The sera of 263 patients with bullous pemphigoid (BP) were tested by indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblot (IB) assay, in order to assess the diagnostic sensitivity of these techniques. Among the 263 sera tested, 198 sera (75%) contained antibasement membrane zone antibodies demonstrable by IIF reacting to the epidermal (98%) or both the dermal and epidermal sides (2%) of SSS. One hundred and eighty-two of the 263 sera (69%) reacted by IB with BP antigens (Ag), most commonly the BPAg1 (93 cases, 51%), and a complex of BPAg1 and the 180 kDa minor BP antigen (BPAg2) (47 cases, 26%). BPAg2 alone was found in 42 cases (23%). A good correlation was found between the detection of autoantibodies by IIF and labelling of BPAg1 and/or BPAg2 by IB assay, in which 152 of 198 sera with an epidermal pattern in IIF identified a BP antigen. IB analysis of the 65 sera negative by IIF yielded positive results in 30 cases (46%). Thirty-one percent (13 of 42) of sera recognizing by IB BPAg2, were negative by IIF, as compared with 12% (11 of 93) of those recognizing BPAg1 (P < 0.01). Comparing the sensitivity of the two tests, IIF (75%) was found to be more sensitive than IB (69%). Thirty-five of the 263 sera (13%) remained negative by both techniques. It can be concluded from this study that IIF on SSS appears to be a sensitive and reliable assay for screening BP; IB should be performed for the sera that are negative by IIF as it may reveal circulating antibodies, particularly to BPAg2.
Summary We report the case of a 50‐year‐old male homosexual suffering from AIDS, who developed diffuse annular hyperkeratotic lesions on the arms and legs. Histopathological examination revealed typical features of porokeratosis, which clinically was of the disseminated superficial type. Ultrastructural examination showed a paucity of keratohyalin granules and lamellar bodies. Immunohistochemical studies showed an almost complete absence of Langerhans cells in lesional epidermis. Involucrin and filaggrin expression were altered in areas of cornoid lamella formation, whereas basal keratinocytes in these areas expressed PCNA/cyclin and, to a lesser degree, p53 protein. Porokeratosis may affect immunocompetent patients, but has also been reported in the setting of immunosuppression following organ transplantation. As far as we are aware, the development of porokeratosis during the course of HIV infection has not been reported previously.
We report changes in the antigen recognition pattern of sera from two pemphigus foliaceus patients with a long-term follow-up. The patients' sera were analysed by immunoblotting using different antigenic sources: cultured human keratinocytes, bovine tongue epithelium and a recombinant protein corresponding to the C-terminal end of the 230-kDa bullous pemphigoid antigen. While initial serum samples reacted exclusively with the 160-kDa desmoglein 1, the later sera reacted both with desmoglein 1 and a 190-kDa antigen immunolocalized to the desmosomal plaque, previously demonstrated to be recognized by sera of some patients with paraneoplastic pemphigus. IgG subclass analysis further showed that antidesmoglein 1 antibodies were of IgG1 and/or IgG4 subclasses, while anti-190-kDa antibodies were IgG3. The patients were free of malignancy.
In this study we treated 6 patients with epidermotropic cutaneous T cell lymphoma (CTCL) with intermediate doses of recombinant alpha 2a interferon (18–100 × 106 IU/week) for 2–6 months. One patient experienced complete clinical remission in spite of a persistent dense lymphocytic skin infiltrate. One patient was markedly improved and 2 patients were moderately improved. The clinical condition of the 2 remaining patients was unchanged by interferon treatment. In all cases lesions relapsed a few weeks after treatment was discontinued. This study shows that interferon can be used to treat epidermotropic CTCL. However, a 2- to 6-month treatment using moderate doses did not lead to the high percentage of remission previously reported by others with high doses of recombinant alpha 2a interferon, for longer periods. This result suggests that interferon should be used at high doses and/or for long time periods for clinical improvement of CTCL patients.
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