Leukocyte Adhesion Defect Type 1 Presenting with Recurrent Pyoderma Gangrenosum.
Leukocyte adhesion deficiency 1 (LAD.1) is a rare autosomal recessive disorder of leukocyte function. LAD.1 affects about 1 per 10 million individuals and is characterized by recurrent bacterial and fungal infections and depressed inflammatory responses despite striking blood neutrophilia. Patients...
| Publicado en: | Indian Journal of Dermatology Vol. 58; no. 2; pp. 158 - 159 |
|---|---|
| Autores principales: | , , , |
| Formato: | case study diagnostic images pictorial Journal Article |
| Publicado: |
Wolters Kluwer India Pvt Ltd
Mar/Apr2013
|
| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104263055&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 104263055 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 00195154 259H jtl: Indian Journal of Dermatology issn: 00195154 maglogo: N pubinfo: dt: Mar/Apr2013 vid: 58 iid: 2 pid: 16919 pub: Wolters Kluwer India Pvt Ltd artinfo: ui: 104263055 86678167 10.4103/0019-5154.108076 NLM23716823 PMC3657233 104263055 ppf: 158 ppct: 1 formats: tig: atl: Leukocyte Adhesion Defect Type 1 Presenting with Recurrent Pyoderma Gangrenosum. aug: au: Thakur, Neha Sodani, Ravitanya Chandra, Jagdish Singh, Varinder affil: Department of Pediatrics, Kalawati Saran Children Hospital, New Delhi, India sug: subj: Pyoderma Gangrenosum Diagnosis Recurrence Cell Adhesion Molecules Cicatrix Pyoderma Gangrenosum Therapy Child, Preschool Female Disease Attributes Child, Preschool: 2-5 years Female ab: Leukocyte adhesion deficiency 1 (LAD.1) is a rare autosomal recessive disorder of leukocyte function. LAD.1 affects about 1 per 10 million individuals and is characterized by recurrent bacterial and fungal infections and depressed inflammatory responses despite striking blood neutrophilia. Patients with the severe clinical form of LAD.1 express <0.3% of the normal amount of the ƒÃ2.integrin molecules, whereas patients with the moderate phenotype may express 2.7%. Skin infection may progress to large chronic ulcers with polymicrobial infection, including anaerobic organisms. The ulcers heal slowly, require months of antibiotic treatment, and often require plastic surgical grafting. The diagnosis of LAD.1 is established most readily by flow cytometric measurements of surface CD11b in stimulated and unstimulated neutrophils using monoclonal antibodies directed against CD11b. Pyoderma gangrenosum (PG) is an uncommon condition characterized by recurrent sterile, in.ammatory skin ulcers. Commonly, PG occurs in the context of in.ammatory bowel disease or rheumatic, hematologic, or immunologic disorders. Here, we present a 5.year.old female with a long history of PG, which healed with atrophic scarring, who was ultimately diagnosed with leukocyte adhesion deficiency type 1 (LAD1). She had a good response to high.dose prednisone therapy (2 mg/kg) and was discharged after 3 weeks of admission but only to be re.admitted 3 weeks later with severe pneumonia. During hospital stay, she developed pneumothorax and pneumomediastinum and later succumbed to her illness. pubtype: Academic Journal doctype: case study diagnostic images pictorial Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
|---|