Morphea

Question 1

What percentage of patients with morphea have positive anti-topoisomerase II antibodies?

Answer 1

75% overall, 85%-90% in generalized morphea

Question 2

Which forms of morphea are more likely to be ANA positive?

Answer 2

Linear and generalized morphea

Question 3

What antibodies are often positive in linear and generalized morphea?

Answer 3

anti-ssDNA and anti-histone antibodies

These antibodies correlate with disease severity and activity.

Question 4

What type of infiltrate is observed early in histopathology in morphoea?

Answer 4

Lymphocytic infiltrate with plasma cells at dermal-SQ junction

Question 5

What is lost in the early phase of histopathology in morphea?

Answer 5

CD34+ dendritic cells

Question 6

What differentiates the loss of CD34+ dendritic cells in histopathology in morphoea from nephrogenic systemic fibrosis (NSF) and scleromyxedema?

Answer 6

In NSF and scleromyxedema, CD34+ dendritic cells are still present

Question 7

What are histopath signs in late stage morphea?

Answer 7

Decreased inflammation
■ “Square biopsy” sign
■ Pale, edematous, and homogenized papillary dermis
■ Loss of pilosebaceous units/periadnexal fat
■ “Trapped eccrine glands” = eccrine glands/ducts compressed by surrounding sclerotic/keloidal collagen

Question 8

Bloods for
Morphea?

Answer 8

Crp/esr - can be elevated
ANA - positive in linear and generalised, negative in plaque.
Antihistone and anti ssDNA - positive in linear and generalised, sign of disease severity/activity

Absent in all morphea:
anti-Scl70 (Topo I) and anti-centromere antibodies (positive in systemic sclerosus)