Dermatology Online Journal

Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus (lupus ab-igne)
Emily Berger MD, Maria Robinson MD, Rishi Patel MD, Andrew G Franks Jr MD
Dermatology Online Journal 18 (12): 17

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

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Abstract

A 63-year-old woman with a ten-year history of systemic lupus erythematosus presented with a three-year history of a large, pruritic lesion that was located on the back and a pruritic lesion that was located on the right thumb. She was a baker by profession for many years during which time she often stood with her back to a hot oven. Physical examination of the lower back showed a large, atrophic, and reticulated plaque with a hyperpigmented border. On the right thumb was a well-demarcated, erythematous plaque with adjacent loss of the medial nail plate on the affected finger. Histopathology of both skin lesions showed cutaneous lupus erythematosus. This patient displays the Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus. We propose the term lupus ab-igne to describe her skin lesions. There are a few reports of the Koebner phenomenon occurring in patients with lupus erythematosus in response to contact dermatitis, tattoos, zoster, operative scars, scratching, or pressure from clothing. To our knowledge, this is the first report of heat as the physical factor leading to the formation of cutaneous lupus lesions.

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History

A 63-year-old woman with a ten-year history of systemic lupus erythematosus (class V lupus nephritis, photosensitivity, malar rash, oral ulcers, anemia, lymphopenia) presented to the Dermatology Clinic at Bellevue Hospital Center with a three-year history of a large, pruritic lesion that was located on the back and a pruritic lesion that was located on the right thumb. She was a baker by profession for many years during which time she often stood with her back to a hot oven. While cooking daily, she stirs food in pots and pans with her right hand. Review of symptoms included Raynaud phenomenon. The skin lesions continued to develop while the patient was taking systemic glucocorticoids and have since responded symptomatically to antimalarial therapy.

Physical examination

Figure 1	Figure 2

On the midline lower back is a large, atrophic and focally reticulated plaque with hyperpigmentation that is greatest at the periphery. On the right thumb is a well-demarcated, erythematous plaque with adjacent loss of the medial nail plate on the affected finger.

Laboratory data

A complete blood count, serum chemistry profile, liver function tests, C4, C3, and urinalysis were normal. Double stranded DNA antibody was negative. Antinuclear antibody was positive with a titer of 1:1280 in a speckled pattern.

Histopathology

Figure 3

There is a perivascular and somewhat band-like infiltrate comprised predominantly of lymphocytes with occasional plasma cells. Some lymphocytes extend to the overlying hyperplastic epidermis where there is vacuolar alteration at the dermoepidermal junction, scattered necrotic keratinocytes, parakeratosis, and follicular plugging. A periodic acid-Schiff stain shows focal basement membrane thickening.

Discussion

Chronic cutaneous lupus, the most common form of which is discoid lupus erythematosus (DLE), typically occurs above the neck. Lesions are oftentimes photo-distributed because photosensitivity may have a prominent role in the pathogenesis of cutaneous lupus. Typical skin lesions of DLE are indurated and erythematous plaques with scale, atrophy, follicular plugs, and dyspigmentation. Our patient has systemic lupus erythematosus (SLE), which develops in between 5 percent and 10 percent of patients with DLE [1]. Histopathologic features of DLE are characteristic. Microscopic examination of a well-developed lesion shows follicular dilatation with keratin plugs, liquefactive degeneration of the basal layer of the epidermis, basement-membrane thickening, and a superficial and deep (characteristically periadnexal) lymphocytic infiltrate [2].

The isomorphic response, which also is known as the Koebner phenomenon, refers to the induction of inflammatory skin lesions in response to skin injury that occurs in several dermatologic conditions, most commonly psoriasis and lichen planus. The mechanism for this response likely involves recruitment of inflammatory cells to the site of injury that eventually include those cells specific to a patient’s cutaneous disease (e.g., T-cells in the case of psoriasis). These cells, in turn, contribute to the formation of a skin lesion typical for that patient’s condition [3].

Few cases of the Koebner phenomenon occurring in patients with lupus erythematosus (most commonly DLE) have been reported [3-9]. In these reports skin lesions of lupus have occurred in response to contact dermatitis, tattoos, zoster, and operative scars as well as to innocuous stimuli, such as scratching or pressure from clothing. It also has been proposed that, in addition to inflammatory pathways, cellular repair mechanisms are involved in the pathogenesis of the Koebner phenomenon in lupus [4]. It is reasonable to expect that the Koebner phenomenon in response to physical factors could occur in lupus, owing to the prevalence of photosensitivity in patients with lupus. Prior authors did not observe a Koebner phenomenon in patients with lupus who were taking systemic glucocorticoids [4].

Erythema ab-igne describes the pattern of the skin’s reaction to low-grade, chronic heat exposure at a level below that which causes a burn. The histopathologic features of erythema ab-igne include atrophy of the epidermis in addition to vasodilation and the dermal deposition of melanin and hemosiderin. In older lesions there may be basal layer vacuolar change/interface changes. Various etiologic heat sources have been described, which include, but are not limited to, types of open stoves (the first reports of erythema ab-igne), electric heaters or radiators, heating pads or blankets, hot water bottles, and laptop computers [10].

We propose the term lupus ab-igne to describe our patient’s DLE lesions, the origin of which is reminiscent of erythema ab-igne. The histopathologic features and clinical appearance are consistent with DLE. Notably, our patient’s DLE lesions, most prominently the large plaque on her back, may be explained by chronic exposure to heat received while standing with her back facing a hot oven for many hours during many years as a baker. Despite the reports of a Koebner phenomenon noted above, there has not yet been a report of heat as the physical factor that leads to the formation of cutaneous lupus lesions. This case thus provides interesting insight into the potential role of skin injury in the pathogenesis of cutaneous lupus.

References

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3. Ueki H. Koebner phenomenon in lupus erythematosus with special consideration of clinical findings. Autoimmun Rev 2005;4:219 [PubMed]

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5. Longhi BS, et al. Koebner’s phenomenon in systemic lupus erythematosus. Rheumatol Int 2011 Mar 25. (Epub ahead of print) [PubMed]

6. Lukacs A, et al. Nickel-induced Koebner phenomenon in chronic cutaneous lupus erythematosus. J Dtsch Dermatol Ges 2011;9:475 [PubMed]

7. Jolly M. Discoid lupus erythematosus after tattoo: Koebner phenomenon. Arthritis Rheum 2005;53:627 [PubMed]

8. Barnett JH. Discoid lupus erythematosus exacerbated by contact dermatitis. Cutis 1990;46:430 [PubMed]

9. Abdel-Aal H, Abdel-Aal MA. Koebner phenomenon and unusual manifestation of discoid lupus erythematosus (a case report). J Egypt Med Assoc 1972;55:704 [PubMed]

10. Smith ML.: Environmental and sports-related diseases. In: Bolognia J, et al., eds. Dermatology. 2nd edition, London: Mosby, 2008:1355

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