Prevention and Treatment of Infections in Patients with Autoimmune Blistering Disease

Fig. 65.1

Patient with pemphigus vulgaris treated with rituximab and intravenous corticosteroids. Superficial swabs revealed the presence of Proteus mirabilis, Pseudomonas aeruginosa, and Candida parapsilosis

Fig. 65.2

Patient with pemphigus vulgaris taking methotrexate (20 mg/week) and prednisone who developed superinfection with herpes simplex virus type 1, as confirmed with polymerase chain reaction (PCR)

Fig. 65.3

Patient with bullous pemphigoid treated with methotrexate (20 mg/week) and prednisone who developed superinfection with methicillin-sensitive Staphylococcus aureus

Not only does excess microbial growth inhibit cutaneous healing, but it can also create a portal of entry for systemic infection, a serious source of morbidity and mortality [4–10]. Many forms of bacterial, viral, fungal, and mycobacterial infections have been reported in patients with autoimmune blistering disease [4, 9, 10]. For example, Staphylococcus sp. commonly colonize and infect damaged skin. Serious complications, such as erysipelas, necrotizing fasciitis, and sepsis, have resulted from bacterial infection of the skin in patients with bullous pemphigoid [9]. Other systemic bacterial infections, such as nocardiosis, may occur [10]. Viruses such as herpes simplex virus can superinfect skin lesions in patients with AIBD and may go unrecognized [11, 12]. Patients being treated with immunosuppressive medications for AIBD also experience reactivation of herpes zoster virus (shingles) with greater frequency than their unaffected counterparts (Lehman and Kalaaji, unpublished data). A patient with pemphigus vulgaris developed Kaposi’s sarcoma, a low-grade vascular malignancy associated with human herpesvirus 8, at the site of intralesional corticosteroid treatments [13]. Patients with AIBD may develop superficial fungal infections, such as dermatophytosis or candidiasis, or serious systemic fungal infection by organisms such as Cryptococcus neoformans and Aspergillus sp. [14, 15]. Patients being treated with systemic immunosuppressive medications for AIBD may be at risk for potentially fatal Pneumocystis pneumonia [11, 12]. Localized or systemic mycobacterial infections or reactivation of latent mycobacterial infection may also occur [16–18].

65.2 Prevention of Infection in Patients with AIBD

Patients should try to take practical measures to reduce undue exposure to potentially pathogenic microorganisms [10]. Examples include avoidance of places with a high incidence of infection (e.g., hospitals, day-care centers, and nursing homes), environments with a high density of people or reduced air circulation (e.g., airplanes), or fomites (e.g., shared computer keyboards and telephones). Hence, if at all possible, it would be best if AIBD patients being immunosuppressed could be treated as outpatients. Regular hand washing, with particular attention paid to nail hygiene, is essential. Wearing of a face mask in crowded areas or when gardening may be considered. The companionship and psychosocial benefits of a pet should be weighed against the potential for infection by zoonotic organisms, such as Salmonella (reptiles), group A beta-hemolytic streptococcus (cats and dogs), Bartonella henselae (cats), Campylobacter jejuni (dogs, cats, and birds), Chlamydia psittaci (birds), and dermatophytes (cats and dogs). For immunosuppressed patients who wish to keep pets at home, helpful guidelines have been developed to minimize the chance for zoonotic infection [19].

Although specific immunization recommendations have not been published for patients with AIBD, those developed for immunocompromised patients in general indicate that patients should be brought up-to-date on age-appropriate immunizations where feasible [20, 21]. Although delivery of live attenuated vaccinations, such as the herpes zoster vaccine and the live form of the influenza vaccine, is contraindicated during active immunosuppression, these immunizations can be given up to 3 weeks prior to initiation of iatrogenic immunosuppression [22]. Since immunosuppressive medications can lead to reactivation of latent infectious hepatitis or tuberculosis, high-risk patients should be screened before such medications are initiated. For patients unable to receive the recommended vaccinations, it is particularly important that close contacts and family members be up to date with their immunizations to help protect the patient via the principle of “herd immunity.”

An important principle in infection prevention in these patients is reduction of surface colonization and restoration of the skin barrier. This can be achieved with diligent personal hygiene practices, meticulous skin care, and implementation of localized antiseptic measures, such as distilled white vinegar wet dressings, chlorhexidine gluconate solution, potassium permanganate solution (Fig. 65.4), and bleach baths [1, 23, 24]. Expert wound care consultation and home health nursing aides may be required for patients with refractory wounds or personal disability. Cleaning tense vesicles and bullae with either 70 % alcohol or sterile spray water/normal saline before gently deflating these with a sterile needle may prevent skin denudation from traumatic bullous rupture. The blister roofs should be left intact to function as a biologic barrier. The skin should not be scrubbed [2]. The use of bland emollients can hasten healing and protect the skin.

Fig. 65.4

Patient pictured in Fig. 65.1, being treated in medical whirlpool bath with potassium permanganate solution and aided by skilled wound care nurses

Patients using topical corticosteroid preparations in the mouth often benefit from concomitant prophylactic use of anti-candidal medications (such as clotrimazole troches). Other measures important in preventing infection include minimization of the dose and duration of immunosuppressive medications as well as smoking cessation and optimization of blood sugar levels and nutritional status. To prevent opportunistic infections, patients taking high doses or prolonged courses of immunosuppressive medications may benefit from the addition of Pneumocystis prophylaxis [25, 26]. In patients who have already experienced infectious complications, such as superinfection with Staphylococcus aureus or herpes simplex virus, chronic suppressive antimicrobial treatments could be considered as secondary prevention.

65.3 Management and Treatment of Infection in Patients with AIBD

Patients with AIBD should be educated about the signs and symptoms of localized or systemic infection and urged to present for medical attention promptly for early evaluation and intervention. Physicians should have a low threshold to culture slow-healing skin lesions, because accurate identification of the implicated organism may be essential for proper treatment. Since a common pitfall in the management of erosive AIBD is failure to recognize superinfection with herpesviruses [11, 12] or dermatophytes [27], skin swab for polymerase chain reaction (PCR) or fungal culture may be helpful.

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