[A case of cervical actinomycosis developing for 7 years without any treatment].
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Biomedical subjects
Publications and source records attributed to B Labeille.
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41 patients with pemphigoid entered a multicentre randomised study of the efficacy of plasma exchange. All patients received 0.3 mg/kg daily oral prednisolone, increased weekly if the disease remained active. 24 patients received plasma exchanges in addition (eight large-volume exchanges over 4 weeks), and 17 had prednisolone only. 4 patients, 2 in each group, were withdrawn from the study. The initial dose of prednisolone was effective in 13 of 22 patients receiving plasma exchange but in none of 15 patients receiving prednisolone only. Control of the disease was obtained with a mean daily prednisolone dose of 0.52 +/- 0.28 mg/kg in the plasma exchange group v 0.97 +/- 0.33 mg/kg in the other group and a mean cumulative dose of 1240 +/- 728 mg v 2770 +/- 1600 mg. This finding suggests that plasma exchange allows a substantial saving of corticosteroids in the management of pemphigoid. This sparing effect was observed whether or not serum anti-basement membrane antibodies had been detected before treatment.
BACKGROUND: Bullous lesions of acral distribution are an uncommon finding in Bazex's syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases. PATIENTS AND METHODS: A 65 year-old-man, an alcoholic and a smoker, presented with characteristic lesions of Bazex's acrokeratosis paraneoplastica associated with a right cervical lymph node mass. An epidermoid carcinoma of sinus piriformis was then discovered. The acrokeratosis lesions worsened and spread to the knees, elbows, trunk and genitalia, with an erosive aspect, pain in the extremities, and tender, hemorrhagic bullous lesions on the toes and sides of the feet. Histological examination of a biopsy sample showed a subepidermal blister with numerous eosinophilic leucocytes. Direct immuno-fluorescence showed C3 deposits on dermal capillaries and IgA and IgM on colloid bodies in the papillary dermis. Blood eosinophilia and high levels of IgE were noted. Indirect immunofluorescence was negative for anti-epidermal and anti-basement membrane antibodies. Radiation and chemotherapy for the neoplasia resulted in healing of the bullous lesions and almost complete disappearance of keratotic squamous lesions with residual hyperpigmentation. DISCUSSION: Although bullous lesions are rare in Bazex's syndrome (acrokeratosis paraneoplastica), they were noted as early as 1968 by Degos et al. These acral bullae mimic autoimmune bullous disease. An immunological reaction is thought to occur with antigens of the dermal-epidermal junction and eosinophils appear to be involved.
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