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CORRESPONDENCE
Year : 2023 | Volume
: 41
| Issue : 2 | Page : 125-126
One case of a rare dermatosis: Lichen myxedematosus
Ayinuer Maimaisawuti, Palida Abulizi
Department of Dermatology, The First Affiliated Hospital of Xinjiang Medical University, Urumqi, China
Correspondence Address:
Dr. (Prof) Palida Abulizi
Department of Dermatology, The First Affiliated Hospital of Xinjiang Medical University, Urumqi
China
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ds.DS-D-22-00072
Dear Editor,
Lichen myxedematosus (LM) is a rare, chronic, progressive mucinosis that usually occurs in middle-aged people; there is no race or sex predominance. The clinical manifestations are waxy papules that can be localized or generalized, nodules, and mucin plaques involving the face, neck, trunk, and limbs. There is extracutaneous involvement in 70% to 77% of patients,[1] including neurologic, cardiac, pulmonary, hematologic, gastrointestinal, and ocular manifestations. Such involvement results in symptoms and signs corresponding to the system involved and can even lead to death. Due to its rarity, there are neither a large number of prospective studies nor any therapeutic guidelines available for the management and long-term treatment of patients who respond differently to the existing treatment methods.
A 49-year-old man presented with a 5-month history of generalized, progressive skin thickening, and stiffness. He complained of difficulty opening his mouth and limited movement of his hands and fingers due to skin stiffness. Dermatologic examination revealed diffuse, waxy, firm papules, and nodules; some were coalescing into plaques that covered his entire face. There were also many deep folds and furrows on his forehead and glabella, producing a leonine appearance [Figure 1]a. This was accompanied by swelling and erythema of the glabella and both ears. There were numerous millimetric, waxy, firm papules on his neck, abdomen, back, and limbs [Figure 1]b. The skin of the fingers was tight, and he had limited finger movement.
Figure 1: (a and b) Leonine appearance and numerous millimetric, waxy, firm papules on the forearm. (c) Histopathologic examination reveals thickened and disordered collagen fibers, mucin deposition, fibroblast proliferation, and fibrosis (H and E, ×10). (d) Positive Alcian blue staining (Alcian blue [pH 2.5], ×10). (e and f) Follow-up images.The results of routine laboratory investigations were within normal limits, including thyroid function tests and autoantibodies associated with scleroderma. There were no abnormalities noted on thyroid ultrasonography or lung computed tomography. Bone marrow evaluation revealed insufficient evidence for plasmacytoma. A skin biopsy taken from a lesion on the forehead showed mild thickening of the epidermis, mucin deposits between collagen fibers [Figure 1]c, fibroblast proliferation, and fibrosis. Alcian blue staining was positive (mucin deposition appears in blue [Figure 1]d).
We diagnosed the patient with LM and began a treatment regimen of dexamethasone, 5 mg per day, gradually reduced over the course of 1 month. He was taking cyclophosphamide combined with thalidomide for 5 months then he disappeared for 1 year until March this year. He complained of remission after using this therapeutic regimen then he stopped taking any medicine. Then, his body was stiffer than the first time, he could hardly open his eyes or mouth [Figure 1]e and [Figure 1]f. Bone marrow evaluation revealed suspected hematological involvement. Since the previous treatment was effective, cyclophosphamide combined with thalidomide was continued and follow-up regularly.
The dermatologic condition LM, also known as papular mucinosis or Arndt-Gottron syndrome, was first described by Dubreuilh in 1906; it was named and classified by Montgomery and Underwood in 1953.[1] In 2001, Rongioletti and Rebora.[1],[2] proposed a classification system comprising three subtypes: scleromyxedema (SM), localized papular mucinosis, and atypical forms of LM. Montgomery defined SM by the following four criteria: A generalized papular and sclerodermoid eruption; the microscopic triad of mucin deposition, fibroblast proliferation, and fibrosis; monoclonal gammopathy; and the absence of thyroid disease.[1],[2] More recently, it has been suggested in studies and case reports that monoclonal gammopathy and thyroid disease should not be included in the diagnostic criteria for LM,[1] and this patient lacked evidence of these two. Accordingly, the subtype of our patient was SM.
The pathogenesis of the disease is unclear. Scholars have put forward some hypotheses, including that circulating cytokines such as interleukin-1, tumor necrosis factor-α, and transforming growth factor-β may stimulate glycosaminoglycan synthesis and fibroblast proliferation in the skin, and increased paraprotein levels may promote the deposition of mucin.[1],[3] However, paraprotein levels do not correlate with disease severity or treatment response. Interestingly, the serum of affected patients is capable of inducing fibroblast proliferation in vitro, suggesting that there may be an unknown relevant factor in the blood.[4] Some literature suggests that the disease is associated with viral infection.[5],[6] In this patient's examination, hepatitis B virus, hepatitis C virus, and HIV were negative.
Due to the rarity of the disease, there are no treatment guidelines; a variety of therapeutic drugs are used, with varying results. At present, intravenous immunoglobulin is considered first-line treatment,[3],[7] especially for patients with dermatoneuro syndrome. Thalidomide is also commonly used.[3],[7] Oral tretinoin, methotrexate, cyclophosphamide, psoralen-ultraviolet-A (PUVA), autologous stem-cell transplantation, plasmapheresis, dermabrasion plus surgery, and carbon dioxide laser therapy are also useful.[1],[3],[7]
This case suggests not only to pay attention to treatment and systemic involvement but also to ensure close and long-term follow-up.
Declaration of patient consent
The authors certify that they have obtained appropriate patient consent form. In the form, the patient has given his consent for the images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Data availability statement
Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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