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Scleredema-associated IgA myeloma with myelofibrosis in a young adult: a case report/Genc bir yetiskinde miyelofibroz ile sklerema iliskili IgA miyelom: bir vaka raporu.

Introduction

Scleredema of Buschke or scleredema adultorum is a rare primary cutaneous fibromucinosis of unknown etiology that is characterized by extensive woody, non-pitting induration of skin throughout the body, with systemic involvement being rather uncommon. There is a known association with benign monoclonal gammopathy and less commonly with frank myelomas [1,2]. The disease runs a variable course and is usually self-limiting; however, a few fatal cases have been reported [3].

Case Report

A 24-year-old male presented with an eight-year history of progressive diffuse cutaneous thickening, which initially started from the face and slowly progressed over time to involve the trunk, arms and thighs. Hands and feet were relatively spared. He had difficulty in swallowing and walking. There was no preceding history of diabetes, infection or trauma. There was no history of Raynaud's phenomenon. On examination, he had diffuse induration of skin on face, trunk, arms and legs, and consistent pressure demonstrated a brawny edema. The face was woody and expressionless (Figure 1) and diffuse thickening of the tongue was noticed. There was limitation of neck, arms and feet movements. The spine showed kyphotic deformity.

On investigation, the patient had anemia (Hb 105 g/L) with normal total leukocyte and platelet counts, and erythrocyte sedimentation rate (ESR) of 55 mm in the first hour. Peripheral smear showed rouleaux formation. However, no leukoerythroblastic picture or tear-drop poikilocytes were seen. Blood urea, electrolytes, blood glucose level, liver function test, thyroid function test, rheumatoid factor and antinuclear antibody tests were normal. Serum protein electrophoresis showed an abnormal M band (25.5%, 1.8 g/dl) in the [beta]-[gamma] interzone, which on immunofixation was identified as a monoclonal IgA-kappa protein. Total serum protein levels were 7.9 g/dl. Serum [beta]2 microglobulin level was 5500 [micro]g/L (normal: 700-3400 [micro]g/L). Polyclonal [gamma] globulins were reduced. Urine was negative for Bence Jones protein, and urine protein electrophoresis did not reveal any M band. Twenty-four hour urine albumin was 0.02 g/day. Chest X-ray and skeletal survey revealed diffuse osteoporosis of the spine and bilateral phalanges.

Pathological findings

A skin biopsy from the left shoulder showed normal epidermis and increased broad fibrous bands in the deep dermis. However, Alcian blue stain for any mucin deposition in the skin was negative. Congo red staining for amyloidosis was negative. A bone marrow aspirate from the iliac crest showed 55% plasma cells including immature forms (Figure 2). The bone marrow biopsy showed loose, edematous stroma with interstitial as well as focal increase in plasma cells. There were areas of increased fibrosis with presence of coarse reticulin fibers (reticulin grade 3) (Figure 3). The Alcian blue stain for any mucin deposition was negative. Focally, the bony trabeculae showed areas of new bone formation (Figure 4). Hence, the patient was diagnosed as scleredema-associated IgA multiple myeloma with myelofibrosis and started on thalidomide, dexamethasone and zoledronic acid.

[FIGURE 1 OMITTED]

Discussion

Scleredema was first described by Buschke in 1902 as a systemic disease with woody, non-pitting induration of the skin starting at the nape of the neck and gradually spreading throughout the body, sparing the palms and soles [4]. It usually affects young adults with slight female predominance. The disease has been divided into two main types. The first is a self-limiting form often occurring after an acute febrile episode and resolving spontaneously within a few months [4]. The second type is associated with diabetes mellitus and runs a prolonged course [5]. There have been many reports of an association with a monoclonal gammopathy [6,7]. Rarely, there have been cases of scleredema associated with multiple myeloma [1,2]. In such cases, the scleredema presents several years before the monoclonal gammopathy [7].

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

[FIGURE 4 OMITTED]

Various hypotheses have been proposed to explain the association of scleredema with monoclonal gammopathy. According to Kovary et al. [8], the presence of immunoglobulins reflects a state of chronic immunostimulation due to antigenic substances present in the connective tissue. Another possibility is that the scleredema is caused by the monoclonal gammopathy, as the serum from the affected patient has been shown to stimulate collagen synthesis in normal skin fibroblast cultures [6]; however, any clear evidence is lacking. It is also possible that the gammopathy and scleredema are caused by the same etiological factor, which is hitherto unknown.

Mild marrow fibrosis may be observed in at least 27% of cases of myeloma but extensive fibrosis is rare [9,10]. Although mucin deposition [11] in the bone marrow has been reported in scleredema, to the best of our knowledge, myelofibrosis has not been reported. Whether the myelofibrosis was part of the primary disease process itself, reflecting extracutaneous systemic involvement, or whether it was secondary to myeloma is difficult to discriminate, since mucin can be absent in late lesions of scleredema, which show only fibrosis.

A variety of treatment approaches have been tried with variable success rates, including corticosteroids, immunosuppressants, thyroid hormones, antifibrotic therapy, hyaluronidase and electron beam therapy. The myeloma chemotherapy usually results in pronounced improvement of the associated scleredema [1,2,6]. Our patient also showed an improvement in the texture of the skin and generalized well-being after receiving chemotherapy, but he has not yet been evaluated with follow-up serum immunoglobulin levels or a repeat bone marrow biopsy. He was discharged after the first cycle of chemotherapy and is on regular follow-up.

Received: January 11, 2008 Accepted: September 10, 2008

Gelis tarihi: 11 Ocak 2008 Kabul tarihi: 9 Eylul 2008

References

(1.) Salisbury JA, Shallcross H, Leigh IM. Scleredema of Buschke associated with multiple myeloma. Clin Exp Dermatol 1988;13:269-70.

(2.) Hodak E, Tamir R, David M, Hart M, Sandbank M, Pick A. Scleredema adultorum associated with IgG-kappa multiple myeloma-a case report and review of the literature. Clin Exp Dermatol 1988;13:271-4.

(3.) Leinwand I. Generalized scleredema: report with autopsy findings. Ann Intern Med 1951;34:226-38.

(4.) Buschke A. Ueber scleredema. Brit Klin Wochenschr 1902;39:955-6.

(5.) McNaughton F, Keczkes K. Scleredema adultorum and diabetes mellitus (scleredema diutinum). Clin Exp Dermatol 1983;8:41-5.

(6.) Ohta A, Uitto J, Oikarinen AI, Palatsi R, Mitrane M, Bancila EA, Seibold JR, Kim HC. Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro. J Am Acad Dermatol 1987;16:96-107.

(7.) Angeli-Besson C, Koeppel MC, Jacquet P, Andrac L, Sayag J. Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinemia. Br J Dermatol 1994;130:394-7.

(8.) Kovary PM, Vakilzadeh F, Macher E, Zaun H, Merk H, Goerz G. Monoclonal gammopathy in scleredema. Observation in three cases. Arch Dermatol 1981;117:536-9.

(9.) Abildgaard N, Bendix-Hansen K, Kristensen JE, Vejlgaard T, Risteli L, Nielsen JL, Heickendorff L. Bone marrow fibrosis and disease activity in multiple myeloma monitored by amino terminal propeptide of procollagen III in serum. Br J Haematol 1997;99:641-8.

(10.) Krzyzaniak RL, Buss DH, Cooper MR, Wells HB. Marrow fibrosis and multiple myeloma. Am J Clin Pathol 1988;89:63-8.

(11.) Basarab T, Burrows NP, Munn SE, Russel Jones R. Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia. Br J Dermatol 1997;136:939-42.

Seema Rao, Rakhee Kar, Hara Prasad Pati, Renu Saxena

Department of Hematology, AIIMS, New Delhi, India

Address for Correspondence: M.D. Renu Saxena, Dept. Of Hematology, irch Building, all India Institute Of Medical Sciences, ansari Nagar, new Delhi-110029 11002 New Delhi-India, Phone: 91-011-26594670-91 E-mail: renusax@hotmail.com
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Title Annotation:immunoglobulin A
Author:Rao, Seema; Kar, Rakhee; Pati, Hara Prasad; Saxena, Renu
Publication:Turkish Journal of Hematology
Article Type:Case study
Geographic Code:9INDI
Date:Dec 1, 2008
Words:1237
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