Treatment of recalcitrant generalized morphea with infliximab.

A 66-year-old white woman with a history of hypertension and degenerative arthritis was seen in July 2003 by her primary care physician (PCP) for erythematous and sclerotic patches on her trunk. A skin biopsy specimen taken by the PCP at that time was interpreted (by an outside pathology laboratory) to indicate granuloma annulare. The patient began treatment with systemic corticosteroids and ceased taking her antihypertensive medication. However, her cutaneous symptoms continued to progress, and she was referred to our clinic in January 2004. At that time, the patient denied any illnesses prior to her skin eruption and noted that the only change in her medications was from lisinopril to valsartan. A review of systems found no shortness of breath, dysphagia, worsening of chronic joint symptoms, or Raynaud phenomenon. The patient was noted to have extensive white sclerotic patches and a few eczematous inflammatory patches on the upper chest, breasts (excluding the areola), upper abdomen, flanks, and proximal extremities (Figure 1). Her skin was firm and sclerotic on palpation. No sclerodactyly was present. A skin biopsy specimen was obtained. Light microscopic examination revealed a striking pandermal sclerosing reaction. The collagen bundles were of wider caliber and oriented parallel to the long axis of the epidermis. In addition, the overlying epidermis was attenuated with prominent hyperkeratosis. The subjacent papillary dermis had a hyalinized appearance with vascular dropout. The findings were compatible with an overlap between morphea and lichen sclerosus et atrophicus (LS&A). In situ hybridization studies demonstrated focal staining of the endothelium for cytomegalovirus (CMV) and tumor necrosis factor (TNF) RNA transcript expression. Direct immunofluorescence showed deposits of C5b-9 within the microvasculature most compatible with a humorally mediated microangiopathy syndrome (Figure 2). Serologic studies revealed positive anti-CMV IgM and IgG. However, the findings of antinuclear antibody assays, extractable nuclear protein antibody tests, an interstitial lung battery, complete blood cell count, and comprehensive chemical analysis were all within normal limits, as was the erythrocyte sedimentation rate. The patient was also found to be very hypertensive and resumed taking antihypertensive medications along with class 1 topical corticosteroids. Figure 1. Eczematous inflammatory patches.