Clinically amyopathic dermatomyositis (CADM) is a rare entity that presents with cutaneous manifestations of classic dermatomyositis but without muscle weakness or abnormal muscle enzymes. It is more common in young white and Asian females. A subset of patients with CADM has a specific antibody known as anti-MDA5. These patients have a more aggressive course with distinct cutaneous features, pulmonary involvement and early death. Here, we present the case of a 64-year-old Caucasian male with no significant medical history who was admitted with marked weight loss and a painful rash for 6 months. Patient had no muscle weakness and his rash was characteristic of classic dermatomyositis. Skin biopsy was suggestive of dermatomyositis but muscle enzymes were normal. His serum was positive for anti-MDA5 antibody. Extensive workup failed to detect any malignancy but he did show non-specific interstitial pneumonia. He was treated with prednisone and mycophenolate with good clinical response.
SUMMARY
Cutaneous T-cell post-transplant lymphoproliferative disorder (PTLD) is a rare clinical presentation that can potentially turn aggressive in solid-organ transplant recipients if not detected and intervened on early. We encountered a rare case of rapidly worsening primary cutaneous CD30-positive, Epstein-Barr virus-negative anaplastic large cell lymphoma (ALCL) of T-cell origin, manifesting as an isolated nasal tip lesion in a 71-year-old man 4 years after orthotopic liver transplantation. Excisional biopsy with partial rhinectomy showed subepithelial diffuse infiltration of medium-to-large lymphoid cells having round-to-irregular nuclei, partially condensed chromatin and prominent nucleoli. Immunophenotypic studies revealed CD30-positive primary cutaneous ALCL. Positron emission tomography/CT imaging revealed a locally active disease, and radiation therapy was initiated with complete response. A high index of suspicion for PTLD when evaluating skin lesions in a post-transplant patient is paramount for its early recognition, prompt diagnosis and timely intervention while the window for curative therapy remains possible.
A 56-year-old Hispanic woman presented to the Emergency Department with a syncopal episode. Her medical history was significant for primary biliary cholangitis (stage I-II), breast cancer status post lumpectomy and chemoradiation, gastroesophageal reflux disease, and multiple episodes of gastrointestinal bleeding. Her vital signs on admission were normal. On physical examination, the patient had regular heart rate and rhythm, pale conjunctivae with icteric sclera, dry mucosal membrane, mild epigastric abdominal tenderness, firm liver without hepatosplenomegaly, scattered telangiectasia on chest and abdomen, and sclerodactyly on both hands up to the metacarpophalangeal joints. A digital rectal examination was positive for melena.
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