ABSTRACT
Plasmacytosis circumorificialis (PLC), a benign chronic inflammatory disease with an unknown pathogenesis, is characterized by erythema, erosion nodules and ulcers around the openings of the human body. In this report, we describe two cases of PLC successfully treated with topical tacrolimus. Interestingly, immunohistochemical staining revealed that prominent CD163(+) proinflammatory macrophages and IL-17-producing cells were infiltrating around plasma cells, which might suggest the reason for the therapeutic effect of topical tacrolimus on PLC.
Subject(s)
Focal Dermal Hypoplasia/genetics , Membrane Proteins/genetics , Sequence Deletion , Acyltransferases , Asian People , Child, Preschool , Female , Humans , JapanSubject(s)
Antigens, CD/analysis , Antigens, Differentiation, Myelomonocytic/analysis , Diabetes Mellitus, Type 1/immunology , Interleukin-17/analysis , Macrophages/immunology , Necrobiosis Lipoidica/immunology , Receptors, Cell Surface/analysis , Skin Pigmentation , Skin/immunology , Th17 Cells/immunology , Adult , Biopsy , Diabetes Mellitus, Type 1/pathology , Female , Humans , Immunohistochemistry , Necrobiosis Lipoidica/pathology , Predictive Value of Tests , Skin/pathologyABSTRACT
Myeloid sarcoma, which sometimes accompanies recurrent systemic leukemia, is a rare manifestation of congenital leukemia. We describe the case of a 1-month-old Japanese girl with myeloid sarcoma, who did not fulfill the criteria of systemic leukemia. The physical examination revealed a bruise-like plaque on the right side of her chest. Histopathologically, there were CD4+ CD56- CD68+ atypical large lymphocytes densely infiltrating the upper dermis and the subcutaneous tissue. Biopsy specimens from bone marrow contained only 3.4% of blast cells, showing an abnormality of the mixed-lineage leukemia gene. The skin lesions disappeared 10 days after chemotherapy, and there was no recurrence for one and a half years.
ABSTRACT
A 76-year-old woman farmer with a 20-year history of oral steroid treatment for polyarteritis nodosa, noted a rash consisting of numerous small papules on the dorsum of her right hand in February 2007. The rash worsened over time, and in April 2008 red papules and pustules as big as grains of rice fused to form plaques extending up her arm from the dorsum of the right hand to the extensor surface of the forearm. The plaques were accompanied by erosions, ulcers, and crusts. Skin biopsy specimens from the dorsum of the right hand showed a dense cell infiltration in the upper to middle layer of the dermis, with numerous morula-like microorganisms within the cells. Cultures of the specimens on Sabouraud dextrose agar produced creamy, yeast-like colonies. The colonies produced sporangia, with cells of varying sizes arranged in the pattern of a spoked-wheel. The microorganisms were identified as Prototheca wickerhamii on the basis of their morphological characteristics, sugar assimilation patterns, and molecular biological techniques. Treatment with amphotericin B was planned, but the patient died from respiratory failure before initiating treatment. We review the characteristics of the cutaneous protothecosis cases reported to date in Japan.
