Clinically amyopathic dermatomyositis associated with a metastatic undifferentiated carcinoma

Authors: Felix Paolo J. Lizarondo, MD1, Eileen Liesl A. Cubillan, MD, FPDS

Abstract

Introduction: Clinically amyopathic dermatomyositis (CADM) presents with the characteristic cutaneous findings of dermatomyositis (DM) but without any clinical evidence of muscle weakness. There is an established association of malignancy in adult-onset DM, although patients with CADM may have a decreased risk of having an associated malignancy compared to classic adult onset DM.

Case Summary: A 40-year old Filipino male was diagnosed with of CADM after presenting with pruritic photodistributed symmetric erythematous-violaceous patches associated with nail fold telangiectasias and the pathognomonic Gottron papules without clinical evidence of muscle weakness. Histological examination of a plaque on the knuckle and a macule on the chest both revealed interface dermatitis with increased mucin deposition in the reticular dermis consistent with dermatomyositis. Muscle enzyme labs (creatine kinase- total, creatine kinase-MB) and liver enzyme tests (AST, ALT) were not suggestive of myositis. Immunologic work-up was negative for anti-nuclear antibodies-immunofluorescence (ANA-IF) and anti-Jo. The patient shortly presented with multiple cervical lymphadenopathies, which was shown to be a metastatic undifferentiated carcinoma. Screening laboratories done for occult malignancy were within normal limits including serum prostate specific antigen (PSA), complete blood count, urinalysis and stool occult blood testing. Nasopharyngeal examination was normal.

Conclusion: This case highlights the role of a dermatologist in diagnosing CADM, an uncommon variant of DM. Second, this case presented as a metastatic undifferentiated carcinoma presenting as cervical lymphadenopathies, which is also a rare presentation of cancer in patients with DM

 

Citation

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Keywords

dermatomyositis, clinically amyopathic dermatomyositis, metastatic undifferentiated carcinoma, carcinoma

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