Document Detail

Early onset childhood cicatricial pemphigoid: a case report and review of the literature.
MedLine Citation:
PMID:  20537062     Owner:  NLM     Status:  MEDLINE    
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.
Monia Kharfi; Aida Khaled; Raja Anane; Becima Fazaa; Mohamed Ridha Kamoun
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Pediatric dermatology     Volume:  27     ISSN:  1525-1470     ISO Abbreviation:  Pediatr Dermatol     Publication Date:    2010 Mar-Apr
Date Detail:
Created Date:  2010-06-11     Completed Date:  2010-09-27     Revised Date:  2011-01-11    
Medline Journal Info:
Nlm Unique ID:  8406799     Medline TA:  Pediatr Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  119-24     Citation Subset:  IM    
Departement of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.
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MeSH Terms
Adrenal Cortex Hormones / therapeutic use
Blindness / prevention & control
Child, Preschool
Chronic Disease
Corneal Opacity / diagnosis*,  drug therapy,  prevention & control
Cyclosporine / therapeutic use
Dapsone / therapeutic use
Entropion / complications,  pathology
Hypopigmentation / diagnosis,  drug therapy
Immunosuppressive Agents / therapeutic use
Pemphigoid, Benign Mucous Membrane / complications,  diagnosis*,  drug therapy
Reg. No./Substance:
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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