A Rare Lymphoma in a Patient with Amyopathic Dermatomyositis

Abstract

A 37-year-old Caucasian woman was evaluated for a photosensitive dermatitis. A positive anti-nuclear antibody with a titer of 1 : 1280 and a speckled pattern was noted and she was diagnosed with subacute cutaneous lupus erythematosus (SCLE). Although the initial dermatologic diagnosis was SCLE, a skin biopsy suggested the additional possibility of dermatomyositis because of increased dermal mucin. We began following her at the request of the dermatology department, and a diagnosis of amyopathic dermatomyositis was made based on the lack of objective muscle weakness, normal muscle enzymes, negative double-stranded DNA, SSA/SSB, and RNP/Smith antibody panel, and especially on the cutaneous examination findings. A malignancy evaluation included a normal CT scan of her chest, abdomen, and pelvis, esophagogastroduodenoscopy, colonoscopy, mammography, pelvic ultrasound, Papanicolaou smear, and endometrial biopsy. She developed vaginal bleeding 1 year after the onset of her skin manifestations. Repeat gynecologic evaluation, including cervical biopsy, revealed a large B-cell cervical lymphoma. Amyopathic dermatomyositis and lymphoma of the cervix are both rare conditions. Our case emphasizes the importance of considering underlying malignancy at the time of diagnosis and while the patient is followed clinically. Additionally, the clinician must remain vigilant in evaluating any new clinical changes in follow-up care. To our knowledge, this is the first documented case of lymphoma of the cervix in the setting of amyopathic dermatomyositis.

Treatment of clinically amyopathic dermatomyositis in adults: a systematic review

“…Thirty‐one patients used oral methotrexate at some point in their treatment . Eleven patients (35%) did not improve and seven (23%) had partial improvement.…”

“…In total, 84 patients received one or more antimalarial agents at some stage of their treatment . Hydroxychloroquine 200 mg twice daily was the most common antimalarial treatment.…”

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

“…Malignancies occur in 13–42% of patients with dermatomyositis, with the highest risk during the first and second year after diagnosis [ 213 ]. Although most of the cases of malignancy-associated dermatomyositis occur in patients with solid tumours, both classic and amyopathic dermatomyositis have been shown to rarely occur in patients with HMs, including non-Hodgkin’s lymphomas, Hodgkin’s disease, myeloid leukaemia and myelodysplastic syndromes [ 214 – 217 ]. The presence of erythroderma in dermatomyositis is a clinical hallmark of malignancy, especially of lymphoid origin [ 213 ].…”

18F-FDG PET Demonstration of Cancer Recurrence Presenting as Dermatomyositis in a Rare Case of Primary Pleural Lymphoma

“…Malignant tumors originating from lung, breast, ovary, and gastrointestinal track are known to be frequently associated with dermatomyositis [2][3][4]. However, lymphoma or other hematologic malignancies rarely cause dermatomyositis [6,7]. Moreover, primary pleural lymphoma, as in this case, is an extremely rare malignancy [8,9], and no case associated with dermatomyositis has been reported till date.…”