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2015 ; 2
(1
): 1-8
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English Wikipedia
Wong-Type Dermatomyositis Showing Porokeratosis-Like Changes (Columnar
Dyskeratosis): A Case Report and Review of the Literature
#MMPMID27047930
Umanoff N
; Fisher A
; Carlson JA
Dermatopathology (Basel)
2015[Jan]; 2
(1
): 1-8
PMID27047930
show ga
BACKGROUND: Wong-type dermatomyositis (DM) exhibits simultaneous pityriasis rubra
pilaris (PRP) features. CASE REPORT: A 50-year-old woman presented with a
heliotrope rash, Gottron's papules, and a poikilodermic, erythematous rash in
shawl distribution without evidence of muscle weakness. Despite topical
corticosteroids, the eruption progressed 9 months later to include generalized
hyperkeratotic follicular papules, islands of sparing, and atrophic macules with
a collarette of scale suggestive of porokeratosis. Mild dysphonia was the only
sign of muscle weakness. Serology showed positive ANA. Histopathology revealed
interface dermatitis with dermal mucin and melanophages, irregular psoriasiform
hyperplasia, alternating mounds of para- and orthokeratosis, and tiers of
dyskeratotic cells (columnar dyskeratosis). Systemic corticosteroid therapy was
not tolerated; acitretin diminished the hyperkeratosis. While hyperpigmentation
persisted, no progression of cutaneous or muscular symptoms has occurred after 22
months of follow-up and cessation of the therapy. Overall, her course did not
differ from the natural history documented in the literature review of Wong-type
DM. The most similar case also exhibited pseudocornoid lamella changes.
CONCLUSION: Wong-type DM is a clinicopathologic DM-PRP hybrid that can also
exhibit porokeratosis-like features best described as columnar dyskeratosis.
Recognizing these types of lesions in DM is warranted in order to make an
accurate assessment of their prognostic significance.