ABSTRACT
Linear IgA bullous dermatosis (LABD) is an auto-immune disease affecting young children and adults, characterized by the linear deposition of IgA at the basement membrane zone with resultant complement activation and a cascade of immune reactions. There is a loss of adhesion at the dermo-epidermal junction and subsequent blister formation. It is a rare disease that has a good prognosis with adequate therapy. However, the underlying depressed immunity associated with the disease may expose them to such infections as tuberculosis. We report the case of an 11-years-old Nigerian female adolescent with LABD, diagnosed at the age of four years but defaulted on follow-up, who developed disseminated tuberculosis (pulmonary, lymph nodes, abdominal and pericardial effusion) seven years after the appearance of the initial blistering skin lesions. She commenced anti-tuberculosis drugs, steroids, and a tube pericardiostomy for the pericardial effusion. Dapsone was initiated for the LABD during the continuation phase of anti-tuberculosis therapy, with subsequent disappearance of the skin rash within two weeks.
La dermatose bulleuse linéaire à IgA (DBL) est une maladie auto-immune affectant les jeunes enfants et les adultes, caractérisée par le dépôt linéaire d'IgA dans la zone de la membrane basale, avec l'activation du complément qui en résulte et une cascade de réactions immunitaires. Il y a une perte d'adhérence à la jonction dermo-épidermique et une formation ultérieure de vésicules. C'est une maladie rare qui a un bon pronostic avec un traitement adéquat. Cependant, l'immunité déprimée sous-jacente associée à la maladie peut les exposer à des infections telles que la tuberculose. Nous rapportons le cas d'une adolescente nigériane de 11 ans atteinte de la LABD, diagnostiquée à l'âge de quatre ans mais en défaut de suivi, qui a développé une tuberculose disséminée (pulmonaire, ganglions lymphatiques, épanchement abdominal et péricardique) sept ans après l'apparition des lésions cutanées vésiculeuses initiales. Elle a commencé à recevoir des médicaments antituberculeux, des stéroïdes et une péricardiostomie par sonde pour l'épanchement péricardique. La dapsone a été initiée pour la DLB pendant la phase de continuation du traitement antituberculeux, avec une disparition de l'éruption cutanée en deux semaines. Mots clés: IgA linéaire, dermatose bulleuse, tuberculose disséminée, adolescent.
Subject(s)
Linear IgA Bullous Dermatosis , Pericardial Effusion , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Immunoglobulin A/therapeutic use , Linear IgA Bullous Dermatosis/diagnosis , Linear IgA Bullous Dermatosis/drug therapy , Linear IgA Bullous Dermatosis/pathology , NigeriaABSTRACT
A total of 614 inhabitants of Calabar, south eastern Nigeria were tested with anti-M, anti-N and anti-S sera to determine the frequency of MNSs blood groups in that population. The results showed that neither ABO blood group nor sex has influence on the occurrence of MNSs blood groups in the population. It was also observed that whereas S antigen was more frequently associated with M antigen, s associated more frequently with N antigen. One half of the Su antigen occurred with MN phenotype. Significantly, the Su antigen occurred in 94% of the male subjects.
Subject(s)
Black People/genetics , Gene Frequency , MNSs Blood-Group System/genetics , ABO Blood-Group System/genetics , Adolescent , Adult , Female , Humans , Male , Middle Aged , Nigeria , Phenotype , Reference Values , Sex CharacteristicsABSTRACT
Haemoglobin C (Hb C) is very rare in eastern Nigeria (east of River Niger). Isolated cases have been reported. The incidence is however as high as 6% in western Nigeria. This study was undertaken to locate the isolated cases with a view to determining their origin. Hb electrophoresis was performed on 4,263 subjects in Akwa Ibom State in south eastern region of Nigeria. The subjects were selected from all the local government areas of the state and were aged 15 years and above. They comprised secondary school children, civil servants, farmers and fishermen. The results revealed complete absence of Hb C from the upland population whereas this was present in the fishing settlement population (6 or 0.4% Hb AC and one or 0.07% Hb SC subjects). The possible entry points of Hb C gene into south eastern Nigeria is discussed.
Subject(s)
Hemoglobin C Disease/epidemiology , Adolescent , Adult , Blood Protein Electrophoresis , Child , Genotype , Hemoglobin C Disease/blood , Hemoglobin C Disease/genetics , Humans , Incidence , Middle Aged , Nigeria/epidemiology , Occupations , Population Surveillance , Residence CharacteristicsABSTRACT
A neutral metalloprotease with marked specificity for an O-sialoglycoprotein has been isolated from culture supernatants of Pasteurella haemolytica A1. The 35-kDa enzyme cleaves human erythrocyte glycophorin A, which is O glycosylated, but does not cleave N-glycosylated proteins or nonglycosylated proteins. Glycophorin A was cleaved when it was present in situ in erythrocyte ghost plasma membranes or when it was free in solution. The glycoprotease did not hydrolyze glycophorin A from which sialate residues had been removed by neuraminidase treatment. An immobilized preparation of the enzyme cleaved glycophorin A at several positions, with a major site of cleavage at Arg-31-Asp-32. The glycoprotease is inhibited by EDTA, citrate, and ascorbate, but inhibition appears to be due to the masking of metal ion activators rather than to their removal. The enzyme is not inhibited by phosphoramidon, an inhibitor of other bacterial neutral metalloproteases.
