Sclerodermiform Lupus Erythematosus: A Series of Five Patients.

Background: Sclerodermiform lupus erythematosus (SDLE) is a rare Type 3 overlap syndrome of morphea and cutaneous lupus diagnosed with histopathologic features of both diseases present. It was first reported in 1976 by Umbert et al with a case series of four patients. SDLE is more common in young to middle-aged female patients. Methods: After IRB approval, we searched our internal pathology database for cases of SDLE, excluded any patients that did not fulfill the diagnostic criteria, and verified each case with a board-certified dermatopathologist. Results: Five patients with SDLE were identified; three of the patients are male and three are Black. Consistent with prior reports in the literature, the lesions were described as hyperpigmented plaques or nodules. The most commonly involved location was the extremities. Histopathology showed diffuse sclerosis in all five patients' biopsies, vacuolar interface changes in three biopsies, basement membrane thickening in one biopsy, and increased dermal mucin deposition in two of the four biopsy specimens stained with colloidal iron. Improvement was noted in patients treated with topical, intralesional, or systemic corticosteroids, topical calcineurin inhibitors, and oral antimalarials. Conclusion: We describe five cases of SDLE which is the largest series to date and the first series with a majority of patients being male. Improved recognition and a more thorough understanding of SDLE is necessary for appropriate diagnosis and management.