morphea

Question 1

what are the subtypes?

Answer 1

plaque (most common adult)
linear (most common Peds) 
generalized
bullous
deep
nodular 
morphea-LSA combo
profunda
guttate
variants (coup de sabre, parry-romberg, atrophoderma of passini and pierini, linear atrophoderma of moulin)

Question 2

plaque morphea

Answer 2

erythematous to violaceous patches on trunk and extremities –> hyperpigmented or ivory plaques (hairless, anhidrotic) +/- lilac-violaceous inflammatory rim (sign of persistent disease)

Question 3

linear morphea

Answer 3

similar to plaque morphea but linear distribution, along blaschko’s lines; a/w poor morbidity ( + contractures, permanent undergrowth of limbs)

Question 4

epidemiology

Answer 4

most cases present in childhood.

Question 5

pathogenesis

Answer 5

genes, trauma, radiation, medications, borrelia infx

Question 6

who should we refer pediatric patients with head/neck morphea to ?

Answer 6

ophthalmology

Question 7

what are the associations with parry-romberg syndrome (progressive hemifacial atrophy)?

Answer 7

a/w epilepsy, exophthalmos, headache, trigeminal neuralgia, myopathy of eye muscles, cerebral atrophy, white matter hyper intensity, or alopecia

Question 8

generalized morphea

Answer 8

expansive, involves trunk&limbs, a/w muscle atrophy and difficulty breathing

Question 9

deep morphea

Answer 9

resides in subq, poor response to steroids; may develop osteoma cutis; complications: deformity, ulcers, SCC, contractures,

Question 10

what is the target autoAb

Answer 10

anti-topoisomerase II

*unlike systemic scleroderma which is Scl-70/topoisomerase I

Question 11

where do we see melorheostosis?

Answer 11

in linear morphea; this is roughening of long bone surface underlying area of linear morphea, on XRAY looks like wax dripping down side of candle

Question 12

TX

Answer 12

mild: topical CS/CI, phototherapy (will only penetrate to dermis, so not beneficial for deeper lesions)
moderate-severe disease: MTX + prednisone