Treatment with chemotherapy of scleredema associated with Ig A myeloma
Authors: Santos‐Juanes Jorge , Osuna C. Galache , Iglesias J. R. Curto , De Quiros J. F. B. , Del Río J. Sánchez 
Published: March 2003

A 70‐year‐old woman was referred to the dermatology outpatient clinic in January 1999. During the previous 12 months she had developed a progressive induration and stiffness of the skin of her face, neck, shoulders, and upper aspect of her arms. There was no history of a preceding infection or of diabetes mellitus. An examination revealed that the skin of her face, back, shoulders, arms and chest was shiny, erythematous, and diffusely indurated, with a wooden consistency and decreased range of motion. Consistent firm pressure demonstrated a brawny edema. The face lacked expression, with minimal wrinkling (Fig. 1). Lymphadenopathies were not found in any location.

Woody face with minimal wrinkling

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A skin biopsy specimen was taken from the left shoulder. A hematoxylin‐eosin stain revealed fenestrated collagen bundles separated by wide, clear spaces in the mid and reticular dermis (Fig. 2). Alcian blue staining (present at pH 2.5 but lost at 0.5) demonstrated nonsulfated acid mucopolysaccharides in bands and streaks between collagen bundles in the mid and reticular dermis. A diagnosis of scleredema was made.

Collagen bundles separated by wide, clear spaces in the mid and reticular dermis

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The hemoglobin value, RBC and WBC counts, and differential cell count, chemistry, urinalysis, glucose tolerance test, antistreptolysin titre, and thyroid function tests were all within normal limits. The erythrocyte sedimentation rate was raised to 70 mm/h. Serum protein electrophoresis showed an abnormal ‘‘M’' band in the lambda region composed of Beta chains. Serum protein was abnormal with a monoclonal spike in the Beta region, which on subsequent immunoelectrophoresis was identified as a monoclonal Ig A‐lambda protein. Beta 2 microglobulin level was 28.6 g/L (6.0–11.5). Inmunoglobulin levels in the serum samples were as follows: Ig A, 18.30 g/L (0.8–4.0); Ig G, 4.12 g/L (6.15–16); Ig M, 2.6 g/L (0.4–3.0). Urine contained Bence‐Jones protein (L light chain). Proteins in urine at 24 h: 0.3 gr/day. ANA negative, C3 y C4 normal. Chest X‐rays was normal and skeletal survey revealed diffuse osteoporosis. CT scan of the abdomen was normal.

Bone marrow aspirate and biopsy specimens were taken from the sternum. Their examination showed 38% plasma cells.

A diagnosis of scleredema associated with Ig A lambda multiple myeloma was made. The patient was commenced on treatment with six pulses of oral melphalan 17 mg, given over 4 days (10 mg/m2 ) accompanied by oral prednisona 100 mg (60 mg/m2 ), each pulse at an interval of one month. After the third treatment, her skin began to become mobile, the Ig A levels decreased to 6.0 (0.8–4.0) and Beta 2 microglobulin level was 11.4 g/L (6.0–11.5). No bone marrow aspirate after chemotherapy was made. After the third treatment laboratory studies revealed a mild trombopenia: 98 × 109/L (140–440 × 109/L). The fourth course was delayed 2 weeks. No more complications were noted.

During a follow‐up period of 18 months, a clinical evidence of softening of the involved skin was observed. The current Ig A level is 9.96 g/L.