AB023. An atypical clinical presentation of alopecia in two patients with systemic lupus erythematosus

Abstract: Alopecia in varying patterns is a common feature of lupus erythematosus (LE). Several forms of alopecia that are not specific to LE can occur in the setting of lupus, such as alopecia areata, telogen effluvium, and anagen effluvium. LE-specific alopecias are defined as those with histology consistent with LE, and include forms of acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). Common patterns of LE-specific alopecia include the non-scarring diffuse hair thinning and fragility of acute LE and the scarring, erythematous scaly plaques with follicular keratotic plugs, peripheral hyperpigmentation, and central hypopigmentation of discoid lupus erythematosus (DLE). Lupus erythematosus tumidus (LET) may also present on the scalp as well-defined, non-scarring alopecia without overlying scale, atrophy, and dyspigmentation. We report two male patients with large circular non-scarring alopecic plaques on the scalp without overlying scale or erythema, but with central hyperpigmentation and scarring – findings reminiscent of but also distinct from both DLE and LET. Punch biopsy from the hyperpigmented area of alopecia revealed peri-follicular lymphocytic infiltrate, follicular dropout, and increased dermal mucin consistent with LE; further workup and serologic testing revealed systemic lupus in both patients. These two cases demonstrate a unique clinical presentation of central scarring alopecia within a larger non-scarring alopecic plaque in the setting of SLE that deviates from typical lupus-related alopecia. Teaching point: these two cases underscore the morphologic heterogeneity of CCLE and need for thorough evaluation of systemic disease in patients presenting with both scarring and non-scarring alopecia.

Keywords: Alopecia; chronic cutaneous lupus erythematosus (CCLE); discoid lupus erythematosus (DLE); lupus erythematosus tumidus (LET); systemic lupus erythematosus (SLE)