Correlates of low bone mass in children with generalized forms of epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) is a family of rare, heterogeneous, genetic disorders characterized by fragility of the skin and mucous membranes. Reduced bone mass and fractures have been recognized as complications of generalized forms of EB. OBJECTIVES: We sought to describe the range and to estimate the prevalence of low bone mass in children with generalized EB, and to identify correlates of low bone mass in this population. METHODS: This was a prospective, observational study of 24 patients with generalized EB. Each patient completed a history, physical examination, laboratory studies, bone age, and x-rays of the lumbar spine. Those aged 6 years and older underwent dual energy x-ray absorptiometry scans of the lumbar spine. Primary outcomes were areal bone mineral density (aBMD) based on chronologic age, bone age, and adjusted for height Z-score. Descriptive statistics were used to summarize results, and linear regression was used to determine factors associated with low aBMD. RESULTS: Mean lumbar spine aBMD Z-scores ± SD were: -2.6 ± 1.4 for chronologic age, -1.7 ± 1.3 for bone age, and -1.0 ± 1.2 after adjusting for height Z-score. aBMD Z-scores were less than or equal to -2 in 64% for chronologic age, 50% for bone age, and 28% after adjusting for height Z-score. aBMD correlated with height Z-score, weight Z-score, extensive blistering, immobility, albumin, hemoglobin, iron, erythrocyte sedimentation rate, and c-reactive protein values. LIMITATIONS: Small sample size was a limitation. CONCLUSIONS: Children with severe, generalized recessive dystrophic EB have low aBMD for age. Deficits in aBMD were reduced after adjusting for delayed skeletal maturation and small body size.

Adolescent hair loss.

PURPOSE OF REVIEW: Hair loss, or alopecia, may occur as a primary skin disorder or because of an underlying health problem. It may be upsetting to patients, particularly adolescents who are experiencing physical, emotional, and psychological transitions. Understanding the impact of alopecia is important for care providers who see adolescents. RECENT FINDINGS: The most common forms of alopecia in adolescence are telogen effluvium, androgenetic alopecia, and alopecia areata. Telogen effluvium may present suddenly or insidiously secondary to a variety of triggers. Androgenetic alopecia may begin in adolescence, and topical minoxidil is effective at retarding further hair loss. It may be a sign of underlying androgen excess, particularly polycystic ovary syndrome in women. Alopecia areata is less common, but may be distressing, especially if hair loss is extensive. Because treatments for alopecia are not curative, helping affected patients cope by offering support and nonpharmacologic techniques to help appear more like their peers should be part of care. SUMMARY: Physicians need to be skilled in evaluating the most common forms of alopecia presenting in adolescence and should be aware of potential treatments, including the value of psychosocial support.