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Morphea with discoid lupus erythematosus

  • Author(s): Mir, Adnan
  • Tlougan, Brook
  • O'Reilly, Kathryn
  • Tzu, Julia
  • Meehan, Shane
  • Kamino, Hideko
  • Jr, Andrew G Franks
  • et al.
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Morphea with discoid lupus erythematosus
Adnan Mir MD PhD, Brook Tlougan MD, Kathryn O’Reilly MD PhD, Julia Tzu MD, Shane Meehan MD, Hideko Kamino MD, Andrew G Franks Jr MD
Dermatology Online Journal 17 (10): 10

Department of Dermatology, New York University, New York, New York

Abstract

The presence of lupus erythematosus with morphea in the same patient has rarely been reported. In this case, we describe a woman with the overlap of discoid lupus erythematosus with superficial morphea, diagnoses that are supported by histopathologic features and laboratory studies.



History

A 20-year-old woman presented to the Charles C. Harris Skin and Cancer Pavilion for evaluation of a spreading, scaly, tight, hyper- and hypopigmented plaque on her right upper arm. The lesion started out the size of a grape on her mid-upper arm and over the course of the next six months, spread to cover an area from her neck to her antecubital fossa. The plaque was pruritic and she felt pins and needles when exposed to heat. Additionally, her range of motion was limited at the shoulder, owing to tightness of the skin. She was initially treated with a two-month course of doxycycline without improvement and subsequently with chloroquine, which she discontinued because of side effects. She also was treated locally with intralesional and topical glucocorticoids. Although there have been some short periods without further spread, the lesion continues to progress. Throughout her course, the patient has denied fatigue, weight loss, shortness of breath, and all other systemic symptoms. Her decreased range of motion has been successfully addressed through physical therapy.


Physical examination


Figure 1Figure 2

Figure 3Figure 4

Upon initial presentation, there was a hyper- and hypopigmented, atrophic plaque with prominent follicular plugs over the shoulder. Eight months later, the large, hyperpigmented plaque with atrophy had progressed and covered the right shoulder and extended from the base of the neck anteriorly down the arm to the antecubital fossa, there was a large, hyperpigmented plaque with atrophy.


Laboratory data

A complete blood count and chemistry profile were normal. Rheumatoid factor, anti-RNP antibody, and anti-Sm antibody were negative. Antinuclear antibody was positive with titer of 1:80 and speckled pattern Anti-dsDNA antibody was positive at 97 (NL≤75). Lyme IgM antibody titer was 2 (NL<0.91), and Lyme western blot detected a single IgM band.


Histopathology


Figure 5

There is sclerosis of the superficial dermis that is associated with epidermal atrophy, hypergranulosis, and compact orthokeratosis. There are interface changes and follicular plugs. A Verhoeff-van Gieson stain shows elongated elastic fibers between collagen bundles, and there is decreased expression of CD34. There are increased deposits of connective-tissue mucin as highlighted by a colloidal iron stain.


Discussion

The occurrence of morphea rarely has been reported in patients with lupus erythematosus. In each of the reported cases, however, the lesions of morphea were distinct from the lesions of cutaneous lupus [1-5]. In two of these cases, the patients had systemic lupus erythematosus (SLE) and developed linear morphea of the face, four years and six months after the initial diagnosis of SLE, respectively [1, 2]. Two more reports described patients with long histories of morphea who subsequently developed joint pains, a malar eruption, and laboratory evidence of SLE [3, 4]. A fifth report described an eight-year-old girl who presented with morphea but no other symptoms and was found to have co-existent SLE [5].

In none of the cases described above, however, do the authors report the coexistence of cutaneous features of lupus erythematosus and morphea within the same lesion. Our patient developed such a lesion that enlarged over the course of several months to cover a large part of her right upper extremity. The histopathologic features support a diagnosis of morphea with overlap of discoid lupus erythematosus (DLE) and the laboratory data suggest a diagnosis of SLE.

One other possibility to consider in this case is the diagnosis of Lyme borreliosis with features of discoid lupus erythematosus. The spectrum of Lyme borreliosis ranges from erythema chronicum migrans to acrodermatitis chronica atrophicans [6]; there is a growing body of evidence that in some cases, morphea is linked to Lyme borreliosis [7]. Although the patient does not report a history of tick bite, the presence of Lyme IgM in the serum suggests exposure. However, owing to the circulating anti-dsDNA antibodies and the fact that her plaque continued to spread despite three-weeks of therapy with doxycycline, the diagnosis of cutaneous lupus and morphea overlap is favored. Although this interpretation does not preclude the possibility that exposure to Borrelia burgdorferi potentiated the development of morphea-like features in this case, the liklihood of a direct relationship is low. The possibility that Borrelia infection can alter the skin lesions of patients with SLE is an interesting one that requires further study.

References

1. Lee CW, et al. Linear scleroderma occurring in a patient with systemic lupus erythematosus. J Kor Med Sci 1994; 9:197 [PubMed]

2. Yo JY, et al. Multifocal lesions of morphoea in a patient with systemic lupus erythematosus. Clin Exp Dermatol 2009; 34:676 [PubMed]

3. Kleiner-Baumgarten A, et al. Linear scleroderma, hemiatrophy, and systemic lupus erythematosus. J Rheumatology 1989; 16:1141 [PubMed]

4. Kahana, M, et al. Appearance of lupus erythematosus in a patient with lichen sclerosus et atrophicus of the elbows. J Am Acad Dermatol 1985; 12:127 [PubMed]

5. Mitchell AJ, et al. Circumscribed scleroderma with immunologic evidence of systemic lupus erythematosus. Arch Dermatol 1980; 116:69 [PubMed]

6. Mullegger RR, Glatz M. Skin manifestations of lyme borreliosis: diagnosis and management. Am J Clin Dermatol 2008; 9:355 [PubMed]

7. Weide B, et al. Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol 2000; 142:636 [PubMed]

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