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Bullous pemphigoid (BP) is a cutaneous autoimmune blistering disorder. Recently, it has been reported that dipeptidyl peptidase-4 inhibitors (DPP4i) is associated with the development of BP (DPP4i-BP). Patients with DPP4i-BP have autoantibodies (autoAbs) preferentially targeting full-length BP180, but not the BP180NC16a domain. In this report, we described a case of anti-BP230 antibody (Ab)-positive BP receiving DPP4i. A 78-year-old male with a medical history of type 2 diabetes receiving vildagliptin at 100 mg daily for 38 months was referred to our hospital with itching blisters on his body and extremities. Skin biopsy showed subepidermal bulla with eosinophil infiltration. Direct immunofluorescence staining revealed a linear staining pattern with complement C3 and IgG at the subepidermal basement membrane zone. By laboratory testing, autoAbs against BP180NC16a and full-length BP180 were negative by enzyme-linked immunosorbent assay (ELISA); however, anti-BP230 Abs were positive by ELISA (index: 123.91). His HLA genotype was DQB1*04:01 and 05:01. Based on these results, we diagnosed the patient with anti-BP230 Abs-positive BP associated with DPP4i. To the best of our knowledge, this is the first case of DPP4i-BP with only anti-BP230 Abs. Further accumulation of DPP4i-BP cases is needed to clarify the relationship between overall features of DPP4i-BP and anti-BP230 Abs.

A topical application of a nonsteroidal anti-inflammatory drug (NSAID) may induct an adverse reaction of photoallergic contact dermatitis. The occlusive usage may provoke concomitant photoallergic sensitizations to an NSAID and other ingredients. We describe a 58-year-old woman with photoallergic contact dermatitis from ketoprofen and hydrogenated rosin glycerol ester in the applied compress. Our case indicates that photopatch testing with all ingredients is required to verify the actual photoallergen(s).

Fournier’s gangrene is a life-threatening disorder caused by aerobic and anaerobic bacterial infection. We report a case of genital infection as the initial warning sign of acute myeloid leukemia. We were able to prevent progression to Fournier’s gangrene in our patient by immediate intensive therapy with incision, blood transfusions and intravenous administration of antibiotics. This case suggests that hematologists and dermatologists should keep in mind that genital infection can be a first sign of hematologic malignancy.

Atypical features of scabies occur in infants and children and patients with prolonged use of corticosteroids or immunosuppression. We report a non-immunosuppressed 15-year-old female case of scabies showing scaly reddish papules over the proximal interphalangeal joints mimicking Gottron papules in classic dermatomyositis. Periungal erythema was also seen. Four months’ topical corticosteroids from previous clinics had been used. Dermoscopic findings were consistent with typical pictures of scabies. Scraping of hand crusts demonstrated scabies mites and ova. Skin lesions of the patient were cured with oral ivermectin and topical 10% crotamiton. This case suggests that a lesion resembling Gottron papules may be added to the panel of unusual presentations of scabies.

Skin fibrotic disorders such as systemic sclerosis (SSc) are characterized by an excessive production of extracellular matrix (ECM) and understood to develop under the influence of certain growth factors. Connective tissue growth factor (CTGF) is a cysteine-rich mitogenic peptide that is implicated in various fibrotic disorders and induced in fibroblasts after activation with transforming growth factor- (TGF-). To better understand the mechanisms of persistent fibrosis seen in SSc, we previously established an animal model of skin fibrosis induced by exogenous application of growth factors. In this model, TGF- transiently induced subcutaneous fibrosis and serial injections of CTGF after TGF- caused persistent fibrosis. To further define the mechanisms of skin fibrosis induced by TGF- and CTGF in vivo, we investigated in this study, the effects of growth factors on the promoter activity of the proa2 (I) collagen (COL1A2) gene in skin fibrosis. For this purpose, we utilized transgenic reporter mice harboring the -17 kb promoter sequence of the mouse COL1A2 linked to either a firefly luciferase gene or a bacterial -galactosidase gene. Serial injections of CTGF after TGF- resulted in a sustained elevation of COL1A2 mRNA expression and promoter activity compared with consecutive injection of TGF- alone on day 8. We also demonstrated that the number of fibroblasts with activated COL1A2 transcription was increased by serial injections of CTGF after TGF- in comparison with the injection of TGF- alone. Furthermore, the serial injections recruited mast cells and macrophages. The number of mast cells reached a maximum on day 4 and remained relatively high up to day 8. In contrast to the kinetics of mast cells, the number of macrophages was increased on day 4 and continued to rise during the subsequent consecutive CTGF injections until day 8. These results suggested that CTGF maintains TGF--induced skin fibrosis by sustaining COL1A2 promoter activation and increasing the number of activated fibroblasts. The infiltrated mast cells and macrophages may also contribute to the maintenance of fibrosis. 2004 Wiley-Liss, Inc. [ABSTRACT FROM AUTHOR]