Aarti Shah
NHL Medical College, Ahmedabad, India
A 53-year-old female patient presented with alopecia totalis and loss of eyelashes since 2 years, edema with asymptomatic papulonodular lesions on both lower limbs since 3 years, and few hemorrhagic bullous lesions over both hands since 7-10 days. Facial edema and yellowish hue of face was seen. She was anemic and had leukocytosis. The renal function tests showed severe proteinuria (4+). Urine examination showed the presence of albumin (4+). Patient had hypothyroidism. Ultrasonography of the abdomen and pelvis showed the presence of multiple 1-2 cm sized lymph nodes in right inguinal region with subcutaneous edema in lower abdominal wall. Histopathologic examination showed scleredema adultorum. Renal biopsy specimen showed deposition of periodic acid-Schiff (PAS) negative material in mesangium (Congo red and Thio-T were positive). Patient was given tablet Melphalan 10 mg twice daily by the oncologist with steroids, but she expired of pleural effusion due to cardiac failure (? amyloid deposition) after 3 months of presentation at the skin department. Alopecia may be patchy or widespread in primary systemic amyloidosis. In our patient, alopecia totalis was present. Smooth, shiny, waxy papules or plaques are usually clustered in folds in axilla, anal, or inguinal region, face or neck, mucosal areas such as ear and tongue. Periorbital ecchymosis was also seen. Also, multiple myeloma can be associated with scleredema as seen with our case. High dose of chemotherapy is not recommended if more than two systems are involved or cardiac amyloid is present. Where myeloma and AL amyloidosis co-exist, choice of treatment for myeloma should take into account the extent of organ involvement with amyloid and the potential toxicities of individual treatments.
