rituximab, she is in partial remission, suffering occasional and small trauma-induced blisters only, such that she has manage- able disease with a relatively normal quality of life. The dose of prednisolone has been reduced to 5 mg daily, MMF contin- ued 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 9 L )1 to <0Æ01 · 10 9 L )1 immediately post-treatment (normal 0Æ06– 0Æ47 · 10 9 ). One year later, B-cell levels remain low at <0Æ01 · 10 9 L )1 . Total IgG was mildly reduced at 5Æ7gL )1 (normal 6–16) prior to rituximab, presumably due to immu- nosuppressive treatment. Levels fell to 4Æ9gL )1 following treatment and remain low at 4Æ7gL )1 at 1 year later. EBA is a rare acquired, subepidermal bullous disease associ- ated 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 con- ventional immunosuppression in a patient who had aggres- sive, 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 pem- phigus vulgaris. 1 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 re- cently published another patient with EBA in whom rituximab resulted in sustained clinical remission. 3 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 im- munosuppression. Rituximab should be considered as a thera- peutic option in such patients. Acknowledgments We thank Dr Nori Oyama for immunoblotting and Mr B.S. Bhogal for indirect immunofluorescence. S.M. C RICHLOW N.J. M ORTIMER K.E. H ARMAN Department of Dermatology, University Hospitals Leicester, Leicester Royal Infirmary, Leicester LE1 5WW, U.K. E-mail: sharon.crichlow@uhl-tr.nhs.uk References 1 Schmidt E, Hunzelman N, Zillikens D et al. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol 2006; 31:503–8. 2 Arin MJ, Engert A, Krieg T et al. Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus. Br J Dermatol 2005; 153:620–5. 3 Schmidt E, Benoit S, Brocker EB et al. Successful adjuvant treatment of recalcitrant epidermolysis bullosa acquisita with anti-CD20 anti- body rituximab. Arch Dermatol 2006; 142:147–50. Conflicts of interest: none declared. Spotted and rippled reticulate hypermelanosis: a possible variant of Dowling–Degos disease DOI: 10.1111/j.1365-2133.2006.07592.x SIR, Pigmentation disorders derive from melanoblast migration, biogenesis of melanosomes, and secretion of melanosomes into keratinocytes. 1 Reticulate hyperpigmentary disorders have been classified into dyschromatosis symmetrica hereditaria (DSH) of Toyama (MIM 127400), dyschromatosis universalis hereditaria (DUH) (MIM 127500), Dowling–Degos disease (DDD) (MIM 179850) and reticulate acropigmentation of Kitamura (RAK). We encountered a 3-year-old girl showing asymptomatic and asymmetrical, spotted and rippled reticulate hypermelanosis without hypomelanosis. Here, we discuss the similarities to and differences from previously defined disorders. A 3-year-old Japanese girl presented with spotted and rip- pled reticulate hypermelanosis without hypopigmented spots. The pigmentation showed an asymmetrical distribution. The pigmentation gradually developed from the age of 1 year. Rip- pled reticulate hypermelanosis was observed around the left axilla, and faint hypermelanosis around the right axilla. Spot- ted and rippled reticulate hypermelanosis was present on the flexures of the extremities, especially on the right thigh (Fig. 1a). Spotted reticulate hypermelanosis was present on the dorsa of the hands and feet (Fig. 1b). The dorsum of the left foot showed more pronounced hypermelanosis (Fig. 1b). V-neck-shaped asymmetrical hyperpigmentation was observed. The patient had normal palms and soles, and no other cutane- ous abnormalities. Her relatives did not have reticulate hyper- pigmentation. Skin biopsies were taken from sun-protected areas of the upper back: a hyperpigmented lesion on the left side and a normal skin-coloured lesion on the right side. His- topathology of both biopsy specimens showed the same fea- tures. Haematoxylin and eosin staining showed no papillary downgrowth of the epidermis. Fontana–Masson staining indi- cated asymmetrical distribution of melanin granules and per- sistence of melanin granules through the epidermis (Fig. 2a). Electron microscopy showed a small number of stage II mel- anosomes, about 45% of melanosomes being stage III, and the remainder stage IV (Fig. 2b). The features of the patient were as follows: (i) the onset of hypermelanosis from age 1 year; (ii) clinical appearance: (a) spotted and rippled reticulate hypermelanosis that developed asymmetrically on the flexures and acral regions, and (b) pro- nounced asymmetrical hyperpigmentation after sun exposure; Ó 2006 The Authors Journal Compilation Ó 2006 British Association of Dermatologists • British Journal of Dermatology 2007 156, pp163–201 196 Correspondence