CAMEO
MULTIPLE MYELOMA PRESENTING AS MIGRATORY PANNICULITIS PAUL-SYLVAIN ERENETTE, M.D., ORA B. PALTIEL, M.D., M.S.C., F.R.C.P.C, THOMAS HUDSON, M.D., E.R.C.P.C, MARIANNA NEWKIRK, PH.D., AND A. KEVIN WATTERS, M.D., E.R.C.P.C.
A 70-year-old man was admitted to our hospital with a 4month history of migratory pain, erythema, and swelling around the pelvic girdle. The patient was first seen 2 weeks after the onset of his symptoms in another hospital where a deep abscess of the right inguinal region was suspected. He received broad spectrum antibiotics and underwent surgical exploration. A biopsy of the involved area was interpreted as extensive inflammation with steatonecrosis. During the next 2 months, his condition deteriorated; the reddened areas of his skin waxed and waned, migrating to his buttocks, thighs, inguinal, and sacroiliac regions. Each appearance of the erythema was accompanied by a fever (~38.5-39.0°C) and profound weakness. Physical examination revealed a weak, cachectic man. Vital signs revealed a blood pressure of 95/50 with a 10 mmHg orthostatic drop, pulse 90/min, normal temperature. The liver edge was palpable with a span of 13 cm. There was no splenomegaly. Extremities showed an 8 x 10 cm erythematous, warm, tender area in the medial region of the left thigh with induration in the inguinal region. The remainder of the examination was unremarkable. The leukocyte count was 20.3 x 10^ with a normochromic normocytic anemia (HG 96 g/L); Wintrobe sedimentation rate was 65 mm/hr; serum protein electrophoresis showed an abnormal paraprotein band in the end gamma region, which measured 17.1 g/L and typed as IgG lambda. Serum IgG was 25.7 (5-18 g/U, IgA 1.4 (0.2-5 g/L), and IgM 0.37(0.5-3.0 g/L). Urine electrophoresis did not detect any protein bands. The 24 hour urine collection demonstrated 0.09 g of protein per day. A bone marrow aspiration revealed areas of replacement with immature plasma cells, representing 19% of total cells. A complete skeletal survey showed questionable lytic lesions in the left and right distal humerus. Coagulation tests were normal, with a mild increase in plasma fibrinogen. The amylase and liver function tests were normal. Antinuclear, antimitochondrial, and antismooth muscle antibody, C3, C4, a-antitrypsin, and VDRL were all negative or normal. Anti-parietal cell antibody was positive. Borrelia burgdorferi serology was negative. An abdominal CT scan failed to reveal any abnormality of the pancreas. A wedge biopsy of the skin was repeated and showed a lobular panniculitis with fat necrosis
without evidence of vasculitis (review of original biopsy demonstrated the same findings [Fig. 1]). The patient was given indomethacin, 25 mg t.i.d., with marked Improvement of his skin lesions. His multiple myeloma was treated subsequently with melphalan and prednisone. No recurrence has been documented 8 months after initiation of therapy. In order to Investigate a possible relationship between the paraprotein and the panniculitis, indirect immunofluorescence on heterologous normal skin was carried out using several dilutions of the patient's purified IgG. No antibody binding could be demonstrated with this study.
DISCUSSION
Weber-Christian disease (WCD) classically occurs in young women, presenting with fever, malaise, arthralgia, and skin lesions that are usually located over the extremities but can present on multiple other areas.^ Our case was similar to those described in the literature although the migratory behavior of the lesions is not typical. Several diseases have been associated with lobular panniculitis including tuberculosis, diabetes mellitus, lupus erythematosus, disseminated intravascular coagulopathy, ai antitrypsin deficiency, trauma, Lyme disease, pancreatitis, and pancreatic carcinoma.^•^•''
From the Departments of Medicine and Pathology, Montreal General Hospital, McGill University, Montreal, Quebec, Canada. Address for correspondence: Dr. Ora Paltiel, Montreal General Hospital, 1650 Cedar Avenue, Suite 7116, Montreal, Quebec, Canada, H3G 1A4.
Figure 1. Hematoxylin and eosin stained sections of suhcutaneus tissue showing lohular panniculitis without vasculitis (original magnification x 50). 424
Multiple Myeloma Frenette et al.
REEERENCES
Skin lesions in multiple myeloma (with the exception of amyloid deposits and extramedullary plasmocytoma) are rare.' The only other report of an association between multiple myeloma and WCD [Medline search] was found in the Japanese literature.'' In that case, wCD had been present 20 years prior to the diagnosis of IgC lambda myeloma. The monoclonal peak demonstrated binding activity to cardiolipin and the patient had a lupus anticoagulant. Panniculitis has been seen in other lymphoproliferative disorders.^'^ Other authors have postulated a causal relationship with an immune process.* We could not demonstrate any antibody binding in our study. There may be several explanations to account for a negative study: the frozen section may have retained an inadequate quantity of fat to detect binding activity; cofactors which are required for antibody binding in vivo may be absent when using a heterologous and in vitro preparation. Alternatively, this case may merely represent a chance association between two relatively rare conditions. '
• .
'
^,
Lever WF, Schaumburg-Lever G. Histopathology of the skin. Philadelphia: JB Lippincott, 1990:275.
2.
Panush RS, Yonker RA, Dlesk A, et al. Weber-Christian disease. Medicine 1985; 64:181-191. Forstrom L, Winkelmann RK. Acute panniculitis. Arch Dermatol 1977; 113:909-917. Kramer N, Rickert RR, Brodkin RH, et al. Septal panniculiris as a manifestation of lyme disease. Am J Med 1986; 81:149-152. Shigekiyo T, Kosaka M, Saito S, et al. IgG (lambda) myeloma occurred during the course of Weber-Christian disease: a case report. Rinsho Ketsueki 1986; 27: 2291-2297.
3. 4.
5.
6.
7.
' ;
indomethacin: Indocin
Peters MS, Winkelmann RK. Cytophagic panniculitis and B cell lymphoma. J Am Acad Dermatol 1985; 13: 882-885. Aronson IK, West DP, Variakojis D, et al. Panniculitis associated with cutaneous T-cell lymphoma and cytophagocytic histiocytosis. BrJ Dermatol 1985; 112:87-96. Ciclitira PJ, Wight DGD, Dick AP. Systemic WeberChristian disease: a case report with lipoprotein profile and immunological evaluation. BrJ Dermatol 1980; 103: 685-690.
A popular pair of tattoos (see p.428). From the World of Tattoos collection, Honolulu, HI. Submitted hy Norman Goldstein, M.D., Honolulu, HI. 425
:
1.
8. DRUC NAME
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MULTIPLE MYELOMA PRESENTING AS MIGRATORY PANNICULITIS PAUL-SYLVAIN ERENETTE, M.D., ORA B. PALTIEL, M.D., M.S.C., F.R.C.P.C, THOMAS HUDSON, M.D., E.R.C.P.C, MARIANNA NEWKIRK, PH.D., AND A. KEVIN WATTERS, M.D., E.R.C.P.C.
A 70-year-old man was admitted to our hospital with a 4month history of migratory pain, erythema, and swelling around the pelvic girdle. The patient was first seen 2 weeks after the onset of his symptoms in another hospital where a deep abscess of the right inguinal region was suspected. He received broad spectrum antibiotics and underwent surgical exploration. A biopsy of the involved area was interpreted as extensive inflammation with steatonecrosis. During the next 2 months, his condition deteriorated; the reddened areas of his skin waxed and waned, migrating to his buttocks, thighs, inguinal, and sacroiliac regions. Each appearance of the erythema was accompanied by a fever (~38.5-39.0°C) and profound weakness. Physical examination revealed a weak, cachectic man. Vital signs revealed a blood pressure of 95/50 with a 10 mmHg orthostatic drop, pulse 90/min, normal temperature. The liver edge was palpable with a span of 13 cm. There was no splenomegaly. Extremities showed an 8 x 10 cm erythematous, warm, tender area in the medial region of the left thigh with induration in the inguinal region. The remainder of the examination was unremarkable. The leukocyte count was 20.3 x 10^ with a normochromic normocytic anemia (HG 96 g/L); Wintrobe sedimentation rate was 65 mm/hr; serum protein electrophoresis showed an abnormal paraprotein band in the end gamma region, which measured 17.1 g/L and typed as IgG lambda. Serum IgG was 25.7 (5-18 g/U, IgA 1.4 (0.2-5 g/L), and IgM 0.37(0.5-3.0 g/L). Urine electrophoresis did not detect any protein bands. The 24 hour urine collection demonstrated 0.09 g of protein per day. A bone marrow aspiration revealed areas of replacement with immature plasma cells, representing 19% of total cells. A complete skeletal survey showed questionable lytic lesions in the left and right distal humerus. Coagulation tests were normal, with a mild increase in plasma fibrinogen. The amylase and liver function tests were normal. Antinuclear, antimitochondrial, and antismooth muscle antibody, C3, C4, a-antitrypsin, and VDRL were all negative or normal. Anti-parietal cell antibody was positive. Borrelia burgdorferi serology was negative. An abdominal CT scan failed to reveal any abnormality of the pancreas. A wedge biopsy of the skin was repeated and showed a lobular panniculitis with fat necrosis
without evidence of vasculitis (review of original biopsy demonstrated the same findings [Fig. 1]). The patient was given indomethacin, 25 mg t.i.d., with marked Improvement of his skin lesions. His multiple myeloma was treated subsequently with melphalan and prednisone. No recurrence has been documented 8 months after initiation of therapy. In order to Investigate a possible relationship between the paraprotein and the panniculitis, indirect immunofluorescence on heterologous normal skin was carried out using several dilutions of the patient's purified IgG. No antibody binding could be demonstrated with this study.
DISCUSSION
Weber-Christian disease (WCD) classically occurs in young women, presenting with fever, malaise, arthralgia, and skin lesions that are usually located over the extremities but can present on multiple other areas.^ Our case was similar to those described in the literature although the migratory behavior of the lesions is not typical. Several diseases have been associated with lobular panniculitis including tuberculosis, diabetes mellitus, lupus erythematosus, disseminated intravascular coagulopathy, ai antitrypsin deficiency, trauma, Lyme disease, pancreatitis, and pancreatic carcinoma.^•^•''
From the Departments of Medicine and Pathology, Montreal General Hospital, McGill University, Montreal, Quebec, Canada. Address for correspondence: Dr. Ora Paltiel, Montreal General Hospital, 1650 Cedar Avenue, Suite 7116, Montreal, Quebec, Canada, H3G 1A4.
Figure 1. Hematoxylin and eosin stained sections of suhcutaneus tissue showing lohular panniculitis without vasculitis (original magnification x 50). 424
Multiple Myeloma Frenette et al.
REEERENCES
Skin lesions in multiple myeloma (with the exception of amyloid deposits and extramedullary plasmocytoma) are rare.' The only other report of an association between multiple myeloma and WCD [Medline search] was found in the Japanese literature.'' In that case, wCD had been present 20 years prior to the diagnosis of IgC lambda myeloma. The monoclonal peak demonstrated binding activity to cardiolipin and the patient had a lupus anticoagulant. Panniculitis has been seen in other lymphoproliferative disorders.^'^ Other authors have postulated a causal relationship with an immune process.* We could not demonstrate any antibody binding in our study. There may be several explanations to account for a negative study: the frozen section may have retained an inadequate quantity of fat to detect binding activity; cofactors which are required for antibody binding in vivo may be absent when using a heterologous and in vitro preparation. Alternatively, this case may merely represent a chance association between two relatively rare conditions. '
• .
'
^,
Lever WF, Schaumburg-Lever G. Histopathology of the skin. Philadelphia: JB Lippincott, 1990:275.
2.
Panush RS, Yonker RA, Dlesk A, et al. Weber-Christian disease. Medicine 1985; 64:181-191. Forstrom L, Winkelmann RK. Acute panniculitis. Arch Dermatol 1977; 113:909-917. Kramer N, Rickert RR, Brodkin RH, et al. Septal panniculiris as a manifestation of lyme disease. Am J Med 1986; 81:149-152. Shigekiyo T, Kosaka M, Saito S, et al. IgG (lambda) myeloma occurred during the course of Weber-Christian disease: a case report. Rinsho Ketsueki 1986; 27: 2291-2297.
3. 4.
5.
6.
7.
' ;
indomethacin: Indocin
Peters MS, Winkelmann RK. Cytophagic panniculitis and B cell lymphoma. J Am Acad Dermatol 1985; 13: 882-885. Aronson IK, West DP, Variakojis D, et al. Panniculitis associated with cutaneous T-cell lymphoma and cytophagocytic histiocytosis. BrJ Dermatol 1985; 112:87-96. Ciclitira PJ, Wight DGD, Dick AP. Systemic WeberChristian disease: a case report with lipoprotein profile and immunological evaluation. BrJ Dermatol 1980; 103: 685-690.
A popular pair of tattoos (see p.428). From the World of Tattoos collection, Honolulu, HI. Submitted hy Norman Goldstein, M.D., Honolulu, HI. 425
:
1.
8. DRUC NAME
.
