J.Ph. Lacour

Porphyria cutanea tarda and antibodies to hepatitis C virus

We have studied the prevalence of hepatitis C virus antibodies (anti‐HCV) in 13 patients suffering from sporadic porphyria cutanea tarda. The sera were tested by Abbott second‐generation enzyme immunoassay; seropositivity was confirmed by Ortho second‐generation recombinant immunoblot assay. Ten cases (76.1%) were anti‐HCV positive; one patient was also seropositive for HIV. This preliminary study suggests that HCV could be a frequent triggering factor for sporadic porphyria cutanea tarda.

Demodicidosis in an Immunodeficient Child

Abstract: A 15‐month‐old girl developed acute lymphoblastic leukemia. Chemotherapy had induced a complete remission and she was continued on maintenance therapy. At 3 years of age, she developed an eruption consisting of excoriated papules and pustules on the face. Demodex folliculorum seemed to be the cause. Topical treatment with metronidazole applied twice a day over a period of 2 weeks resulted in partial improvement. The dermatosis finally cleared gradually with oral erythromycin therapy and one overnight application of 1% lindane cream per week for 2 successive weeks.

Pseudo-dermatomyositis induced by long-term hydroxyurea therapy: report of two cases.

1 Tsui |KC, Hisen A. Stocssl A] el al. Double-blind study of hotulinum loxin in spasmodic torticollis. lMiicel 1986: ii: 245-7. 2 (Sirliinda 1*. Vita (.!. NJcolosi C el iiL Botulinum toxin therapy: distant effects on neuromuscular transmission. / Neurol Neursurg Fsfichiatrij 1992: S5:K44-5. J Sthnider P. Bricbta A, Schmied M, Auff E. Gallbladder dysfunction induced hy botulinum A toxin. iMiiret 1993: 342: 811-12 (letter). 4 iiushara KO. Park DM. Botulinum roxin and sweating. / Neurol FsfichitUrn 1994: 57: 14i7-S.

Acral hyperpigmented macules and longitudinal melanonychia in AIDS patients

Two patients with AIDS are described who developed acral hyperpigmented macules of the fingers, palms and soles, buccal mucosa and genitalia, associated with longitudinal melanonychia. These pigmentary changes seemed to be independent of zidovudine and were associated in one with diffuse melanoderma and elevated levels of α‐MSH. Histological and ultrastructural studies showed an increase of the dendrites and pigmentation of the melanocytes and few melanosomes in the keratinocytes.

Juvenile pityriasis rubra pilaris associated with hypogammaglobulinaemia and furunculosis

We report a case of follicular keratosis with inflammatory changes, consistent with a diagnosis of atypical juvenile pityriasis rubra pilaris. An unusual feature was the occurrence of severe Staphylococcus aureus folliculitis and furunculosis, a phenomenon rarely encountered in pityriasis rubra pilaris and the other follicular keratoses. Standard antibiotic and antiseptic treatment for chronic S. aureus infection was ineffective. The patient was subsequently found to have hypogammaglobulinaemia, and treatment with human polyvalent immunoglobulin infusions was successful in eradicating the sepsis. It is therefore probable that the hypogammaglobulinaemia played a pathogenic role in the development of cutaneous sepsis.

Fibroblastic rheumatism: clinical, histological, immunohistological, ultrastructural and biochemical study of a case.

We report a case of fibroblastic rheumatism (FR). Only eight other cases of this recently described entity have been reported previously. FR is characterized hy polyarthralgia and joint stiffness without joint destruction, associated with cutaneous nodules and sclerodactyly. Histology shows an increase in the number of fihroblasts and marked dermal fibrosis, Rheumatological and skin manifestations may improve with corticosteroid therapy.

Congenital constriction band of the trunk

Abstract: Constriction band of the trunk is a rare congenital malformation. A 2‐year‐old girl with a congenital circumferential constriction band around the waist is described. Six other cases of congenital constriction band of the trunk are reviewed. Like constriction band of the limbs, this malformation is probably related to the amniotic band syndrome. Surgical correction should be considered carefully because natural evolution is usually benign and the postoperative esthetic benefit may be questionable. There is no evidence of any risk of familial recurrence.

Expression of the antigen recognized by mAb GB36 in normal skin and in skin tumours

GB36, a mouse monoclonal antibody (mAb) raised against an epithelial antigen of the human trophoblast, reacts with the epithelial basement membrane of chorionic villi: It does not react with the invasive extravillous cytotrophoblast. Expression and characterization of the antigen of GB36 (designated GBA36) were investigated in normal keratinocytes by immunoprecipitation, immunofluorescence and immunoelectron microscopic studies. Immunoprecipitation experiments demonstrated that the proteins identified on keratinocytes by mAb CB36 and a rat mAb anti‐integrin α6(GoH3) were the same. Using immunofluorescence and immunoelectron microscopic methods, GBA36 was localized on the cell membrane facing the epithelial basal lamina of basal keratinocytes. GBA36 distribution in benign and malignant skin tumours was evaluated by immunostaining methods (immunofluorescence and immunoperoxidase). Analysis of tumours revealed that whereas benign epithelial tumours and intradermal naevi displayed high levels of GBA36, the expression of this antigen decreased progressively in spinocellular and basal cell carcinomas, and in cutaneous melanomas in relation to invasiveness. During cell transformation, GBA36 undergoes quantitative alterations, and expression is down‐regulated. Although the functional relevance of these changes remains unknown, the correlation of decreased GBA36 expression with tumour progression may indicate a role for altered integrin expression in tissue invasion by human skin carcinoma and melanoma.