Indian Journal of Dermatology
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CORRESPONDENCE
Year : 2014  |  Volume : 59  |  Issue : 2  |  Page : 202-204
A case of localized papular mucinosis showing excellent response to cyclophosphamide


1 Department of Dermatology, Hi-Tech Medical College, Rourkela, India
2 Department of Dermatology, KPC Medical College, Kolkata, India
3 Department of Dermatology, Katihar Medical College, Bihar, India
4 Department of Dermatology, Medical College and Hospital, Kolkata, India

Date of Web Publication21-Feb-2014

Correspondence Address:
Piyush Kumar
Department of Dermatology, Katihar Medical College, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.127697

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How to cite this article:
Sa DK, Ghosh A, Kumar P, Gharami RC. A case of localized papular mucinosis showing excellent response to cyclophosphamide. Indian J Dermatol 2014;59:202-4

How to cite this URL:
Sa DK, Ghosh A, Kumar P, Gharami RC. A case of localized papular mucinosis showing excellent response to cyclophosphamide. Indian J Dermatol [serial online] 2014 [cited 2019 Nov 17];59:202-4. Available from: http://www.e-ijd.org/text.asp?2014/59/2/202/127697


Sir,

Papular mucinosis (PM), also called lichen myxedematosus, is a primary cutaneous mucinosis and is characterized by dermal mucin deposition in absence of thyroid disorder. [1] Many attempts have been made to classify PM; the current and most accepted classification is by Rongioletti et al.[2] Scleromyxedema, the most common form, is almost always associated with paraproteinemia. Localized PM is confined to a few sites and is not usually associated with systemic findings. However, a few cases have been reported in Human immunodeficiency virus (HIV) infected patients. [3] Discrete papular PM, the most common type of localized form occurs symmetrically over trunk and limbs with sparing of face and distal parts of extremities. [1] Here, we report a case of discrete papular type of PM, who showed excellent response to oral cyclophosphamide therapy.

A 52-year-old male presented with multiple, closely set, skin colored flat-topped pruritic papules distributed symmetrically over neck, trunk, and scalp for 2 years [Figure 1]. On palpation, there was thickening and tightening of skin over the lesions. Rest of the history and examination was unremarkable. Before he presented to us, he was treated with topical corticosteroid and topical retinoid, but without any improvement. The complete blood count, thyroid profile, blood sugar, lipid profile, and urine (routine and microscopic) analysis did not reveal any abnormality. Patient was found seronegative for HIV and serum electrophoresis showed no paraproteinemia. Skin biopsy showed normal epidermis, mucin deposition in the dermis and dermal fibrosis [Figure 2]. We diagnosed the case as discrete papular type of localized PM. We started him on oral cyclophosphamide (50 mg/day), with monthly monitoring of complete blood count, liver function test, and urine analysis. After 3 months, there was a marked improvement in skin lesions. The lesions had disappeared, pruritus subsided and there was a significant improvement of skin texture [Figure 3]a-c.
Figure 1: Grouped skin colored papules on head, neck, and upper back (a). Close up of lesions over scalp (b) and chest and flank (c)

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Figure 2: Deposition of mucin in dermis (a) and confirmed on alcian clue stain (b)

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Figure 3: (a) Post - treatment photographs documenting resolution of lesions over upper back (b) Post - treatment photographs documenting resolution of lesions over scalp (b) and flank (c)

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PM or lichen myxedematosus is clinically characterized by few to multiple, 2-5 mm, skin colored, firm, waxy, dome shaped papules which may coalesce together to form plaque causing thickening and hardening of skin. [1] The etiology of PM is not completely understood; however, it is believed that PM occurs due to proliferation of fibroblasts with fibrosis and excessive deposition of mucin (glycosaminoglycans) in the dermis. Paraproteins may play a role in scleromyxedema. Localized PM may occur due to local plasma cell dyscrasia, but other triggering factors must be involved. Autoantibodies and cytokines such as interleukin-1, tumor necrosis factor-α, and transforming growth factor-β stimulate fibroblast proliferation. [4] Histochemically, they are heterogeneous mixture of acid glycosaminoglycans which stain positive with alcian blue and toluidine blue. [2],[4]

Treatment of PM is difficult and often ineffective. Scleromyxedema has shown some improvement with Psoralen-Ultraviolet A therapy (PUVA) therapy, oral glucocorticoids, retinoids, thalidomide, plasmapheresis, extracorporeal photopheresis, dermabrasion, carbon dioxide laser excision, melphalan, interferon-α, and intravenous immunoglobulin in isolated cases. [1],[2],[4] Some cases of localized PM were treated successfully with topical tacrolimus. Topical corticosteroid was found to be ineffective in localized PM. The disease is usually progressive, but it has potential for long-term regression. [1],[2]

Jessen et al.[5] have documented the role of oral cyclophosphamide as an effective therapy in PM. Cyclophsphamide is an alkylating agent and acts by cross-linking of DNA strands, leading to cell death. It probably suppresses B cell function more as compared to T cell and hence, it might disrupt local plasma cell dyscrasia leading to reversal of factors responsible for fibroblast proliferation and mucin deposition in PM. [5] Adverse effects of cyclophosphamide include leukopenia, thrombocytopenia, anemia, dysuria, urinary urgency, hemorrhagic cystitis, hepatotoxicity, amenorrhea, and azoospermia. [5] As observed in our case, cyclophosphamide appears to be safe and effective treatment modality in localized PM. However, large scale study is needed to establish or refute its role in PM.

 
   References Top

1.Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273-81.  Back to cited text no. 1
    
2.Rongioletti F. Lichen myxedematosus (papular mucinosis): New concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100-4.  Back to cited text no. 2
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3.Rongioletti F, Ghigliotti G, De Marchi R, Rebora A. Cutaneous mucinoses and HIV infection. Br J Dermatol 1998;139:1077-80.  Back to cited text no. 3
    
4.Rongioletti F, Rebora A. Cutaneous mucinoses: Microscopic criteria for diagnosis. Am J Dermatopathol 2001;23:257-67.  Back to cited text no. 4
    
5.Jessen RT, Straight M, Becker LE. Lichen myxedematosus. Treatment with cyclophosphamide. Int J Dermatol 1978;17:833-9.  Back to cited text no. 5
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