PMC3995401_01
a Clinical features at the first visit. Symmetrical 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 tended to be notably resolved. Mucosal areas, such as the oral cavity and penis, were not involved. b Clinical features 1 week after consultation. Spiking fever and symmetrical bullous erythema worsened 1 week after admission, suggestive of pemphigus foliaceus. c Histopathological features on the erythematous lesion. Bars = 100 mum.
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F1
The center of the erythematous lesions in the skin tended to be notably resolved (fig. 1a), while the mucosal areas were intact. A fever and symmetrical bullous erythema worsened 1 week after admission (fig. 1b). 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. 1b). Pigment incontinentia was notable, along with severe inflammation (fig. 1c).
Results of immunohistochemical analysis for immunocompetent cells. Paraffin-embedded tissue samples from the patient were stained with CD4 (a), CD8 (b), CD1a (c), HLA-DR (d), CD68 (e), CD163 (f), IL-17A (g) and Foxp3 (h). CD8+ cells, instead of CD4+ cells, and HLA-DR+-activated T cells densely infiltrated into the epidermis of the erythematous lesion. CD1a+ Langerhans cells were decreased in number. While CD68+ macrophages densely infiltrated into the upper dermis, there was only slight infiltration of IL-17A+ cells and Foxp3+ cells. Bar = 250 mum.
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F2
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. 2a-h).
Clinical course after admission, and the distribution of melanocytes and melanosomes in each lesion. a The image shows symmetrical bullous erythema that worsened 1 week after admission. b The crusted bulla and fused erythema remitted after systemic steroid treatment. Twenty-eight days after starting the treatment, annular hypopigmentation appeared corresponding to the edge of the erythematous lesion. c The hypopigmentation was decreased 40 days after admission. d-f Masson-Fontana staining of erythematous (d), annular hypopigmented (e) and uninvolved lesions (f). The erythematous and hypopigmented lesions were accompanied by extensive pigment incontinentia. In contrast, basal melanosomes were increased in the erythematous lesion. Bars = 100 mum. g-i Melan-A immunohistochemical staining of erythematous (g), hypopigmented (h) and uninvolved lesion (i). The number of melanocytes was decreased in the erythematous and hypopigmented lesions compared to the uninvolved lesion. Bar = 100 mum. j, k. Results of the ultrastructural analysis for the hypopigmented lesion. While melanosome production in melanocytes was attenuated, adjacent keratinocytes (j) possessed a significant number of mature melanosomes (k), suggesting no trafficking disturbance. Original magnification x15,000.
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F3
An increased dose of oral prednisolone was effective not only for spiking fever, cytopenia and hypocomplement level, but also crusted bullae and fused erythema (fig. 3a). Instead, annular incomplete hypopigmentation tracing the crusted lesions was noted 1 month later (fig. 3b). Our histological observations of the hypopigmented lesion showed a decrease, but not total absence, of melanosomes and melanocytes detected by Masson-Fontana (fig. 3d-f) and Melan-A (fig. 3g-i) 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. 3j, k). The hypopigmented lesions recovered 2 weeks later, without any additional treatment (fig. 3c).