In general, tapering of medications should be slow and methodical, to identify the lowest dose of medicine needed to control the disease and to minimize flares with tapering (e).
The list of complications from pemphigus and from its therapy is extensive and weight gain, anemia, hypertension, diabetes are not uncommon; secondary infections by candida (oral) and herpes simplex virus are also seen. (ddd)
- Corticosteroid management:
- Chronic Steroid Usage: Chronic dermatologic diseases such as pemphigus vulgaris, and bullous pemphigoid often require long-term therapy with oral corticosteroids. Such chronic systemic steroid use poses significant health concerns, including adrenal suppression, cushingoid morphology, hypertension, fluid retention, bone mineral density (BMD) decline, diabetes, ulcers, cataracts, glaucoma, increased susceptibility to infections, and reactivation of tuberculosis.
- Attempt to use the lowest successful dose of corticosteroids (see earlier)
- Consider early use of ‘steroid sparing’ medications (such as the immuno-suppressive medications)
- Consider medical monitoring (see section on diagnosis earlier), and other medical preventive measures (such as low salt and low sugar diets) in patient on steroids.
- Prevention of Osteoporosis: Thinning of the bones or osteoporosis is of particular concern because it silently and progressively increases the risk of pathologic bone fractures and there are ways to minimize this risk.
- Many dermatologists remain unaware that patients receiving long-term oral corticosteroid therapy should routinely be receiving concomitant bisphosphonate therapy (tt).
- These risks are increased in the elderly; a study of long-term corticosteroid use demonstrated that 28% of patients had at least 1 vertebral deformity and 11% had 2 or more vertebral deformities, with patients aged 70 to 79 having a 5-fold increased risk of deformity compared with patients younger than 60 years (ss).
- TheAmericanCollegeof Rheumatology (ACR) recommends the addition of bisphosphonate therapy for patients taking long-term (longer than 3 months) oral corticosteroids, at a dose of more than 5 mg prednisolone/day (or equivalent steroid) (uu). The one exception is for women of child-bearing potential who plan to have children.
- The ACR notes that “Bone mass measurement is reimbursed by Medicare for “patients receiving long-term glucocorticoid therapy at doses of 7.5 mg/day or greater”. The Committee recommends “obtaining a baseline measurement of bone mineral density (BMD) at the lumbar spine and/or hip when initiating long-term (i.e., >6 months) glucocorticoid therapy. Longitudinal measurements may be repeated as often as every 6 months for monitoring glucocorticoid-treated patients to detect bone loss. In patients who are receiving therapy to prevent bone loss, annual follow-up measurements are probably sufficient”
- Use of Dual energy x-ray absorptiometry (DEXA) can help determine fracture risk for patients taking glucocorticoid therapy and for those starting therapy (vv)
- The ACR also recommends supplementation with both calcium and vitamin D should be required for glucocorticoid-treated patients.
- Corticosteroids also suppress clinical signs of infection and may allow diseases such as septicemia or tuberculosis to reach an advanced stage before diagnosis, and are associated with osteoporosis, adrenal insufficiency, hypertension, hyperlipidemia, and hyperglycemia. In addition, myopathy, glaucoma, cataracts, stomach ulcers, weight gain, skin thinning, avascular necrosis of the hip, hirsutism, anxiety and depression and other side-effects have been documented.
Other medication complications include (not an exhaustive list) (ccc)
- Bone marrow suppression has been reported in patients receiving immunosuppressants, as has increased incidence of leukemia and lymphoma
- Secondary infection, which may be either systemic or localized to the skin, may occur because of the use of immunosuppressant and the presence of multiple erosions.
- Malignancies resulting from immunosuppressant have been reported.
- Growth retardation has been reported in children taking systemic corticosteroids and immunosuppressant.
- Immunosuppressants are also associated with myelosuppression, hepatitis, gastrointestinal disturbances including hemorrhage, impaired immune responsiveness, and hemorrhagic cystitis, bladder cancer and infertility (cyclosphosphamide), reduced kidney function and hypertension, and tremor (cyclosporin).
- Gold has been associated with rashes, mouth ulcers, nephrotic syndrome, and myelosuppression.
- Methotrexate has been associated with bone marrow suppression, hepatitis and cirrhosis, and pulmonary fibrosis.
- Laboratory monitoring of these drugs is required as specified by each drug’s manufacturer.