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Coexistence of extensive calcification and membranocystic changes in lupus erythematosus panniculitis associated with systemic lupus erythematosus

European Journal of Dermatology. Volume 17, Number 1, 86-8, January-February 2007, Clinical report

DOI : 10.1684/ejd.2007.0194

Summary

Author(s) : Takane Suda, Hiroyuki Hara, Tomoyoshi Okada, Hiroyuki Suzuki , Department of Dermatology, Nihon University School of Medicine, 30-1 Oyaguchi-kamimachi, Itabashi-ku, Tokyo, 173-8610, Japan.

Summary : We report a case of lupus erythematosus panniculitis with long-standing systemic lupus erythematosus. The patient developed widespread calcification and membranocystic changes in the subcutaneous tissue. There have been no reports of the coexistence of calcification and membranocystic changes in the literature. The mechanism for the coexistence of the calcification and membranocystic changes might be due to the degenerative changes in fat cells from local circulatory disturbance.

Keywords : lupus erythematosus panniculitis (LEP), systemic lupus erythematosus (SLE), calcification, membranocystic changes

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ARTICLE

Auteur(s) : Takane Suda, Hiroyuki Hara, Tomoyoshi Okada, Hiroyuki Suzuki

Department of Dermatology, Nihon University School of Medicine, 30-1 Oyaguchi-kamimachi, Itabashi-ku, Tokyo, 173-8610, Japan

accepté le 18 Août 2006

The membranocystic change in fat tissue was first described by Nasu, as membranous lipodystrophy [1]. Membranocystic changes have also been observed in collagen diseases, such as dermatomyositis, Systemic lupus erythematosus (SLE) and lupus erythematosus panniculitis (LEP) [2]. The calcification of soft tissues may also be a manifestation of connective tissue, such as systemic sclerosis, dermatomyositis and has been rarely documented in patients with SLE [3]. However, the coexistence of calcification and membranocystic changes in the subcutaneous tissue has not been reported in the English literature. Herein, we report a case of LEP associated with SLE. Histopathological findings revealed the coexistence of membranocystic changes and calcification in the subcutaneous tissue.

Case report

A 46-year-old Japanese woman, who had a history of SLE fulfilling the American Rheumatism Association criteria, initially presented 8 years ago with a complaint of photosensitivity, Raynaud’s phenomenon, arthralgias, malar rash, ulcers of the oral mucosa and fever. She responded to prednisolone. In 2002, the patient had a flare-up of the cutaneous lesions of the face. Small subcutaneous, hard nodules appeared on her bilateral breasts. The nodules on her breasts became bigger and then skin ulcers developed. Subsequently, new hard nodules developed on her lower extremity, and buttocks. Physical examination revealed that large, bony-hard plate-like subcutaneous masses measuring 5 × 7 cm and 8 × 10 cm in diameter on her left and right breasts, respectively ( (figure 1) ), 10 × 10 cm on the abdomen, 4 × 5 cm on the left leg, and 2 × 2 cm to 4 × 4 cm on the buttocks. In addition, punched-out skin ulcers were observed on the lesions of her both breasts ( (figure 1) ) and left leg. A discharge of white chalky debris could be seen through the ulcers. Atrophic plaques with peripheral erythema and scarred centers were also seen on the arms and back. Many reticular erythemas could be seen on the upper arms and trunk ( (figure 2) ). Neurological examination findings were normal.

Radiographs showed widespread and large amounts of sand-like calcifications on the subcutaneous tissue of the breasts, left leg and the buttocks.

The antinuclear antibody (ANA) test was positive in a titer of 1:1280, with a peripheral pattern. Anti-double strand DNA and anti-single strand DNA were positive in titer of 1:280 and 1:206, respectively. Tests for anti-SS-A, anti-SS-B, anti-Scl 70, and anti-RNP antibodies were all negative. Her serum calcium level and phosphorous level, corrected for the serum albumin level, were within normal limits, respectively. Creatine kinase, C-terminal parathyroid hormone, calcitonin, alkaline phosphatase, and 1,25 dihydroxycholecalcifererol levels were also within normal ranges.

Biopsy specimens were taken from the breast and the buttock. Histopathologic examination showed an atrophic epidermis and degeneration of the basal layer. Mild perivascular inflammatory infiltrates were present in the dermis and the subcutaneous tissue. Peculiar membranocystic lesions, with an arabesque-like appearance, were observed in the subcutaneous tissue ( (figure 3) ). In the septae of the fat tissue, there was basophilic degeneration in which the presence of calcium was confirmed by von Kossa’s stain. The calcification was mostly found in the periphery of the membranocystic structure ( (figure 4) ). Extensive hyalinized collagen fibers and mucin deposition were observed in the dermis and the subcutaneous tissue. Mucin deposition was prominent in the septae of the fat tissue. Direct immunofluorescence specimens demonstrated no deposition of immunoglobulins or complement.

Discussion

A case of lupus erythematosus panniculitis (LEP) in a patient with long-standing systemic lupus erythematosus (SLE) was reported. LEP may occur on its own or in association with discoid lupus erythematosus (DLE) or SLE. Histopathologic findings revealed that the calcification was co-localized with the membranocystic changes in the subcutaneous tissue.

Although the membranocytic changes and the calcification are pathologic conditions of the subcutaneous tissue, this is the first report of the coexistence of these conditions.

The membranocystic structure consists of multiple cysts lined by anuclear fibrillar or tubular materials forming an arabesque-like appearance [1]. Membranocystic changes have been reported in patients with dermatomyositis, lupus panniculitis, discoid lupus erythematosus, erthema nodosum, Weber-Christian disease, and stasis dermatitis [2]. Furthermore, membranocystic changes have been reported in patients with ischemic necrosis of the legs secondary to chronic arterial obstruction [4] and chronic panniculitis caused by venous stasis [5]. Rutishauser et al. also experimentally produced similar membranous structures in the bone marrow fat secondary to ischemia [6]. Because membranocystic changes have been observed in 21% of the fat necrosis cases, membranocystic changes are regarded as the result of ischemic injury of the fat tissue [5]. Meanwhile, histopathological examination of the ulcer revealed extensive ischemic necrosis of the dermis and subcutaneous tissue without calcification in dermatomyositis [7].

Compared to the calcification in systemic sclerosis, which is generally small (up to fingertip size), the calcification in SLE tends to be a large and plate-like mass [3, 8, 9]. Analysis of the SLE cases reveals that soft-tissue calcification occurred in patients with a long-standing disease and that the subcutaneous calcification had occurred at the sites of tissue injury due to vasculitis or panniculitis [10]. Although systemic steroids have never been directly implicated in the formation of soft tissue calcification [11], the influence of systemic steroids have been postulated. Powell speculated that local tissue ischemia could be produced through a pressure phenomenon caused by steroid-induced hypertrophied fat cells [12].

The precise pathogenesis of the coexistence of membranocystic changes and calcification remains unknown. Taken together, both conditions may occur from the degenerative process of subcutaneous fat cells.

In summary, we demonstrated both membranocystic changes and calcification in the subcutaneous tissue in a case of LEP associated with SLE. Calcium deposition was observed around the wall of the membranocystic structures. Based on these facts, it is suggested that the coexistence of calcification and membranocystic changes is due to degenerative changes in fat cells from local circulatory disturbance. The coexistence of the membranocystic changes and the calcification might have been overlooked or unrecognized in the past.

Acknowledgements

Financial support: None. Conflict of interest: None.

References

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