We present a case of a 68-year-old white female who presented with the complaint of fatigue and hair loss for several months. She had a past medical history of benign essential hypertension, bradycardia s/p (status post) cardiac pacemaker secondary to Mobitz type 2 atrioventricular block, hyperlipidemia, essential tremor, eczema, and osteopenia. An evaluation by dermatology had confirmed a diagnosis of alopecia totalis. This progressed to complete hair loss over the next few months. She was referred to nephrology for worsening creatinine (0.9-1.9 mg/dL over the course of 1 year) and proteinuria. On physical examination, she exhibited normal vital signs. Skin examination revealed complete hair loss of the body including the scalp (), eyebrows, eyelashes, and axillary () and pubic hair. Cardiac and lung examinations were unremarkable although she was noted to have trace of lower extremity edema.\nUrinalysis disclosed significant albuminuria, albumin/creatinine ratio of 8 g/g of creatinine, and urine protein/creatinine ratio of 9.8. Serum albumin was low at 3 g/dL. Urine microscopic examination was bland. Serological workup including anti-Ro, anti-La, anti-RNP, anti-Sm, anti Scl-70, anti Jo-1, ANA, anti–double stranded DNA, ANCA screen, C3, C4, hepatitis profile, HIV screen, cryoglobulins, and rheumatoid factor were all negative. Serum and urine protein electrophoresis revealed no paraproteins. She was noted to be anemic with hemoglobin of 12 g/dL. Given declining renal function, significant proteinuria, and a negative workup, renal biopsy was pursued. This revealed eosinophilic material in the mesangium with apple-green birefringence on Congo-red stain. Deposition of fibrillary material was seen in electron microscopy (). Immunohistochemistry of the biopsy specimen confirmed the presence of amyloid, AL type (kappa). Serum-free light chain analysis demonstrated an elevated kappa/lambda ratio of 79.8; kappa concentration was 1014 mg/dL, while free lambda was 12.7 mg/dL. Bone marrow examination showed 28% plasma cells with atypia 46, XX; fluorescence in situ hybridization demonstrated monosomy-13 with gains in chromosomes 9 and 11.\nThe patient was initiated on bortezomib and dexamethasone treatment for amyloidosis associated with MM. She was hospitalized 1 month later for symptoms of heart failure and was found to have a reduced left ventricular ejection fraction of 25%. Cardiac dysfunction was presumed to be secondary to amyloidosis. Her renal failure continued to progress and required initiation of hemodialysis. She was switched to ixazomib because of severe adverse effects to bortezomib after 3 cycles. She had a very good partial response to chemotherapy with serum kappa light chain level decreasing from 1854 mg/dL to 165 mg/dL. Renal function failed to recover and she remained dialysis dependent. She experienced an improvement in alopecia with new hair growth following the initiation of chemotherapy coincident with a significant reduction in the kappa light chain levels. The favorable response to chemotherapy may be predictive of an improved overall prognosis in our patient.
A 65-year-old man presented with a 2-year history of progressive hair loss involving the scalp, the eyebrows and the upper trunk, and recent onset of small papules and blisters on the dorsal hands as well as longitudinal ridging and fissuring of the fingernails. Clinical examination showed diffuse alopecia most pronounced on the androgen dependent scalp; the skin was slightly indurated on palpation []. There were focal petechiae. The lateral sides of both eyebrows and most of the hair on the chest were absent too. The axillary, pubic, and limb hair was preserved.\nTrichoscopic examination of the scalp showed prominent salmon colored halos (0.3-1 mm in diameter) surrounding the follicular ostia []. These halos were seen around empty follicles as well as around follicles containing terminal and vellus hairs, black dots, and/or broken hairs. This trichoscopic finding was present over the entire scalp.\nA 4 mm punch biopsy from the alopecic scalp was obtained and bisected in horizontal sections. There was overall preserved follicular architecture with intact sebaceous glands []. There were no terminal anagen follicles, one terminal telogen follicles, two vellus anagen follicles and 10 atrophic follicular structures reminiscent of telogen germinal units (secondary germs). All follicular structures were surrounded by amorphous eosinophilic material, which formed either a compact- or loosely- folded fibrillar cast []. The same material was deposited in all fibrous streamers, which appeared eosinophilic, homogenized and avascular. Congo red stain evaluated under polarized light highlighted the apple green stain of the amyloid [].\nSerum electrophoresis and bone marrow biopsy established the diagnosis of multiple myeloma.