We report our outcomes following combined intestinal and abdominal wall transplantation, focusing on the presentation and treatment of acute rejection of the abdominal wall vascularized composite allograft (VCA). Retrospective analysis of all patients with combined intestinal/VCA transplantation was undertaken. Graft abnormalities were documented photographically and biopsies taken, with histological classification of rejection according to Banff 2007 guidelines. We have performed five combined intestinal and abdominal wall transplants to date. Two patients developed erythematous, maculopapular to papular eruptions confined to the VCA, histologically confirmed as grade II/III rejection, yet with normal bowel on endoscopy. Both patients' rashes resolved within 72 h of increasing immunosuppressive treatment. One patient later developed a recurrence of the rash, confirmed as skin rejection, but did not immediately seek medical attention. Treatment was therefore delayed, and mild intestinal rejection developed. We describe the rash associated with VCA rejection, and propose that while the skin of an abdominal wall VCA may reject independently of the intestinal allograft, delay in treatment of rejection episodes may result in rejection of the intestinal graft.
Treatment of vulvar lichen sclerosus (VLS) in children by topical corticosteroids gives control of symptoms and some resolution of physical signs, but large studies are limited. We report the largest study of 72 prepubertal girls with VLS, 62 of whom were prospectively treated with daily application of an ultrapotent topical corticosteroid (UPTC), clobetasol propionate 0.05% ointment, for 3 months, with a follow-up period of 4-8 years [the remaining 10 patients responded to mild to moderate potency topical corticosteroids (MPTCs)]. The results were compared with a retrospective study of 31 prepubertal girls with VLS treated with MPTCs. MPTCs led to symptom clearance in 32.2% of patients, whereas UPTC led to symptom clearance in 72.6% of patients. Improvement in clinical signs following UPTC occurred in 90.3% of children at 3 months, with total resolution of clinical signs occurring in 29.2% at the 4-year follow-up or at puberty. No serious adverse effects occurred with UPTC treatment. In children with VLS, UPTCs relieve symptoms, resolve signs and possibly prevent scarring. UPTCs should therefore be the treatment of choice for VLS in children.
Hereditary vitamin D-resistant rickets (HVDRR) is a rare autosomal recessive disorder caused by mutations in the vitamin D receptor (VDR) gene. We report the case of an infant presenting with alopecia, growth failure, and gross motor developmental delay. Serum biochemistry and skeletal survey were consistent with rickets. After a poor response to standard treatment, genetic testing confirmed a c.147-2A>T novel mutation in the VDR gene consistent with HVDRR. It is important for dermatologists and pediatricians to recognize alopecia as a presenting sign of HVDRR because appropriate treatment leads to better growth and development of the child.
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