Primary hemochromatosis presented by porphyria cutanea tarda: a case report.

We present a 27-year-old female Caucasian patient, who initially presented with extensive fragility and blistering of mainly the dorsal side of both hands. Histology and urine porphyrin analysis confirmed the diagnosis of porphyria cutanea tarda. Internal screening for underlying disease revealed C282Y mutation-associated primary hemochromatosis, a hereditary iron-overload syndrome that may cause toxicity of a variety of organs. Hemochromatosis and porphyria cutanea tarda are pathogenetically linked as iron interferes with heme synthesis pathway. Patient was successfully treated with phlebotomy and low-dose hydroxychloroquine.

Scabies outbreaks in nursing homes for the elderly: recognition, treatment options and control of reinfestation.

The scabies mite is an ectoparasite able to infest humans. Its clinical presentation is typical, although in immunocompromised, mentally retarded and elderly patients the clinical presentation may be altered. Diagnosis may therefore be difficult in such patient groups, who often reside in nursing homes. Because delay in diagnosis may induce rapid spread of the scabies mite, immediate diagnosis and treatment are necessary. Normal scabies (scabies vulgaris) and crusted scabies (scabies crustosa, scabies norvegica), although sometimes difficult to diagnose, especially in the elderly, are fortunately quite easy to treat. However, the elderly patient may experience toxicity from local or systemic scabicidal treatment. Single cases of scabies vulgaris should be treated with permethrin cream because of its outstanding efficacy and favourable adverse events profile. Scabies outbreaks and cases of scabies crustosa can easily be managed using combination therapy consisting of topical application of permethrin and two oral doses of ivermectin 200 microg/kg (administered 1 week apart). In addition to treatment of the scabies infestation, preventative measures are necessary, particularly in nursing homes.

Topical macrolide immunomodulators: a role in the treatment of vitiligo?

Recently, topical macrolide immunomodulators have been successfully introduced in the treatment of atopic dermatitis. With the growing interest in this new line of topical immunosuppressants, research into the efficacy of these medicines in other T-cell-mediated skin diseases, such as psoriasis, lichen planus, and even vitiligo, is expanding rapidly. It is generally accepted that autoimmune factors play an important role in vitiligo. In this article, the possible use and mechanism of topical macrolide immunomodulators in the treatment of vitiligo are discussed, together with the current state of clinical studies and case reports. These limited reports indicate that topical macrolide immunomodulators may play a role in the treatment of vitiligo, particularly in areas where use of potent corticosteroids is contraindicated.

Dermatological conditions during TNF-alpha-blocking therapy in patients with rheumatoid arthritis: a prospective study.

Various dermatological conditions have been reported during tumor necrosis factor (TNF)-alpha-blocking therapy, but until now no prospective studies have been focused on this aspect. The present study was set up to investigate the number and nature of clinically important dermatological conditions during TNF-alpha-blocking therapy in patients with rheumatoid arthritis (RA). RA patients starting on TNF-alpha-blocking therapy were prospectively followed up. The numbers and natures of dermatological events giving rise to a dermatological consultation were recorded. The patients with a dermatological event were compared with a group of prospectively followed up RA control patients, naive to TNF-alpha-blocking therapy and matched for follow-up period. 289 RA patients started TNF-alpha-blocking therapy. 128 dermatological events were recorded in 72 patients (25%) during 911 patient-years of follow-up. TNF-alpha-blocking therapy was stopped in 19 (26%) of these 72 patients because of the dermatological event. More of the RA patients given TNF-alpha-blocking therapy (25%) than of the anti-TNF-alpha-naive patients (13%) visited a dermatologist during follow-up (P < 0.0005). Events were recorded more often during active treatment (0.16 events per patient-year) than during the period of withdrawal of TNF-alpha-blocking therapy (0.09 events per patient-year, P < 0.0005). The events recorded most frequently were skin infections (n = 33), eczema (n = 20), and drug-related eruptions (n = 15). Other events with a possible relation to TNF-alpha-blocking therapy included vasculitis, psoriasis, drug-induced systemic lupus erythematosus, dermatomyositis, and a lymphomatoid-papulosis-like eruption. This study is the first large prospective study focusing on dermatological conditions during TNF-alpha-blocking therapy. It shows that dermatological conditions are a significant and clinically important problem in RA patients receiving TNF-alpha-blocking therapy.

Selective persistence of dermal CD8+ T cells in lesional plaque psoriasis after clobetasol-17 propionate treatment.

In psoriasis, T-cell infiltration and epidermal hyperproliferation are key phenomena which are closely related. Our aim was to investigate the dynamics among T-cell subsets in relation to epidermal proliferation and clinical severity in psoriasis during treatment with an ultra-potent corticosteroid. Seven psoriasis patients were treated twice daily for 14 days with clobetasol-17 propionate ointment. Punch biopsies were taken at day 0, 3, 7 and 14. Epidermal proliferation marker Ki-67 and CD4+, CD8+, CD45RO+, CD2+ T cells were quantified by immunohistochemical techniques and image analysis. The clinical score declined significantly (60%; p<0.01) and a 47% reduction of Ki-67+ nuclei was observed after only 3 days (p<0.01). In the epidermis all investigated T-cell subsets were significantly reduced at day 14 (p<0.05). In the dermis, treatment resulted in a significant decrease of CD4+, CD45RO+ and CD2+ T cells, but dermal CD8+ T cells persisted. In psoriasis, reduction of clinical severity and epidermal proliferation during the early phase of topical corticosteroid therapy cannot primarily be the result of decreased T-cell subsets. Furthermore, selective persistence of dermal CD8+ T cells was observed, which might be associated with disease relapse.

Lack of upregulation of epidermal fatty acid binding protein in dithranol induced irritation.

The exact role of epidermal fatty acid binding protein (E-FABP) in skin is unknown. A restoration of the barrier function may be associated with an upregulation of E-FABP. Moreover, E-FABP is upregulated in a variety of cells in response to oxidative stress. A recent observation that dithranol induced irritation is not associated with skin barrier impairment prompted us to investigate the expression of E-FABP in this skin condition to elucidate the unknown function of this protein in skin. This study shows lack of E-FABP upregulation after a single application of dithranol on uninvolved skin of patients with psoriasis. The expression of E-FABP in dithranol irritation correlates with the unimpaired skin barrier function as assessed by measurements of TEWL. Furthermore, we did not find evidence for the recently introduced hypothesis that E-FABP functions as an antioxidant protein in the skin irritation induced by dithranol as oxidative stressor.