Cutaneous T-cell lymphoma mimicking myopathy with lipoatrophy.

ABSTRACT

Mycosis fungoides (MF) is the most common form of primary cutaneous T-cell lymphoma. Classic MF usually follows a rather benign course over many years or decades, rarely ever leading to fatal extracutaneous organ involvement. Single cases of muscular involvement have been reported. Here we describe a 42-year-old male patient with hair loss and lipoatrophy since six months diagnosed as follicular MF and with a two months history of progressive distal leg weakness. Muscle biopsy and whole body muscle MRI showed an extensive muscular and subcutaneous fatty tissue infiltration. After therapy with topical steroids and acitretin/PUVA, systemic chemotherapy (CHOP) was initiated. The patient suffered from a rapid disease progression with fatal outcome 2.5 years after the first skin lesions, displaying progressive cachexia, muscular atrophy and weakness with scapuloperoneal distribution and cardiac dysfunction. So far, extensive muscular involvement by MF mimicking a distinct muscular phenotype has not been reported.