A Rare Case of Male Bullous Lupus Erythematosus Complicated with Subsequent Annular Hypopigmentation
A 57-year-old male patient had noticed itchy erythema on the frontal chest in May 2011, and thereafter experienced disease enlargement and partial crust formation. Since no clinical effect of 10 mg/day of oral prednisolone was observed, he was referred to our hospital on July 7, 2011. In terms of the clinical findings at the first visit, symmetric red papules and centrifugal erythema, surrounded by an annular erosive crust, were distributed on the upper extremities and trunk in line with the seborrheic region. The center of the erythematous lesions in the skin tended to be notably resolved (fig. ), while the mucosal areas were intact. A fever and symmetrical bullous erythema worsened 1 week after admission (fig. ). Although his skin disease was initially suspected to be a bullous disease, neither anti-desmoglein 1/3 nor anti-BP 180 antibodies were detected.\nLaboratory tests revealed cytopenia (WBC, 10,470/μl; Neu, 72.6%; lymph, 13.0%; mono, 14.0%; eosino, 0.1%; baso, 0.3%; RBC, 3.85 × 106/μl; Hb, 12.7 g/dl; Ht, 38.5%; platelet, 180 × 103/μl), ANA (1/320, homogeneous + speckled pattern), positive anti-Sm antibodies, anti-RNP, anti-cardiolipin antibodies, and low C3 and C4 levels. Metal patch and photo tests were negative, and no internal malignancy was detected by whole-body imaging. A biopsy of the erythematous lesion showed individual apoptotic keratinocytes and liquefaction with partial cleft formation on the epidermis, and lymphocytes predominantly infiltrated in the upper dermis and perivascular area (fig. ). Pigment incontinentia was notable, along with severe inflammation (fig. ). The direct immunofluorescence findings of the uninvolved lesion were granular deposition of IgG and IgM in the basal membrane.\nThese laboratory and histological findings led to a final diagnosis of BLE type I. In an immunohistochemical analysis for immunocompetent cells, CD8+ cells, instead of CD4+ cells, and HLA-DR+-activating T cells were found to have densely infiltrated into the epidermis of the erythematous lesion. The number of CD1a+ Langerhans cells was decreased. The CD68+ macrophages densely infiltrated into the upper dermis, and there was a slight infiltration of IL-17A+ cells and Foxp3+ cells (fig. ).\nAn increased dose of oral prednisolone was effective not only for spiking fever, cytopenia and hypocomplement level, but also crusted bullae and fused erythema (fig. ). Instead, annular incomplete hypopigmentation tracing the crusted lesions was noted 1 month later (fig. ). Our histological observations of the hypopigmented lesion showed a decrease, but not total absence, of melanosomes and melanocytes detected by Masson-Fontana (fig. ) and Melan-A (fig. ) staining, respectively. In addition, an ultrastructural analysis of a hypopigmented lesion revealed attenuated melanin production, with the presence of immature melanosomes in the remaining melanocytes, whereas no disturbance of melanin transfer to adjacent keratinocytes was noted (fig. ). The hypopigmented lesions recovered 2 weeks later, without any additional treatment (fig. ).