Complications
Often patients with severe pemphigus vulgaris (PV) or paraneoplastic pemphigus (PNP) are unable to eat or drink because of their mucositis and nutritional support may be required.[1]
In volume-depleted patients, supplementation with intravenous fluids is indicated.
The epidermal barrier of the mucosa and skin is compromised in pemphigus, resulting in an increased susceptibility to superficial skin infection with bacteria, yeasts, or viruses such as herpes simplex virus. With large areas of skin involved, infection via the skin can lead to sepsis. The risk of infection is increased when concurrent immunosuppression is present due to either immunosuppressive drugs or malignancy.
Appropriate wound care with emollients may suffice if pemphigus is aggressively controlled with oral drugs. Superficial skin infection is treated with antiseptic washes, antibiotics, antifungal drugs, or anti-herpetic agents such as aciclovir. Bathing in dilute chlorhexidine solution may benefit patients with extensive skin lesions. Aggressive debridement is not indicated because the dermis and portions of the epidermis are intact. This is in striking contrast to burn patients, who have significant involvement of the deep dermis.
Epidemiologic studies in the US have shown that patients with pemphigus admitted to inpatient care units have increased cutaneous, respiratory, multi-organ and systemic infections. Overall, inpatients with pemphigus had double the incidence of serious infection compared with inpatients without this diagnosis (50% vs. 25%).[55]
Mucositis can be painful, preventing normal ingestion of food and water.
Typically, topical lidocaine jelly is effective. Occasionally, patients will require analgesia with opioids. Implementing a soft, bland diet can facilitate oral intake in those with mucosal involvement.
Long-term immunosuppression can lead to an increased risk of actinic keratosis and cutaneous malignancies.
Patients are advised to avoid prolonged exposure to sun and to take appropriate precautions.
A scarring conjunctivitis is likely in patients with PNP only.
Osteoporosis is an adverse effect of long-term corticosteroid use. Therefore, the goal of treatment is to aggressively control disease and limit the cumulative long-term dose of corticosteroids. Early control of the disease process minimises the cumulative dose of corticosteroids.
Post-inflammatory hyperpigmentation or hypopigmentation is due to skin injury resulting from the blistering process. Post-inflammatory pigment change is more noticeable in brown and black skin (skin type V and VI) and may take up to one year to resolve. Patients should be reassured that, if skin inflammation is controlled, pigmentation will normalise within 6-12 months.
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