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Complete remission of scleredema adultorum of Buschke associated with multiple myeloma after bortezomib-based treatment and autologous peripheral blood stem cell transplantation

1 Department of Dermatology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
2 Department of Dermatology, Chang Gung Memorial Hospital, Linkou; School of Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan
3 Department of Dermatology, Chang Gung Memorial Hospital, Linkou; School of Medicine, College of Medicine; Graduate Institute of Clinical Medical Sciences, Chang Gung University, Taoyuan, Taiwan

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Date of Submission07-Aug-2020
Date of Decision18-Mar-2021
Date of Acceptance25-Mar-2021
Date of Web Publication19-Jul-2021

How to cite this URL:
Su HJ, Chang YY, Ng CY. Complete remission of scleredema adultorum of Buschke associated with multiple myeloma after bortezomib-based treatment and autologous peripheral blood stem cell transplantation. Dermatol Sin [Epub ahead of print] [cited 2021 Sep 16]. Available from: https://www.dermsinica.org/preprintarticle.asp?id=321875

Dear Editor,

A 53-year-old male presented with progressive asymptomatic cutaneous hardening on the nape of his neck for 6 months. The lesion had an insidious onset and caused the patient to experience impaired movement of the neck. Physical examination disclosed a symmetric woody-hard, nonpitting indurated erythematous plaque on the nape of his neck [Figure 1]a. A skin biopsy from the nape showed thickened reticular dermis with dermal fenestration [Figure 2]a and [Figure 2]b. Abundant mucin deposition in the deep dermis was demonstrated by Alcian blue stain [Figure 2]c. Scleredema adultorum of Buschke was therefore diagnosed. Laboratory data revealed anemia (9.7 g/dl) and hypercalcemia (10.2 mg/d). His renal function (creatinine: 1.09 mg/dL; range: 0.64–1.27), glycated hemoglobin (6.0%; range: 5.7–6.4), complement 3 (90 mg/dL; range: 90–180), and complement 4 (39 mg/dL; range: 10–40) were within the normal limits. Both anti-nuclear antibody and anti-streptolysin were negative. Serum protein electrophoresis showed a monoclonal band in the γ region and immunofixation confirmed a monoclonal immunoglobulin A and κ light chain. Radiographic skeletal survey showed diffuse osteopenia. In particular, multiple radiolucent lesions were seen at the skull and eighth thoracic spine. A bone marrow biopsy [Figure 2]d and [Figure 2]e showed increased plasma cells (80%) and staining of κ light chain proteins. A diagnosis of scleredema adultorum of Buschke in association with multiple myeloma of IgA-κ was made. The patient was started on VTD regimen (Bortezomib [Velcade] 1–1.3 mg/m2 subcutaneous on days 1, 4, 7, and 11; Thalidomide 100 mg perorally daily; Dexamethasone 40 mg perorally daily, four cycles). Subsequently, he received autologous peripheral blood stem cell transplantation after a preconditioning of high-dose melphalan. The treatment resulted in the rapid decline of monoclonal IgA with complete disappearance of paraprotein. The skin induration gradually improved, and he regained full neck mobility after 9 months of therapy [Figure 1]b. The patient remained disease-free at 24-month follow-up.
Figure 1: (a) Photograph showing a woody hard induration of the nape of our patient. (b) Regression of the skin dermal induration after multiple myeloma treatment with VTD regimen and autologous peripheral stem cell transplantation.

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Figure 2: (a) Histopathologically, the lesion shows thickened reticular dermis with dermal fenestration (Hematoxylin and eosin [H and E] stain ×40 magnification); (b) Higher magnification of figure 2a shows widened spaces with mucin between normal collagen bundles with no increased of fibroblast nor inflammation (H and E stain ×200 magnification); (c) Abundant mucin deposition was confirmed by Alcian blue stain (Alcian blue, original magnification ×40); (d) Histopathology of the bone marrow biopsy shows diffuse sheets of plasma cells (H and E stain ×100 magnification); (e) Higher magnification of figure 2d shows increased plasma cells with large and prominent nucleoli, accounting for 80% of all nucleated cells (H and E stain ×400 magnification).

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Scleredema adultorum of Buschke is a rare connective tissue disorder, characterized by symmetrical firm, woody nonpitting induration of the skin that typically affects the neck and gradually expands to the face, shoulders, and trunk.[1] The hands and feet are usually spared. The differential diagnosis includes scleromyxedema and morphea (localized scleroderma). Histologically, both scleredema and scleromyxedma demonstrate mucin deposition, while morphea showed only fibrosis without mucin deposition. Scleromyxedema is characterized by waxy firm papules on induration, with fibrotic change on pathology examination, which differentiates from scleredema.[2] Scleredema was classified into three groups based on associated comorbidities and different prognosis. Group I accounts for 55% of scleredema and is the classical type following a febrile illness (most commonly streptococcal infection of respiratory tract). It usually resolves spontaneously within months to 2 years. Group II accounts for 25% of scleredema and is associated with paraproteinemia (e.g., monoclonal gammopathy of undeterminated significance and multiple myeloma). These patients are typically younger and experience a protracted course compared with those without gammopathy.[3],[4] Group III constitutes around 20% of scleredema and is related to poorly controlled type II diabetes mellitus (scleredema diabeticorum). Scleredema associated with diabetes or monoclonal gammopathy has a slowly progressive and unremitting course. Although the mechanism is still poorly understood, Ohta et al.[5] have shown that the serum isolated from affected patient stimulates collagen production in normal skin fibroblast cultures. Therefore, serum factors produced by myeloma cells might stimulate the production of mucinosis and dermal thickening.[5]

In the literature review, only 17 cases of multiple myeloma-associated scleredema have been described. The male-to-female ratio was 1.2:1, and the mean age of onset was 60.1 ± 13.6 years. IgG-κ was the most common (45%), followed by IgA-κ (36%), IgG-λ (9%), and IgA-λ (9%).[3],[6] Our patient is in his early fifties and his skin lesion leads to the early diagnosis of underlying multiple myeloma of IgA-κ.

Various treatments have been attempted in scleredema patients with limited success including ultraviolet A1 phototherapy or bath-psoralen plus ultraviolet A phototherapy, local radiotherapy, antibiotics, pituitary extract, and immunosuppressive agents (systemic and intralesional corticosteroid, methotrextate, azathioprine, cyclosporine, and cyclophosphamide). In patients with multiple myeloma-associated scleredema, treatment directed to the underlying myeloma usually results in profound improvement of associated scleredema.[1],[7] To date, several chemotherapy regimens have been used including melphalan, cyclophosphamide, vincristine, thalidomide plus steroids, and bortezomib or CVD regimen (cyclophosphamide, bortezomib [Velcade], and dexamethasone) followed by autologous transplantation.[6],[8] Of note, our case is the first case successfully treated with VTD regimen (bortezomib [Velcade], thalidomide, and dexamethasone) followed by autologous peripheral blood stem cell transplantation. Physicians should be familiar with the presentation of scleredema and its associated systemic involvement as timely diagnosis is important for the early treatment of underlying malignancy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

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Conflict of interest

There are no conflicts of interest.

  References Top

Beers WH, Ince A, Moore TL. Scleredema adultorum of Buschke: A case report and review of the literature. Semin Arthritis Rheum 2006;35:355-9.  Back to cited text no. 1
Chen KY, Tzeng IS, Lee YY, Ting SW, Chang YY, Ng CY. Scleromyxedema and lichen myxedematosus: Is it associated with viral hepatitis? J Dermatol 2019;46:879-85.  Back to cited text no. 2
Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, et al. Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients. J Eur Acad Dermatol Venereol 2015;29:2399-404.  Back to cited text no. 3
Salisbury JA, Shallcross H, Leigh IM. Scleredema of Buschke associated with multiple myeloma. Clin Exp Dermatol 1988;13:269-70.  Back to cited text no. 4
Ohta A, Uitto J, Oikarinen AI, Palatsi R, Mitrane M, Bancila EA, et al. Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro. J Am Acad Dermatol 1987;16:96-107. https://pubmed.ncbi.nlm.nih.gov/31084620/.  Back to cited text no. 5
Keragala BS, Herath HM, Janappriya GH, Dissanayaka BS, Shyamini SC, Liyanagama DP, et al. Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature. J Med Case Rep 2019;13:145.  Back to cited text no. 6
Szturz P, Adam Z, Vašků V, Feit J, Krejčí M, Pour L, et al. Complete remission of multiple myeloma associated scleredema after bortezomib-based treatment. Leuk Lymphoma 2013;54:1324-6.  Back to cited text no. 7
Krejci ZA, Pour L, Michalkova E, Sandecka V, Szturz P, Kral X, et al. Scleredema associated with multiple myeloma or MGUS: treatment report of four cases [abstract]. Clin Lymphoma Myeloma Leukemia 2015;15:e207.  Back to cited text no. 8

Correspondence Address:
Chau Yee Ng,
Department of Dermatology, Chang Gung Memorial Hospital, No. 5, Fushin Street, Linkou, Taoyuan 33305
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_16_21


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