Conditions that manifest by the presence of large bullae or skin vesicles filled with fluid.

The cause of blistering conditions may include: bacterial or viral infections, contact dermatitis, autoimmune or metabolic diseases; as well as blistering disorders associated with benign and malignant lymphoproliferative diseases.

Painful skin lesions called bullae are present. They usually contain clear fluid that may later become filled with purulent material. The lesions rupture easily because of a very thin covering. After rupture of the lesions, they may be difficult to recognize because only erosions, crusts, or thin pieces of the epidermal blister covering may remain.

History and physical examination may help diagnose the condition as being a bullous disorder. Biopsy of blisters in the early stage is imperative as a basis for further diagnosis. Pathologic studies are most helpful if they are done before therapy has been initiated. Immunofluorescence studies on biopsy material from skin lesions may differentiate certain immunologically-mediated disease conditions.

The goal is to stop eruptions and new lesions from developing. Systemic corticosteroids are the main treatment. Patients with severe disease may need to be hospitalized and given high doses of corticosteroids. If bullous conditions are not treated in an adequate manner, they can be fatal. Many persons will require maintenance steroid doses for months or years. Administration of immunosuppressants, such as azathioprine (Imuran) or cyclophosphamide (Cytoxan), alone or with steroids, may assist in reducing the steroid dose. Using silver sulfadiazine cream on the erosions may help prevent secondary infections.

The long-term use of steroid therapy and immunosuppressive drugs may have many undesirable side effects. The person with bullous disorders may suffer an altered body image due to the visible skin lesions, and require counseling. Secondary infection may follow rupture of the bullous lesions. Untreated bullous disorders, such as pemphigus vulgaris, may progress slowly causing extensive denudation, leading to fluid and electrolyte imbalance, sepsis, and death.

• Consider service connection on a presumptive basis as a condition associated with herbicide exposure (38 CFR §3.307(a)(6); §3.307(d); §3.309(e)) if porphyria cutanea tarda manifests to a degree of 10 percent or more within one year of the last date of exposure to herbicides.

• Rate complications and residuals of mucosal involvement (ocular, oral, gastrointestinal, respiratory, or genitourinary) separately under the appropriate diagnostic code.

• 38 CFR 4.118 (a) For the purposes of this section, systemic therapy is treatment that is administered through any route (orally, injection, suppository, intranasally) other than the skin, and topical therapy is treatment that is administered through the skin. 

• 38 CFR 4.118 (b) Two or more skin conditions may be combined in accordance with 38 CFR 4.25 only if separate areas of skin are involved. If two or more skin conditions involve the same area of skin, then only the highest evaluation shall be used.