title: Paraneoplastic Dermatomyositis with Cutaneous and Myopathic Disease Responsive to Adrenocorticotropic Hormone Therapy.
abstract: Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy. The authors present a case report of paraneoplastic dermatomyositis associated with triple negative, BRCA-1 positive, invasive intraductal carcinoma of the breast, whose myopathic and cuteanous symptoms were recalcitrant to high-dose corticosteroid therapy. Herein, the authors describe the first reported case of the use of an injectable adrenocorticotropic hormone agonist gel in a patient with myopathic paraneoplastic disease that achieved clinical resolution of both myopathic and cutaneous symptoms, but subseuqently developed significant hyperpigmentation of her face suspected to be secondary to a chemotherapeutic-induced pigmentary change which was augmented by adrenocorticotropic hormone therapy.
authors.affiliation: St. Barnabas Hospital Dermatology Residency, Bronx, New York.
authors.forename: Marisa
authors.initials: M
authors.lastname: Wolff
authors.affiliation: St. Barnabas Hospital Dermatology Residency, Bronx, New York.
authors.forename: Christopher
authors.initials: C
authors.lastname: Mancuso
authors.affiliation: New York College of Osteopathic Medicine-NYIT, Old Westbury, New York.
authors.forename: Karan
authors.initials: K
authors.lastname: Lal
authors.affiliation: Pathology Associate Pc, Dermatopathology, Portchester, New York.
authors.forename: Damian
authors.initials: D
authors.lastname: Dicostanzo
authors.affiliation: St. Barnabas Hospital Dermatology Residency, Bronx, New York;; Mount Sinai School of Medicine Manhattan, New York.
authors.forename: Charles
authors.initials: C
authors.lastname: Gropper