Content » Vol 100, February

Short communication

Plaque-like Cutaneous Lupus Mucinosis as the First Sign of Systemic Lupus Erythematosus

Meng Gao, Nan Sheng, Xianhong Yang and Yiqun Jiang*

Institute of Dermatology and Hospital of Skin Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, No.12 Jiangwangmiao Street, Xuanwu District, Nanjing, Jiangsu, China. *E-mail: yiqunjiang2017@163.com

Accepted Nov 27, 2019; Epub ahead of print Nov 27, 2019

INTRODUCTION

Cutaneous lupus mucinosis (CLM) is an unusual skin manifestation of lupus erythematosus (LE), which commonly manifests as numerous papules or nodules on the neck or trunk (1). Since the first description of this condition in 1954, fewer than 50 cases have been reported in the English literature (2). Nearly all reported cases of CLM had a prior history or typical symptoms of systemic lupus erythematosus (SLE) before presentation. We report here a patient who experienced plaque-like CLM as the first sign of SLE.

CASE REPORT

A 29-year-old Chinese woman presented with massive plaques on the left shoulder and right upper arm with no other subjective signs. The lesions had gradually increased in size over the last 2 years. She had no significant past medical history or family history of autoimmune diseases. She denied photosensitivity, fever, oral ulcer, arthralgia and other discomfort. Physical examination showed well-defined, rubbery, skin-coloured to light erythematous plaques, approximately 5 cm in diameter, on the left scapular area and the right posterior arm (Fig. 1). Biopsies were taken from both lesions, and histopathological examination revealed significantly increased mucin, highlighted by Alcian Blue stain, in-between collagen bundles involving the entire dermis. Dense perivascular and periadnexal lymphocyte infiltration was also observed. The basement membrane zone was blurred, while the overlying epidermis was unremarkable (Fig. 2). Direct immunofluorescence revealed granular deposition of immunoglobulins IgG and IgM, and complement C3 at the dermoepidermal junction. Laboratory tests showed positive antinuclear antibody (ANA) (1:160), anti-Sm and weak positive anti-dsDNA. The serum IgG level was 17.5 g/l (normal range 8.0–17.0 g/l) and the C4 level was 0.15 g/l (normal range 0.17–0.4 g/l). Complete blood count, urinalysis, blood chemistry and thyroid function were normal. The patient was diagnosed with CLM and treated with topical injection of triamcinolone and oral hydroxychloroquine with satisfactory outcomes. We then lost contact with the patient. Four years later, her primary lesions reoccurred and some erythematous plaques developed in a “butterfly” distribution on her face. Laboratory tests revealed the following abnormal values: low leukocyte count in peripheral blood (3.9 × 109/l), elevated serum IgG level (23.9 g/l), low C4 level (0.07 g/l) and positive ANA (1:640), anti-Sm, anti-dsDNA, anti-Ro/SSA, anti-RNP and anti-C1q. Urinalysis, blood chemistry, electrocardiogram and chest film showed normal results. The diagnosis was modified to SLE and the patient was treated with oral methylprednisolone and oral hydroxychloroquine. At 2-month follow-up her skin lesions had alleviated and leukocyte count returned normal.


Fig. 1. Well-defined, rubbery, skin-coloured to light erythematous plaques, approximately 5 cm in diameter, on (a) right posterior arm and (b) left scapular area.


Fig. 2. (a, b) Significantly increased mucin in-between collagen bundles, dense perivascular and periadnexal lymphocyte infiltration in the dermis. (c) The basement membrane zone was blurred, while the overlying epidermis was unremarkable. (Haematoxylin and eosin, original magnification a: ×40, b, c: ×400). (d) Mucin deposition in the dermis (Alcian blue stain, original magnification: ×40).

DISCUSSION

CLM is an uncommon variant of skin manifestations of LE, which usually presents as multiple scattered papules or nodules on the trunk and upper extremities without discomfort. Histopathologically, CLM is featured by loosening of collagen fibres with striking mucin deposition in the whole dermis. Perivascular and periadnexal infiltration of lymphocytes can also be found in CLM, while typical epidermal and interface changes in LE are rare (3).

The atypical eruptions in the current case may cause diagnostic challenges. Clinically, it should be distingui-shed from acquired cutis laxa, connective tissue naevus, reticular erythematous mucinosis and mucinous naevus. Histopathologically, tumid lupus erythematosus (TLE) can be very similar to CLM. However, the TLE lesions usually appear as urticaria-like papules and plaques on sun-exposed areas, such as the face. Patients with TLE exhibit remarkable photosensitivity, and similar lesions can be reproduced by ultraviolet A/B (UVA/UVB) irradiation in 70% of cases. In addition, TLE is seldom related to SLE. The positive rate of serum ANA is only 10% (4).

CLM has a strong association with SLE. Of the 34 cases of CLM in the English medical literature, 26 had a history of SLE and 6 had demonstrated characteristic manifestations of LE, such as malar rash, discoid LE, and arthralgia prior to CLM presentation. The serum ANA test is positive in approximately 85% of cases (5–8). It is notable that, in the current case, CLM was the first clinical symptom of SLE. This condition indicates that CLM could be the initial manifestation of SLE. Therefore, it is important to follow up these patients for early signs or symptoms of systemic involvement. The pathogenesis of mucin accumulation in CLM remains unclear. Some studies have indicated that some serum factors in patients with SLE, such as autoantibodies and cytokines, may cause upregulation of glycosaminoglycans by fibroblasts (9, 10). In our patient, the CLM lesions reoccurred with progression of SLE, suggesting that the mucin deposition is associated with autoimmunity, which is consistent with previous research.

The most effective treatment for CLM lesions has not been confirmed. Due to the presence of SLE in most cases, systemic corticosteroid and hydroxychloroquine were administered with satisfying outcomes. Systemic corticosteroid and systemic tacrolimus combination therapy also showed efficacy in another case (11). In the current case the patient’s lesions responded well to combined therapy with corticosteroid intralesional injection and oral hydroxychloroquine in the early phase of the disease, offering another therapeutic regime for CLM.

ACKNOWLEDGEMENT

The authors thank Dr Lin Lin of the Buffalo Medical Group PC for critical review of this manuscript.

Source(s) of support: CAMS Innovation Fund for Medical Sciences (CIFMS-2017-I2M-1-017).

The authors have no conflicts of interest to declare.

REFERENCES
  1. Lowe L, Rapini RP, Golitz LE, Johnson TM. Papulonodular dermal mucinosis in lupus erythematosus. J Am Acad Dermatol 1992; 27: 312–315.
    View article    Google Scholar
  2. Gold SC. An unusual papular eruption associated with lupus erythematosus. Br J Dermatol 1954; 66: 429–433.
    View article    Google Scholar
  3. Kano Y, Sagawa Y, Yagita A, Nagashima M. Nodular cutaneous lupus mucinosis: report of a case and review of previously reported cases. Cutis 1996; 57: 441–444.
    View article    Google Scholar
  4. Nishiyama M, Kanazawa N, Hiroi A, Furukawa F. Lupus erythematosus tumidus in Japan: a case report and a review of the literature. Mod Rheumatol 2009; 19: 567–572.
    View article    Google Scholar
  5. Elkeeb L, Spicknall KE, Mutasim DF. Nodular cutaneous mucinosis associated with systemic lupus erythematosus. Int J Dermatol 2014; 53: 1389–1391.
    View article    Google Scholar
  6. Lee WJ, Park GH, Chang SE, Lee MW, Choi JH, Moon KC, et al. Papular mucinosis associated with systemic lupus erythematosus. Ann Dermatol 2008; 20: 233–236.
    View article    Google Scholar
  7. Shekari AM, Ghiasi M, Ghasemi E, Kani ZA. Papulonodular mucinosis indicating systemic lupus erythematosus. Clin Exp Dermatol 2009; 34: e558–560.
    View article    Google Scholar
  8. Su X, Qiao X, Li J, Gao L, Wang C, Wang L. Papulonodular mucinosis, Guillain-Barre syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report. BMC Nephrol 2017; 18: 43.
    View article    Google Scholar
  9. Pandya AG, Sontheimer RD, Cockerell CJ, Takashima A, Piepkorn M. Papulonodular mucinosis associated with systemic lupus erythematosus: possible mechanisms of increased glycosaminoglycan accumulation. J Am Acad Dermatol 1995; 32: 199–205.
    View article    Google Scholar
  10. Johnson WC, Graham JH. Ground substance and fibrinoid changes in cutaneous lupus erythematosus. J Histochem Cytochem 1966; 14: 827–829.
    View article    Google Scholar
  11. Sugiura K, Tanahashi K, Muro Y, Akiyama M. Cutaneous lupus mucinosis successfully treated with systemic corticosteroid and systemic tacrolimus combination therapy. J Am Acad Dermatol 2013; 69: e200–202.
    View article    Google Scholar