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Notes: Summary 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. lgG subclass analysis further showed that antidesmoglein 1 antibodies were of lgGl and/or IgG4 subclasses, while anti-190-kDa antibodies were lgG3. The patients were free of malignancy.

Notes: Summary Primary cutaneous medium and large cell lymphomas (MLCL) other than mycosis fungoides (MF) are rare, and their prognosis and treatment are controversial. The clinical, immunohistological and follow-up data of 54 well-documented cases of primary cutaneous MLCL other than MF, seen in our institutions over a 14-year period, were retrospectively reviewed, in order to determine the prognostic factors related to these lymphomas, and to analyse the results obtained with different treatment regimens. Forty-six patients presented with a solitary tumour or with localized lesions. and eight had disseminated cutaneous lesions. According to the updated Kiel classification, 45 cases (83%) corresponded to B-cell lymphomas: centroblastic lymphomas, 32 cases; centroblastic-centrocytic lymphomas, 11 cases; immunoblastic lymphomas, two cases. Nine cases (17%) were classified as T-cell lymphomas: pleomorphic medium and large cell lymphomas, eight cases; anaplastic large cell lymphoma. one case. Four of eight patients with disseminated skin lesions had a T-cell lymphoma. whereas 41 of 46 patients with a solitary tumour had a B-cell lymphoma. Patients with disseminated skin lesions and elevated serum lactate dehydrogenase (LDH) levels had a poor prognosis. Comparison of patients' overall survival, depending on immunohistological subtype, showed that the median survival of patients with pleomorphic T-cell lymphoma was 2·5 years, whereas it was not reached at 12 years for patients with centroblastic centrocytic and centroblastic lymphoma. The eight patients with disseminated skin lesions were treated with polychemotherapy. Most patients with a solitary tumour or with localized lesions of low tumour bulk were treated by surgical excision or radiotherapy alone, and nine other patients with localized lesions of high tumour bulk were treated with initial polychemotherapy. Clinical presentation (i.e. solitary or disseminated lesions), serum LDH levels, and the immunohistological subtype, are important prognostic factors in cutaneous MLCL. Patients with disseminated skin lesions have a poor prognosis, and should be treated with intensive polychemotherapy regimens, whereas those with a solitary tumour, or with localized lesions of low tumour bulk, are adequately treated by radiotherapy.

Notes: We report the case of a 42-year-old woman with concomitant panniculitis and dermatomyositis. Painful, indurated lesions on the buttocks, thighs, arms, abdomen and breasts were associated with proximal muscle weakness. Skin biopsy revealed lobular panniculitis, and vacuolar degeneration of epidermal basal cells. Direct immunofluorescence was negative. Serum muscle enzyme (creatine-phosphokinase) levels were elevated, and electromyography demonstrated a myositic process. Muscle biopsy showed an inflammatory myositis. These results were consistent with dermatomyositis associated with panniculitis. Only tive cases of this association have been reported previously. The relationship between these two conditions is discussed.

Notes: The serum levels of hyaluronic acid (sHA) were measured using an affinoimmunoenzymatic assay in patients with distal (n= 16) and proximal (n= 15) progressive systemic sclerosis (PSS) and in 31 controls, The severity of PSS was evaluated using a standardized organ-involvement score. The mean sHA was significantly higher in the patients with PSS than in controls (mean ± SD: 80 ± 43.4μg/l vs. 42.3 ± 19.1 μg/l, P 〈 0.001). sHA was significantly higher in patients with proximal PSS than in patients with distal PSS (106.4±44.6 μg/l vs. 55.4±23.8 μg/l. P 〈 0.001). A positive correlation was found between sHA and the disease score (r= 0.67, P 〈 0.001). sHA was also correlated with lung diffusion capacity for carbon monoxide (r=0. 70. P 〈 0.001). but only in those patients who had abnormal lung function, and therefore presumably had lung PSS involvement.We suggest that sHA could be an indicator of the degree of systemic involvement in PSS. Its prognostic value and possible use in the follow up of patients with PSS remain to be clarified.

Notes: Summary We report a case of herpetiform pemphigus induced by thiopronine. Direct immunoelectron microscopy performed on peribullous skin showed IgG deposits predominantly located in the extracellular portion of desmosomes. By immunoblot analysis using bovine tongue extracts as the antigen, the patient's serum recognized a 160-kDa polypeptide which comigrated with desmoglein I. This study underlines the contribution of immunoblot analysis to the diagnosis of atypical cases of pemphigus, and confirms that herpetiform pemphigus may be a clinical variant of pemphigus foliaceus.