Seiichi Kurihara

Capability of complement fixation of pemphigus antibodies in vitro.

The capability of complement fixation of pemphigus antibodies was tested using combined in vitro complement immunofluorescent (IF) staining methods. Three sera out of 25 serum samples from 22 pemphigus patients revealed positive reactions, while all other sera gave negative results. Specificity control tests confirmed the positive reactions to be specific for complement staining. Complement fixing pemphigus antibodies were titrated lower than corresponding IgG antibodies and were demonstrable only in the extensive stage of the disease. Thus, the present work supplied evidence that pemphigus antibodies fix complement in vitro. However, the discrepancy still remains between the in vivo deposition of complement in most cases of pemphigus and in vitro capability of complement fixation in only few cases. More investigations should be needed to explain the exact role of complement in pemphigus acantholysis.SummaryThe capability of complement fixation of pemphigus antibodies was tested using combined in vitro complement immunofluorescent (IF) staining methods. Three sera out of 25 serum samples from 22 pemphigus patients revealed positive reactions, while all other sera gave negative results. Specificity control tests confirmed the positive reactions to be specific for complement staining. Complement fixing pemphigus antibodies were titrated lower than corresponding IgG antibodies and were demonstrable only in the extensive stage of the disease. Thus, the present work supplied evidence that pemphigus antibodies fix complement in vitro. However, the discrepancy still remains between the in vivo deposition of complement in most cases of pemphigus and in vitro capability of complement fixation in only few cases. More investigations should be needed to explain the exact role of complement in pemphigus acantholysis.ZusammenfassungMit Hilfe von dem kombinierten in vitro-Immunofluores-cenzkomplementfixationstest wurde in vitro-Bindung von Komplement durch Pemphigusantikörper untersucht. Drei Seren aus den 25 Seren von 22 Patienten mit Pemphigus zeigten positive Bindung von Komplement (K3), während die übrigen Seren negative Befunde zeigten. Die Spezifitätskontrolltests bestätigten, daß die positive Reaktionen spezifisch für Komplementfixation waren. Diese Komplementbindungsantikörper zeigten einen niedrigeren Titer als die korrespondierenden IgG-Antikörper und konnten nur im ausgedehnten Krankheitszustand gefunden werden. Diese Ergebnisse belegten, daß Pemphigusantikörper das Komplement in vitro fixieren. Jedoch gibt es Diskrepanzen zwischen in vivo-Komplementsvorkommen und in vitro-Komplementsbindung. Die genaue Rolle des Komplements bei der Pemphigusacantholyse muß noch weiter untersucht werden.

Late-onset focal dermal elastosis: clinical and histological features

We describe two patients with lesions clinically resembling pseudoxanthoma elasticum and histologically exhibiting focal elastosis. with normal‐appearing elastic fibres in the mid‐ and deep dermis. We consider that these skin lesions represent a previously undescribed entity, whose pathogenesis appears to be related to the ageing process.

Treatment of Granuloma Annulare with Tranilast

Three cases of granuloma annulare which did not exhibit a self‐limited course were treated with tranilast at the dose of 300 mg/daily. The treatment resulted in the resolution of skin lesions within three months of administration. Although spontaneous resolution is often observed in granuloma annulare, tranilast may provide an alternative therapy for the treatment of cases resistant to spontaneous healing.

Capability of complement fixation by in vivo bound antibodies in pemphigus skin lesions.

Skin biopsy was performed in five cases of pemphigus; direct immunofluorescence (IF) studies demonstrated intercellularly bound IgG and C3 in each case. Sections of lesional skin, containing in vivo bound IgG were, in two cases, capable of further binding C3in vitro from normal human serum, an effect most marked in relation to areas of acantholysis. This work provides additional evidence that the complement system is involved in the process of pemphigus acantholysis.

Atypical pemphigus showing eosinophilic spongiosis

A 79‐year‐old female developed a generalized pruritic annular erythematous eruption without bullae. Histologically there was eosinophilic spongiosis but no associated acantholysis. Using direct immunofluorescence, the deposition of IgG and C3 was detected in the intercellular space, but circulating anti‐intercellular‐antibodies were not demonstrated. We considered this case to have atypical pemphigus. The eruption responded well to oral administration of Diaminodiphenyl sulphone 75 mg daily.

Experimental acantholysis by complement-fixing intercellular antibodies

SummaryComplement-fixing intercellular antibodies were detected in 10 of 17 sera from untreated pemphigus patients. The role of complement in the organ culture system was investigated using these sera. Ten sera possessing complement-fixing intercellular antibodies showed IgG binding to the intercellular substance in the organ-cultured skin and acantholysis-like changes were observed in eight cases. C3 deposition was not seen in any case. However, after treatment of the sections of cultured skin with fresh normal human serum, complement fixation of the intercellular substance by bound IgG was revealed in all the ten cases. No significant differences in the grade of acantholysis-like changes between the complement-depleted system and the complement-supplied system were observed. Complement does not appear to be necessary in the acantholytic process in the in vitro organ culture system, even though we considered the presence of complement-fixing intercellular antibodies.

Prurigo pigmentosa Affecting the Forehead

A little-known dermatosis, prurigo pigmentosa, is characterized by itchy reddish lesions and gross reticular pigmentation which occurs mainly on the trunk. Histologically, it is characterized by lichenoid tissue reaction. We present a 23-year-old male patient who showed typical eruption not only on the trunk but also on the forehead. Although the etiology remains to be explained, this unusual distribution of the eruption might help to know the real cause of this peculiar disorder.

Comparison of in vivo and in vitro Capability of Complement Fixation by Pemphigus Antibodies

Using complement immunofluorescence, the capability of complement fixation by bound antibodies in pemphigus lesional skin was compared with circulating intercellular antibodies of complement-fixing type in the same patient. Five lesional skin samples from 6 patients, containing bound IgG, gave positive binding of complement (C3) supplied from fresh guinea pig serum, while only one serum sample showed complement-fixing pemphigus antibodies in the circulation. These results support the view that complement-fixing pemphigus antibodies are rapidly localized to the skin and play a role on the production of acantholysis.

CORRELATION BETWEEN COMPLEMENT-FIXING PEMPHIGOID ANTIBODY TITRES AND DISEASE ACTIVITY

A positive correlation between complement‐fixing pemphigoid antibody titre and disease activity was observed in 4 of 8 patients with bullous pemphigoid using complement immunofluorescence. The fact that this correlation cannot always be demonstrated seems to contradict the active role of complement‐fixing pemphigoid antibodies in the pathogenesis of bulla formation. The similarity of bullous pemphigoid and herpes gestationis is discussed.

Two Cases of Atypical Bullous Disease Showing Linear IgG and IgA Deposition in the Basement Membrane Zone

Patients showing coexistent linear IgG and IgA deposition along the basement membrane zone on direct immunofluorescence have been described as either bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA bullous dermatosis, or cicatricial pemphigoid, depending on the clinical features and laboratory findings. In the present report, we describe two cases showing atypical clinical features distinct from those of other known bullous diseases. No circulating antibodies were detected by indirect immunofluorescence of normal human skin. Indirect immunofluorescence of 1 M NaCl split skin revealed IgG and/or IgA antibodies reactive with the dermal side of the split. Immunoblotting of normal human epidermal and dermal extracts showed no apparent reactivity with known autoantigens. The results suggest that there may be a unique and distinct bullous disease with linear IgG and IgA deposition at the basement membrane zone.