Pemphigus antibodies

Although rare, cases of renal lupus syndrome and pemphigus blisters have also been reported as a consequence of D-penicillamine-induced immune complexes (Ntoso et al., 1986 Bigazzi, 1988), as well as with other drugs. With renal lupus syndrome, secondary glomerulonephritis may result if granular IgG antibodies are produced and deposited on the basement membranes. In patients with pemphigus blisters, autoantibodies to the intercellular substance of the skin have been recovered from the sera, and dermal biopsies have demonstrated intracellular deposits or... 

Mahoney, M. G., Aho, S., Uitto, J., and Stanley, J. R. (1998). The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin./ Invest. Derm. Ill, 308-313. 

M19. Moncada, B., Kettelsen, S., Hemandes-Moctezuma, J. L., and Ramirez, F. Neonatal pemphigus vulgaris Role of passively transferred pemphigus antibodies. Clin. Res. 28, 251A (1980). 

Bullous pemphigus and pemphigoid with circulating pem-phigus-like autoantibodies, pemphigus foliaceus with anti-intercellular IgG antibodies, and paraneoplastic pemphigus due to interferon alfa have rarely been reported (SED-13,1095), as has extensive oral pemphigus (294). 

Rasmussen HB, Jepsen LV, Brandrup F. Penicillamine-induced bullous pemphigoid with pemphigus-like antibodies. J Cutan Pathol 1989 16(3) 154-7. 

A third series of experiments was done to check the eventual modifications of the intercellular substance (i.s.) in the Malpighian layer and stratum corneum. The antigenic substrate of five of the ichthyotic subjects was treated with serum with anti-i.s. antibodies (titre 1 320) taken from a pemphigus patient (see Figure 5.1). Each section was treated with two different dilutions of pemphigus serum (1 10 and 1 40) and with an anti-IgG conjugate (same characteristics as above). 

Pemphigus antibodies fix to the i.s. in both the Malpighian layer and the stratum corneum (see Table 5.4). It should be noted that the intensity of the fluorescence varies considerably from one substrate to another it also diminishes in relation to the dilution of the serum. 

Table 5.4 Results obtained with pemphigus antibodies on skin biopsies in five cases of ichthyosis vulgaris...

The patient, a 66-year-old man, was treated with 500 mg penicillamine daily for rheumatoid arthritis. After 6 months of treatment a bullous and crythcmatosquamous eruption developed on the trunk, scalp and arms. The Nikolsky phenomenon was positive. An erythematosquamous balanitis was present stomatitis did not develop. On cytological examination acantholytic cells were present. Histological examination revealed both aspects of toxicoderma and pemphigus. Intercellular immunofluorescence was positive in the superficial layers of both affected and apparently normal skin. Electron microscopic examination confirmed acantholysis. Anti-intercellular substances and anti-striational antibodies were detected in the serum ANF and LE cells were absent. The disorder rapidly disappeared after withdrawal of penicillamine, and a course of treatment with a corticosteroid. 

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