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Treatment:  All aphthous ulcers  respond  skin involvement. Any mucous membrane  Treatment: This disease can only be treat-
        well to steroids. Topical steroids are more  sites can be affected, including oral, genital,  ed palliatively. It cannot be cured as it is
        effective on minor aphthous lesions. They  ocular, and nasopharyngeal. Of significance,  an autoimmune disease. Most patients with
        can also be treated  with ‘magic’ mouth  85% of patients present with oral lesions,  limited  disease can effectively  be treated
        rinses as well as topical mid-level steroids.  often the initial manifestation of the disease,  with corticosteroids  (topical  or systemic)
        Minor aphthous ulcers will resolve on their  and 65% present with ocular lesions. There   with  immunosuppressant  medication  as
        own without scarring, however treatment  is a special concern with ocular involvement   needed. To maintain their oral health, it is
        reduces the length of time and possibly the  as eye lesions can lead to corneal desquama-  recommended to gently brush with a soft
        severity of the lesions. The biggest concern  tion and conjunctival adhesions, known as   toothbrush, use nonalcoholic based mouth
        with patients on continuous doses of ste-  symblepharon, which can result in scarring   rinses and have frequent  dental hygiene
        roids is the possibility of adrenal suppres-  and the potential for subsequent blindness.   treatments. Remember that an eye referral
        sion, therefore topical  steroids should be  As such, all patients with biopsy confirmed   is always mandatory for patients with a di-
        used whenever possible.              mucous membrane pemphigoid must have a
                                             referral for an eye exam.            agnosis of mucous membrane pemphigoid.
        Comparison of Herpes Simplex Virus vs.
        Aphthous Ulcers                      Clinical: Mucous membrane pemphigoid is   Pemphigus Vulgaris
                                             considered to be a chronic and progressive
        Secondary HSV1 lesions present with one  disease. Patients often present with only oral   Etiology: Pemphigus vulgaris is a muco-
        of two clinical manifestations. They present  lesions and the gingiva is the most common   cutaneous,  potentially  fatal,  autoimmune
        either as herpes labialis, also referred to as  site of occurrence followed by the buccal   disease. There are many types of pemphi-
        a cold sore or fever blister, or intraorally as  mucosa. The gingiva presents as a desqua-  gus, however, pemphigus  vulgaris  is the
        ulcers on the attached alveolar mucosa, ke-  mative gingivitis with a positive Nikolsky   most common type and 80% of patients
        ratinized gingiva and hard palate, referred  sign, the ability to create a blister with gen-  with pemphigus have pemphigus vulgaris.
        to as herpes recurrans. In healthy people, in-  tile rubbing. Scarring rarely develops with   A biopsy is indicated to confirm the diag-
        traoral herpetic lesions never present on the  oral lesions. Confirmation of the diagnosis   nosis of pemphigus vulgaris and blood tests
        movable mucosa: cheeks, labial mucosa,  is through a biopsy and direct immunohis-  along with both indirect and direct immu-
        buccal mucosa, soft palate, floor of mouth  tochemistry looking for a positive linear  nofluorescence can be used to confirm the
        and underside of the tongue. Therefore, the  epithelial immune deposit at the basement  diagnosis.  Indirect  immunofluorescence
        differential diagnosis of an unknown ulcer-  membrane zone of the epithelium. The sero-  identifies  circulating  pathogenic  IgG  an-
        ation presenting on the intraoral attached  logic detection of autoantibodies by indirect  tibodies against desmoglien 3. In essence,
        non-movable mucosa is by default auto-  immunofluorescence is not always present  the body is attacking the glue, desmoglien
        matically reduced to either an iatrogenic or  in patients with mucous membrane pemphi-  3, which holds the epithelial cells togeth-
        traumatic ulceration or a secondary herpetic  goid and the disease can be diagnosed on  er. This produces a very fragile and easily
        lesion. In the same fashion, intraoral ulcers  biopsy alone (Figures 21, 22).  ruptured  intraepithelial  blister. Before  the
        that occur on the movable mucosa will nev-                                advent of corticosteroids and immunosup-
        er be herpetic in origin; they will either be                             pressant medications,  pemphigus vulgaris
        minor or traumatic ulcers. Although most                                  was fatal in about 90% of all cases. Patients
        clinicians are comfortable with diagnosing                                would die like burn victims die, from flu-
        herpes labialis, the intraoral manifestations
        of secondary herpetic lesions are often mis-                              id loss, dehydration, electrolyte imbalance
        diagnosed. This is especially true in cases                               and subsequent kidney failure.
        where blisters or vesicles were not present                               Clinical: Oral lesions are extremely com-
        when the patient reported initially. It is im-
        portant to remember that clinically this lack                             mon in early manifestations of pemphigus
        of vesicles does not preclude the diagnosis                               vulgaris. Patients typically develop gingi-
        of a secondary herpetic lesion.                                           val lesions followed by lesions of the buc-
                                                                                  cal mucosa. The oral lesions run a chron-
        Mucous Membrane Pemphigoid vs.                                            ic course, causing fragile intraepithelial
        Pemphigus Vulgaris                                                        blisters, which rupture easily  resulting  in
                                                                                  superficial  erosions  and  ulcers.  Gingival
        Mucous Membrane Pemphigoid                                                lesions present as a desquamative gingivi-
                                                Figures 21 and 22. Mucous membrane   tis with a positive Nikolsky sign, but the
        Etiology: Mucous membrane pemphigoid,   pemphigoid.                       blisters are extremely fragile and transient.
        also referred to as cicatricial pemphigoid,                               Biopsying the affected mucosa is difficult
        is an autoimmune blistering disease. Clini-                               as it is quite fragile and falls apart easily,
        cally it presents as sub-epithelial tense fluid   Age/Sex/Race:  Mucous membrane  pem-  like wet tissue paper. Patients have difficul-
        filled blisters at the dermal–epidermal junc-  phigoid tends to present in the elderly pop-  ty maintaining good oral hygiene, which,
        tion.  The blisters result from the patient’s   ulation with a female predominance. It is  in turn makes the lesions more active, and
        autoantigen attacking the basement mem-  rarely seen in children. It is frequently seen  brushing their teeth or using a removable
        brane zone antigen. It is a mucocutaneous   in patients of Ashkenazi Jewish descent.
        disease which can affect single or multiple                               denture  can  occasionally  desquamate  the
        mucous membrane sites, with or without                                    entire surface epithelium. Skin lesions tend


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