Lipschütz ulcers are characterised by a first flare of non-sexually related acute genital ulcers (AGU) occurring in adolescent girls. Epstein-Barr primary infection is the most frequently reported aetiology but other infectious agents are probably implicated. We report the first case of mumps associated with an AGU in a 21-year-old girl. She presented a bilateral parotitis with genital ulcers, and serology confirmed she had mumps. As in our case, most Lipschütz ulcers heal spontaneously within a couple of weeks and the diagnosis should be reconsidered in case of recurrence.
with any of desmosomal and hemidesmosomal antigens mostly recognized by sera from major immuno-bullous diseases (Fig. 2b). Considering these series, however, she was diagnosed as PH, and oral administration of prednisolone (30 mg/day) and dapsone (75 mg/day) gave complete resolution of her skin lesions.PH represents a distinct disease entity harboring clinical and immunological features of dermatitis herpetiformis and pemphigus, respectively. 2 An intriguing question is why our case showed discrepant immunoreactivity between in vivo-bound and circulating autoantibodies. Possible explanations include that: (i) serum anti-Dsg antibody titer was too low to detect by conventional ELISA and immunoblotting; (ii) the accessibility and/or avidity of serum antibodies to in vivo native antigens differ from those to in vitro chemically processed antigens (i.e. baculovirus antigens or during immunoblotting procedure), particularly in PH. Thus, autoantibodies in sporadic PH cases may be very sensitive to the conformational epitope(s) or the potential antigenicity in each target antigen. Alternative interpretation for the negative results in the Dsg-specific ELISA is that antibodies to epidermal intercellular proteins other than Dsgs might be involved in our patient. Compared to well-established anti-Dsg imunno-bullous diseases pemphigus vulgaris or pemphigus foliaceus, 4-6 there has been no plausible evidence for the association between clinical presentations of PH and anti-Dsg antibodies. One may speculate that PH autoantibodies recognize a functionally less important part of Dsg molecules and are therefore unable to induce acantholysis. 3 These characteristic antibodies, however, may still be responsible for inflammatory processes via complement activation or cytokine release by the lesional keratinocytes and inflammatory infiltrates, 7 leading to the focal intercellular edema and eosinophilic spongiosis, a histopathology resembling PH. Collectively, the pathogenic antigen(s) in the minority of PH might not be restricted to Dsgs -such antigens are located in the keratinocyte cell surface -but it may be insufficient to induce acantholysis. Further investigation is required to understand the underlying immunopathogenic nature of this fascinating disease condition.
Epidermolysis bullosa acquisita is a rare autoimmune subepidermal blistering disease, often resisting current treatments, especially systemic corticosteroids. We report a patient having a bullous pemphigoid who relapsed with clinical and immunological features of inflammatory epidermolysis bullosa acquisita. An anti-CD20 monoclonal antibody (rituximab) was proposed because of resistance to high-dose steroids and other immunosuppressive agents. The disease dramatically improved within a few weeks following rituximab infusion allowing the decrease in steroid therapy. Our case illustrates also the possible evolution from bullous pemphigoid to epidermolysis bullosa acquisita that should be suspected when clinical atypia occurs or in case of corticosteroid resistance.
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