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Spotted and rippled reticulate hypermelanosis: a possible variant of Dowling–Degos disease

small trauma-induced blisters only, such that she has manageable disease with a relatively normal quality of life. The dose of prednisolone has been reduced to 5 mg daily, MMF continued at 1 g twice daily and there has been a fall in IIF titres to 1 : 10 (Fig. 2). Predictably, the peripheral B-cell count fell from normal pretreatment levels of 0Æ12 · 10 L to < 0Æ01 · 10 L immediately post-treatment (normal 0Æ06– 0Æ47 · 10). One year later, B-cell levels remain low at < 0Æ01 · 10 L. Total IgG was mildly reduced at 5Æ7 g L (normal 6–16) prior to rituximab, presumably due to immunosuppressive treatment. Levels fell to 4Æ9 g L following treatment and remain low at 4Æ7 g L at 1 year later. EBA is a rare acquired, subepidermal bullous disease associated with autoimmunity to type VII collagen. It is notoriously difficult to treat and due to its rarity, evidence is anecdotal and largely limited to case reports. We embarked on this therapeutic trial because of the lack of response to more conventional immunosuppression in a patient who had aggressive, uncontrolled disease. At the time, there were no published data on rituximab for EBA but the rationale for its use was the growing body of publications reporting successful treatment of other antibody-mediated diseases including pemphigus vulgaris. Although her initial improvement could be partly attributed to methylprednisolone, which was pulsed shortly before rituximab, the long-term disease control is almost certainly the effect of rituximab. Schmidt et al. have recently published another patient with EBA in whom rituximab resulted in sustained clinical remission. Although rituximab is expensive, in this case the cost of dressings alone during her 4-week hospital admission was two-thirds that of the drug. In summary, rituximab used as an adjuvant drug has achieved a clinically useful partial remission in a patient with severe, recalcitrant EBA, resulting in considerable improvement in life quality and allowing a reduction in concomitant immunosuppression. Rituximab should be considered as a therapeutic option in such patients.