Lithium and halogenoderma.
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Biomedical subjects
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Immunoblot assays have been developed to characterize the autoantigens and to detect autoantibodies in muco-cutaneous autoimmune vesiculo-bullous diseases using different substrates. However the results have been inconsistent, because availability and standardization of different substrates has been a major problem. The aim of this study was to develop an immunoblot assay using bovine gingival lysate as substrate because it is easily and readily available as well as inexpensive. Sera from patients with different vesiculo-bullous diseases were studied. These included 25 patients with pemphigus vulgaris (PV), 8 with paraneoplastic pemphigus (PNP), 12 with pemphigus foliaceus (PF), 25 with bullous pemphigoid (BP), and 22 with cicatricial pemphigoid (CP). Serum samples from 40 normal human volunteers were also studied. The autoantibody titers were determined based on the binding pattern of each disease and compared to those obtained by routine indirect immunofluorescence (IIF). Our observations suggest that the titers from immunoblot assays were significantly higher than titers obtained by IIF (P<0.0001). When the autoantibody titers were compared using bovine gingival lysate and human epidermal lysate as substrate, statistically significant differences were not observed. The use of bovine gingival lysate as a substrate will facilitate the rapid and early serological diagnosis of patients with vesiculobullous diseases. It may also be of benefit to laboratory investigators studying these autoantibodies.
Nail involvement in pemphigus vulgaris is relatively rare. We describe a case of severe pemphigus involving both the skin and oral mucosa in which an acute exacerbation was preceded by the onset of nail involvement of all 4 extremities. Nail involvement occurred in the form of hemorrhagic paronychia of multiple digits. Oral, cutaneous, and nail manifestations of the disease were all well controlled by systemic therapy. A review of the literature on nail involvement in pemphigus reveals that this involvement may be manifested in multiple ways, with chronic paronychia and onychomadesis being the most common. Involvement of the nail occurs most frequently either as part of the initial presentation, or just before or concurrent with a flare of pre-existing disease. Nail involvement, when it occurs, is usually present when the disease is severe. Topical therapy is insufficient, and systemic therapy is warranted. In the majority of cases, nail recovery is complete, with no residual damage.
Pemphigoid gestationis is a rare autoimmune vesiculobullous skin disease closely related to the pemphigoid group of blistering disorders. It is unique in that it is most frequently associated with pregnancy. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of the third component of complement along the basement membrane zone of perilesional skin. Abnormal expression of major histocompatibility complex class II molecules in the placenta may trigger the initiating immunologic event in this organ, which is followed by cross-reactivity with an antigen present in the skin and the characteristic cutaneous signs of the disease. The immunogenetics of this disorder are interesting. A role for the involvement of major histocompatibility complex class II antigens seems likely because of the association with human leukocyte antigens DR3 and DR4. Major histocompatibility complex class III associations have also been observed, as has a high frequency of anti-human leukocyte antigen antibodies. We review the current understanding of the molecular biologic and immunogenetic properties of this disease and discuss treatment and potential impact on the fetus.