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Treatment challenges in clinically amyopathic dermatomyositis: A case series and review of new therapeutic options for skin involvement
Author(s) -
RodríguezTejero Andrea,
LópezEspadafor Beatriz,
MonteroVílchez Trinidad,
SánchezDíaz Manuel,
AriasSantiago Salvador,
MolinaLeyva Alejandro
Publication year - 2021
Publication title -
dermatologic therapy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.595
H-Index - 68
eISSN - 1529-8019
pISSN - 1396-0296
DOI - 10.1111/dth.14942
Subject(s) - medicine , dermatomyositis , myositis , interstitial lung disease , dermatology , malignancy , disease , connective tissue disease , pathology , autoimmune disease , lung
The term clinically amyopathic dermatomyositis (CADM) is used to represent a subgroup of patients with the typical cutaneous features of dermatomyositis (DM) in the absence of muscle involvement. Similar to classic DM, CADM can be associated with other connective tissue disorders and systemic manifestations such as interstitial lung disease and malignancy. Owing to the frequent discordance between muscle response and skin disease, the therapeutic approach to CADM represents a challenge. The current literature suggests that CADM treatment should follow a specific protocol, influenced by visceral involvement and the expression of certain myositis‐specific antibodies, and different from the recommendation in the presence of myositis. Here, we present five new cases of CADM. We describe the available therapeutic options for skin manifestations in this type of DM, and we propose a step‐by‐step therapeutic scheme, using the cutaneous dermatomyositis disease area and severity index to assess response. Our literature review establishes mycophenolate mofetil and intravenous immunoglobulin as the most frequently successful therapies in refractory skin disease.