CLINICAL VIGNETTE

Marvin Berkowitz, M.D.

Case Report

A 41-year-old male presented with oral blisters in February 1996 and was diagnosed with pemphigus vulgaris. He was started on high dose steroid therapy and noted control of his oral lesions. He returned to the office after two years of high dose prednisone therapy when he began to experience pain in his left shoulder. He complained of a limp with right hip pain, particularly toward the end of the day when he grew tired. He also complained of stiffness in his right hip, particularly upon rising in the morning and upon exertion, such as rising from a chair. Anti-inflammatory medications were tried, but gave him gastrointestinal distress.

The patient presented with a rounded face, protuberant abdomen, and decreased musculature in the upper and lower extremities. He had no swelling, instability or locking. He walked with an abnormal gait on the right. Radiographs of the hips showed changes consistent with avascular necrosis, including joint space narrowing of approximately one millimeter at the right hip with no collapse of the femoral head. Segmented changes were evident in the right femoral head. The left shoulder showed some decreased joint space, and changes in the humeral head consistent with avascular necrosis. Also evident were some sclerotic changes and flattening of the humeral head.

In September 1998, the patient underwent decompression with cortical press-fit allograft of the right hip, in an effort to prevent progression and eventual collapse of the infarcted outer weight-bearing surface.

Six months following his core decompression hip procedure, the patient was still experiencing episodes of pain, as severe as seven on a 10-point scale. His left hip showed no evidence of avascular necrosis, and no evidence of collapse. The right hip showed no change in either the subchondral bone or the head of the femur, and no evidence of collapse. However, he continued to have pain in both shoulders, greater in the left than the right, with decreased range of motion. The left shoulder showed significant collapse and degeneration secondary to avascular necrosis. Radiographs showed collapse and fracture at the right humeral head.

In April 1999, he stopped taking calcium carbonate, and his joint pains diminished. He still experienced pain when extending the range of motion of either arm.

In January 2000, the patient presented to the emergency room with complaints of light-headedness and generalized weakness. He noted black stools, and his hemoglobin was six. Work-up revealed an upper gastrointestinal bleed secondary to an antral peptic ulcer. The patient\'s medications at this time were prednisone and naproxen.

The following month, MRI scans revealed advanced avascular necrosis of the right femoral head with some evidence of collapse as well as increased avascular necrosis of the left femoral head. The scans also showed degenerative osteoarthritis of both hips.

Discussion

Pemphigus vulgaris is an autoimmune disorder of the skin. This condition is found in higher incidence among patients of Jewish or Mediterranean descent, but does occur across all races. The disease may be genetically linked, or may be transmitted between individuals by unknown pathways. It can be fatal in some cases.

Prednisone is the drug most commonly prescribed to treat pemphigus vulgaris. Prior to the use of corticosteroids in the management of pemphigus vulgaris, the condition was commonly fatal. A study of 107 pemphigus patients seen during a 20-year period reported an overall mortality rate of 32%. Though corticosteroids are effective in the management of pemphigus vulgaris, the researchers reported that death in these cases frequently resulted from the complications of steroid therapy.¹

While steroids are widely used to treat inflammatory conditions, their long-term use can result in serious side effects such as liver and kidney damage, and muscle deterioration. Other side effects may include weight gain, glucose intolerance, cataracts, hypertension, acne, increased susceptibility to infections, bruising, insomnia, and avascular necrosis of bone. Steroids can also affect mood, so depression and mood swings are not uncommon for patients being treated for pemphigus vulgaris outbreaks. However, the most severe side effect of long-term steroid use is osteoporosis and/or necrosis of the major joints. This may necessitate surgery for joint replacement, and/or other surgeries to repair bones and joints.

The side effects of corticosteroids are mainly determined by two predictable factors: daily dosage and duration of treatment. While not all patients experience side effects from similar steroid treatment plans, side effects should be considered in decisions regarding use of these drugs, dosage, and duration of treatment.

Avascular necrosis of the bones is one of the most severe side effects of high dose corticosteroids for an extended time. While prednisone aids immensely in controlling skin eruptions associated with pemphigus vulgaris, the side effect of avascular necrosis is highly significant. For this reason, steroid sparing regimens using cytotoxic agents are being investigated.^2,3

REFERENCES

1. Rosenberg FR, Sanders S, Nelson CT.Pemphigus: a 20-year review of 107 patients treated with corticosteroids. Arch Dermatol. 1976 Jul;112(7):962-70.

2. Fleischli ME, Valek RH, Pandya AG. Pulse intravenous cyclophosphamide therapy in pemphigus. Arch Dermatol. 1999 Jan;135(1):57-61.
3. Pasricha JS, Khaitan BK, Raman RS, Chandra M. Dexamethasone-cyclophosphamide pulse therapy for pemphigus. Int J Dermatol. 1995 Dec;34(12):875-82.