| Early onset childhood cicatricial pemphigoid: a case report and review of the literature. | |
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MedLine Citation:
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PMID: 20537062 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation. |
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Authors:
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Monia Kharfi; Aida Khaled; Raja Anane; Becima Fazaa; Mohamed Ridha Kamoun |
Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: Pediatric dermatology Volume: 27 ISSN: 1525-1470 ISO Abbreviation: Pediatr Dermatol Publication Date: 2010 Mar-Apr |
Date Detail:
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Created Date: 2010-06-11 Completed Date: 2010-09-27 Revised Date: 2011-01-11 |
Medline Journal Info:
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Nlm Unique ID: 8406799 Medline TA: Pediatr Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 119-24 Citation Subset: IM |
Affiliation:
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Departement of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adrenal Cortex Hormones / therapeutic use Blindness / prevention & control Child Child, Preschool Chronic Disease Corneal Opacity / diagnosis*, drug therapy, prevention & control Cyclosporine / therapeutic use Dapsone / therapeutic use Entropion / complications, pathology Female Humans Hypopigmentation / diagnosis, drug therapy Immunosuppressive Agents / therapeutic use Infant Male Pemphigoid, Benign Mucous Membrane / complications, diagnosis*, drug therapy |
| Chemical | |
Reg. No./Substance:
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0/Adrenal Cortex Hormones; 0/Immunosuppressive Agents; 59865-13-3/Cyclosporine; 80-08-0/Dapsone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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