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Pediatr Dermatol ; 35(3): 343-353, 2018 May.
Article in English | MEDLINE | ID: mdl-29536565

ABSTRACT

BACKGROUND/OBJECTIVES: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. The objective was to have a better understanding of the spectrum of chronic cutaneous GVHD (cc-GVHD) in children. METHODS: Hospital records of 14 children with cc-GVHD, registered over 9 years, were reviewed. RESULTS: All the patients had received HSCT from related donors. Median duration between HSCT and onset of cc-GVHD was 7.5 months. Eighty-six percent of the patients had a prior history of aGVHD, and 14% had de novo onset of cc-GVHD. Of 14 patients, 71% had classic cc-GVHD. Overlap syndrome was observed in 29%. Tandem occurrence of multiple morphologies was noticed in 6 (43%) patients. Of classic cc-GVHD, lichen planus-like cc-GVHD was most common (57%) followed by scleroderma-like (29%) and poikiloderma (7%). Rare variants included eczema-like (14%) and psoriasis-like (7%) cc-GVHD. Mucosal involvement was seen in 78.6% of the patients, nail involvement in 50%, and hair abnormalities in 43%. After a median follow-up of 4.8 years, complete remission was observed in 50% and mortality in 14%. CONCLUSION: The study signifies the diverse nature of cc-GVHD and indicates the need for multicenter surveys including larger number of patients to have proper insight into and develop treatment guidelines for cc-GVHD in children.


Subject(s)
Graft vs Host Disease/diagnosis , Hematopoietic Stem Cell Transplantation/adverse effects , Skin Diseases/etiology , Adolescent , Child , Child, Preschool , Chronic Disease , Dermatology , Female , Graft vs Host Disease/etiology , Humans , Infant , Male , Retrospective Studies , Skin/pathology , Skin Diseases/diagnosis , Tertiary Care Centers
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