Ganciclovir-induced drug reaction with eosinophilia and systemic symptoms.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous adverse reaction involving multiorgan failure, with a complex interaction of various drugs, human herpesvirus reactivation and immune abnormalities suggested as the aetiology. We herein present the case of a 70-year-old man with a one-week history of fever, facial oedema, erythematous macules and purpura on his trunk and extremities. He had anti-TIF1γ antibody-positive dermatomyositis and was treated with prednisolone sodium succinate (20 mg/day). Three weeks earlier, he was treated with ganciclovir (250 mg/day) for 7 days to treat asymptomatic cytomegalovirus viraemia. Laboratory investigations revealed eosinophilia with atypical lymphocytes and elevated liver enzyme levels. A histological examination showed interface dermatitis with necrotic keratinocytes, perivascular infiltration of lymphocytes and eosinophils in the upper dermis and erythrocyte extravasation without vasculitis. A lymphocyte transformation test (LTT) was positive for ganciclovir (stimulation index: 260%; normal: <180%). We diagnosed DRESS caused by ganciclovir on the basis of clinical findings and course (Definite; RegiSCAR score: 7). He was treated with prednisolone sodium succinate (40 mg/day) and topical clobetasol propionate (0.05%) ointment twice daily. After the initiation of treatment, the skin lesions and laboratory abnormalities gradually improved. To our knowledge, this is the first case of DRESS caused by ganciclovir. The patients in whom ganciclovir is used are often immunosuppressed and may be overlooked as the causative drug for DRESS by conventional skin tests. We considered that LTT is useful for identifying causative drugs of DRESS, especially in immunosuppressed patients, such as the present case.

Detection of FIP1L1-PDGFRA fusion gene-positive cells in the skin lesion of a patient with hypereosinophilic syndrome.

Hypereosinophilic syndrome (HES) is a heterogeneous group of diseases, characterized by persistent hypereosinophilia and end-organ damage. The FIP1L1-PDGFRA (F/P) fusion gene is found in 3-25% of patients with HES and is an oncogenic driver of myeloid neoplasms with clonal eosinophilia. Although cutaneous symptoms are the most common type of symptom in patients who have F/P fusion gene-positive HES (F/P HES), histological reports are limited. We herein present the case of a 78-year-old man with erythematous macules and severe pruritus on his trunk and extremities. Laboratory investigations revealed marked eosinophilia and elevated serum vitamin B12. A histological examination showed massive infiltration of eosinophils and mast cells around the vessels in the upper dermis. Fluorescence in situ hybridization revealed F/P fusion genes in nuclei in the peripheral blood and the skin lesion. The patient was diagnosed with F/P HES, and showed an excellent clinical and haematological response to imatinib.

Two cases of Hailey-Hailey disease effectively treated with apremilast and a review of reported cases.

Hailey-Hailey disease (HHD) is an autosomal dominant genetic disease caused by a mutation of the ATP2C1 gene. Corticosteroids, antibiotics or cyclosporine have been administered to reduce inflammation and prevent flare-ups, but the efficacy is not always sufficient. We herein report two cases of HHD effectively treated with apremilast and review the previous literature. Patient 1 was a 28-year-old male and patient 2 was a 35-year-old female. Both patients were diagnosed with HHD based on histological and genetic analyses. Both patients were treated with oral antibiotics or topical corticosteroids, but their symptoms were refractory, therefore apremilast was administered to both patients. Two weeks later, the skin lesion of both patients was improved. No adverse reaction was observed except for mild headache in patient 2. There have been 13 reported cases of HHD treated with apremilast, including our cases. Eight cases showed a good response to apremilast, whereas five cases showed no response. There seems to be no association between the disease severity and efficacy of apremilast, although the reason remains unknown. Interestingly, an early improvement of the HHD lesion was observed in all good response cases. Although digestive symptoms, headache, and myalgia were observed as adverse events, the treatment was well-tolerated. The accumulation of a greater number of similar cases and further research will be required. We hypothesize that apremilast may be a useful therapeutic option for skin lesions of HHD.

Treatment of dermatosis papulosa nigra using a carbon dioxide laser.

BACKGROUND: Dermatosis papulosa nigra (DPN) is characterized by the presence of multiple, small, hyperpigmented, warty papules affecting the face, neck, and trunk that bear to histological semblance to seborrheic keratosis. Although the lesions are benign tumors, they can cause distress for cosmetic reasons. OBJECTIVE: We, herein, report the cases of three female Japanese patients (mean age, 46 years) with DPN who were treated using a carbon dioxide (CO2 ) laser with a computerized scanner. The patients were all suffering from an increased number of brown asymptomatic papules (size, 1-5 mm), which were located all over the trunk. Histological examinations revealed acanthosis, hyperkeratosis and horn cyst formation in the epidermis. METHODS: We performed CO2 laser (LASER 30C; Lumenis Inc) treatment (settings: 8-10 W; pulse duration, 0.05-seconds; rest duration, 0.36 seconds; laser spot size, 1.2 mm) for 5-10 months. RESULTS: With CO2 laser treatment, were could completely remove the lesions and achieve excellent cosmetic results without scar formation in all cases. The treated lesions did not relapse for more than 1 year. CONCLUSION: In our opinion, CO2 laser treatment with a computerized scanner is an effective therapeutic option for DPN.

Efficacy of new low-dose oral anticoagulants in recalcitrant livedoid vasculopathy.

Livedoid vasculopathy (LV) is a thrombotic skin disease characterised by recurrent painful ulcerations and irreversible scar formation on the lower legs, which is caused by occlusion of the cutaneous microcirculation. Edoxaban is one of new oral anticoagulants. It directly inhibits factor Xa in the coagulation pathway and prevents thrombus formation. A 17-year-old Japanese male presented with a 1-year history of recalcitrant cutaneous ulcers and livedo racemosa on his lower extremities. Initially, the ulcers were treated with antiplatelets therapies; however, he experienced recurrence of ulcerations during summer time. A histological examination revealed dermal vessel thrombosis consistent with occlusive vasculopathy. These findings were diagnostic for LV. The patient was treated with oral low-dose edoxaban (15 mg/day). The skin ulcers were epithelised and livedo racemosa disappeared within 8 weeks. We herein report the successful treatment of recalcitrant LV with low-dose edoxaban in a patient with no identifiable coagulopathy.