, salt pepper dyspigmentation,
face- limited mouth opening furrows
hand- edema,CREST Calcinosis Raynauds digital infarct; nailfold capillaries Sclerodactylyl(pray)
scleroderma systemic
- muscle motility: dysphagia, proximal myopathy,
- BP( renal crisis), ILD-dry cough, fine creps /pulmonary HT( exertional dysnpea, JVP)
- overlap
Overlap syndromes/MCTD- kusukawa criteria
(SLE, DMS) raynauds, finger/hand swelling/acral sclerosis+U1 RNP, antifibrillin, antiPMScl + 1 feature
morphea classX
Cx: plaque/guttate/generalised(>2 sites); bullous, linear, deep
post strep scleredema
(acute cervicofacial hardening ,mask facies, limited mouth opening/swallowing)
scleredema monoclonal gammopathy,
persisitent;
scleredema diabeticorum
peau d orange erythema induration, waxy distal extremities
papular mucinosis-stigmata , subtypes
clusters/linear/close spaced; flesh/waxy papules
discrete papular/acral,HIV; self healing, nodular,
+ sclerodermoid - scleromyxedema
GVHD ClassX
traditional : 100d post SCT
NIH- organ specific criteria, diagnostic skin *
aGVHD:
Fx: morbilliform, follicular, petechial, acral, (st 4)TEN like 4-6w post HSCT;
staging: BSA, GIT/liver, histo
cGVHD
Fx: 1)LP like: reticulate pink-violet scaly papules plaques/mucosal/nail(dorsal pterygium, anocyhia)
2) papulosquamous psoriasiform/KP follicular erythema/SCLE
3) poikiloderma 4) sclerodermoid (patchy, diffuse symmetric); EF- rippled irregular nodular , groove sign
LSA Fx+ cX?
- Atrophic ivory white wrinkled , obliterated labia majora minora introitus/meatus, cannot retract foreskin, hemorrhage, erosive; lilac margin(Extragenital)
- irreversible scarring/stenosis; malignancy(non healing ulcers fissures nodules)
nail LP
longtudinal ridging, depressions, pterygium subungual hyperkeratosis
Pancreatic panniculitis-
ulcerated oily discharging nodule; fever abdo pain
Schmids triad- subcut nodules, polyarthritis(lytic) eosinophilia
SPTL
recurrent subcutaneous lesions fever, night sweats, fatigue and weight loss,
Cx: Ulceration, haemophagocytic syndrome(pancytopenia, prolonged fever, aggressive)
PRP CLASSx
Subtypes: 1)classical adult, 50%, cephalocaudal, orange red islands sparing, follicular hyperkeratosis, remit 3 y 2)atypical,icthyosiform chronic 3) classic juvenile 10% , 1 y remission
4) 25% circumscribed uncertain prognosis,
5) atypical juvenile, follicular hyperkeratosis familial CARD14, 5%
6) HIV associated( +follicular occlusion triad, lichen spinulosus like,HAART, poor prognosis)
palmar pits:
palmar pits:circular few mm, perpendicular edges; Approach: 1-; 2- keratoses detachment
PPK
calloused palms,soles,fingertips, hypothenar,thenar eminences ;plantar fissuring, thickening weight bearing areas, palmar pits?
PC CLASSx
Focal +associated :Pachyonychia congenita type 1: Jadassohn–Lewandowsky type;2- Jackson–Lawler type; new:K6a/b/c/16/17
EB pruriginosa
: prurigo like,violaceous linear scarring,trauma blistering, milia,nail dystrophy
hypertrophic LP
(symmetric dorsal hyperkeratotic SCC)
perforating GA generalised-
DM, flesh-red colored grouped, central crust/umbilicated
lichen macular amyloid MEN2a-
sipple ripples,AD RET thyroid medullary ca, phaechromo
anetoderma-
circumscribed areas 1-2cm:flaccid, depressions, wrinkling, sac-like protrusions, skin-white blue, buttonhole sign, chest/back/neck/upper extremities
Degos-
porcelain white atrophic papules+central necrosis, erythema/telangiectasia, thrombotic vasculopathy:
EED,
Extensors myeloma, IgA monoclonal gammopathy , MDS, HIV
cowden’s
10queing COWs in a PtEN MOOing(trichilemomma) on COBBLES(tone) on TOES(acral keratoses, pits) - hyperactive(thyroid), Breastfeeding(adenomas), diarrhea(GIpolyp) menstruating(endometrium)
TS
(AD, TSC1/2, Ashleaf, CALM,shagreen(CTN),angiofibromas/collagenomas-Fibroma-gingival/subungual , EPilepsy, Low Intelligence, Angiofibroma( renal angiomyolipoma, pulm lymphangioleiomatosis, cardiac rhabdomyoma)
Muirr torre syndrome
(AD MLH1/MSH2 DNA mismatch repair, MSI; colon/GU/lung.breast CA)