A case of sporadic Bazex-Dupré-Christol syndrome presenting with scarring folliculitis of the scalp.
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We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this disease which merit consideration. Lichen sclerosus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women. The aetiology is unknown. Approximately 20% of affected patients have extragenital lesions that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhagic forms may occur but these are generally localized and reports of extensive or generalized involvement are rare. We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.
The cases of two brothers who both developed pseudopelade at the age of 7 years, with progressive patchy hair loss, are reported. Biopsy in the elder brother showed typical changes with reduced numbers of hair follicles and sebaceous glands, a sparse lymphocytic infiltrate and an absence of widespread scarring. A third family member may have been affected. The absence of a family history in most cases suggests our patients may have a different aetiology. This report of two, possibly three, family members with pseudopelade suggests genetic factors may play a role in this family. Pseudopelade is very rare in children and we are not aware of any reports of familial occurrence. Atrophy rather than scarring is the primary feature of this condition and pseudopelade of Brocq should not be classified as a cicatricial alopecia.
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