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Notes: Aim:  This study was undertaken to assess whether heat-shock protein (HSP)47 is a useful cell marker for skin fibroblasts in formalin-fixed, paraffin-embedded skin specimens.Background:  HSP47, a 47-kDa HSP, is a collagen-specific molecular chaperone localized in the endoplasmic reticulum. HSP47 plays an essential role in collagen biosynthesis in skin fibroblasts.Methods:  Immunohistochemistry was performed to detect HSP47 in skin fibroblast cultures and skin tissue sections.Results:  Immunostaining for HSP47 clearly detected skin fibroblasts in paraffin tissue sections as well as in fibroblast cultures and frozen tissue sections. HSP47 staining on paraffin sections from diseased skin specimens revealed that skin ulcer, keloid, nodular fascitis, spindle cell lipoma, and dermatofibroma had strong signals for HSP47 compared with the signals obtained from normal skin. Dermatofibrosarcoma protuberans had many HSP47 positive cells, but signals on individual cells were not as strong as those seen from the above benign proliferative disease samples. In neurofibroma, a small number of faintly positive cells were detected. Our double-immunostaining studies also demonstrated that HSP47 staining distinguished skin fibroblasts from CD68-positive histiocytes/macrophages, factor VIII-related antigen-positive endothelial cells, or factor XIIIa-positive dermal dendritic cells. CD34-positive interstitial cells coexpressed HSP47 in spindle cell lipoma.Conclusions:  These findings indicate that HSP47 staining can detect skin fibroblasts in routine, paraffin-embedded specimens. A panel approach using HSP47 and other cell markers on paraffin sections may help the identification of the cell type involved with mesenchymal proliferative disorders.

Notes: Summary Background Pemphigus is an antidesmoglein (Dsg) autoimmune disease that is divided into two major subtypes: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). We previously developed enzyme-linked immunosorbent assays (ELISAs) using recombinant Dsg1 and Dsg3 to detect IgG autoantibodies in patients with pemphigus. The protocol for the ELISAs was optimized for serological diagnosis, but under the conditions used, these assays were not particularly useful for monitoring disease activity in certain patients. That is, the sera from some patients with high-titre antibodies continued to show high index values in the ELISA after clinical improvement. Objectives In the study reported here, we modified the ELISA protocol to obtain ‘true’ index values that exhibit a better correlation with disease activity. Methods We tested two cases of pemphigus foliaceus (PF) and four cases of pemphigus vulgaris (PV), each with ELISA index values greater than 150 for Dsg1 or Dsg3. We ran an ELISA with sera from these patients serially diluted from 1 : 100 to 1 : 12,800. We then performed ELISA with a series of PV No. 1 sera diluted to 1 : 800 and PV No. 2–4 and PF No. 1–2 sera diluted to 1 : 1600, after which we plotted the ELISA index values against the time course of disease activity. Results In each of these cases, there was no apparent decline, over the course of the disease activity, in the ELISA index values at a serum dilution of 1 : 100, probably because the antigen–antibody reaction was saturated at that dilution. After running an ELISA with sera serially diluted from 1 : 100 to 1 : 12,800 we found that a linear dose-dependency between the dilution value and the index value was only observed when sera were diluted to 1 : 800 or more in one case (PV No.1) and to 1 : 1600 or more in the other five cases (PV No. 2–4, PF No. 1–2). After performing ELISA with these series as outlined above we plotted the ELISA index values against the time course of disease activity and found that the index values obtained from these appropriately diluted sera fluctuated in parallel with disease activity, and declined with clinical improvement. Conclusions These findings indicate that when appropriate dilutions are used in Dsg1 and Dsg3 ELISA, these assays can provide useful serological information for assessing disease activity in PF and PV.

Notes: We report a 58-year-old patient with acute myeloid leukaemia who developed an oedematous erythema resembling Sweet's syndrome, accompanied by atypical erythema nodosum and bullous pyoderma gangrenosum. Examination of skin biopsies showed dense infiltration with mature neutrophils. although there was peripheral blood leucocytopenia. The oedematous erythema worsened after he was treated with granulocyte-colony-stimulating factor (G-CSF), which was given For his leucocytopenia. We suggest that when a neutrophilic dermatosis complicates leukaemia, alternatives to G-CSF should be considered for the treatment of leucocytopenia. Corticosteroids were very effective in controlling the skin lesions in our patient.

Notes: A 42-year-old man presented with painful erythema with pustules and multiple small ulcers on the shins. He had suffered from ulcerative colitis (UC) and received oral glucocorticosteroid and salicylazosulfapyridine therapies for 7 years. Biopsy of the lesion demonstrated mixed cellular infiltrates with dominant neutrophils. The patient was diagnosed with pyoderma gangrenosum (PG) and underwent leukocytapheresis (LCAP), an extracorporeal leucocyte removal therapy, once a week for 5 weeks without changing the doses of the oral medications. The skin lesions as well as clinical signs of UC rapidly improved after LCAP, and no recurrence was seen during a follow-up period. There were no major complications during LCAP. LCAP will provide an effective and safe tool for the treatment of PG.

Notes: Summary We report a patient with melorheostosis in whom increased procollagen α1(I) mRNA expression and α1(I), α2(I) and α1(III) collagen secretion were observed in dermal fibroblasts obtained from a skin biopsy overlying the involved bone. The patient was a 53-year-old man with melorheostosis lesions over the left knee joint. Multiple pigmented macules were present on the medial aspect of the lower left leg. Hyperpigmentation of the basal keratinocytes, thick-walled vessels in the reticular dermis, and proliferation of normal-appearing collagen around the hair follicles were observed histologically.

Notes: Summary Acute haemorrhagic oedema (AHO) of infancy is a cutaneous leukocytoclastic vasculitis, clinically characterized by the acute development of peripheral oedema and targetoid purpuric lesions on the face and extremities. It usually affects children younger than 2 years of age. The disorder follows a benign course usually without recurrence or long-term complication. In most cases the origin is not clear, but underlying infections are assumed to play an aetiological role. We describe a 7-month-old boy whose clinical and histopathological features are typical of AHO. Serological tests clearly demonstrated a primary infection for cytomegalovirus (CMV). To our knowledge, this is the first reported case of AHO associated with CMV infection.