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Feb 2012

Cerebriform Hand Dermatitis and Facial Papules —Diagnosis

Author Affiliations
 

SECTION EDITOR: MARY S. STONE, MD; ASSISTANT SECTION EDITORS: SOON BAHRAMI, MD; CARRIE ANN R. CUSACK, MD; SENAIT W. DYSON, MD; MOLLY A. HINSHAW, MD; ARNI K. KRISTJANSSON, MD

Arch Dermatol. 2012;148(2):247-252. doi:10.1001/archderm.148.2.247-h

The punch biopsy specimen showed extensive perivascular deposition of pale pink material, which was highlighted by Congo red stain. Apple green birefringence was demonstrated with polarized light microscopy (Figure 3). There were no osteolytic lesions on skeletal survey. A bone marrow biopsy specimen revealed hypercellularity, plasma cell infiltration, and nodular deposits of pink material, which was strongly positive for Congo red stain. Echocardiogram findings included an ejection fraction of 25% (reference range, 50%-70%) and a restrictive filling pattern, consistent with cardiac amyloidosis. The pathology report on the laryngeal nodule excision was reviewed, and amyloid was noted in the microscopic description. The patient was diagnosed as having primary systemic amyloidosis with cutaneous, cardiac, and laryngeal involvement. He was treated with bortezomib, lenalidomide, and dexamethasone and successfully underwent melphalan conditioning and autologous stem cell transplantation 8 months after presentation. His cutaneous findings showed marked improvement, and his cardiac ejection fraction was significantly improved to 40%.

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