A b s t r a c tIntroduction: There are many surgical methods for vitiligo treatment that have been used for over 30 years. Suction blister epidermal grafting (SBEG) is considered one of the simplest and most effective of them. Aim: To determine how effective suction blister grafts with concomitant phototherapy are in vitiligo treatment. Material and methods: The study was conducted on 10 patients with vitiligo that was resistant to previous treatment including phototherapy in monotherapy. Involvement of affected sites was different for every patient. We used cryotherapy for blistering at the recipient site and an automatic suction device for blistering at the donor site. The blister was separated from the donor site and fixed with dressing to the recipient site. After removing the final dressing (about 7 days after SBEG) patients started phototherapy (6 patients had UVB 311 nm and 4 had PUVA). All patients treated with UVB 311 nm were qualified for treatment in our clinic and the method was chosen according to expert recommendations from the European Dermatology Forum (EDF) Guidelines for Vitiligo where narrowband (NB) UVB is the phototherapy of choice. Three patients who had PUVA therapy were treated with this method in other clinical centers and sent to us only to undergo SBEG. One patient had previously received UVB 311 nm for 3 months, which showed no effects. Repigmentation of lesions was evaluated at 3 and 6 months after the surgical procedure. Results: Ten patients (9 females with a mean age of 36.88 years and 1 man aged 39 years) were enrolled in the study. Nine patients showed progressive repigmentation at 3 and 6 months follow-up with a rate varying from 13 to 76% (mean: 44.5%) and 35 to 100% (mean: 67.5%). One patient showed 5% depigmentation at a visit after 6 months in comparison to the follow-up visit 3 months after SBEG. Conclusions: With this technique, patients who did not respond to the usual treatments showed very good repigmentation over a 6-month follow-up. There were no side effects such as scarring.

Topical glicocorticosteroids are the most common drugs to treat acute and chronic inflammatory skin diseases. Prolonged use of them may cause systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal axis suppression. We present a case of four year old girl who developed iatrogenic Cushing syndrome and adrenal insufficiency after atopic dermatitis treatment through the misuse of Mometasone treatment without doctor's prescription. We observe a reddness and a moon face, a buffalo hump, central obesity, ginecomasty, subcutaneous hypertrophy, hirsutism, buttocks muscle atrophy and growth retardation. Wrist X-Ray revealed a bone age of two year old child. Laboratory values revealed hypothalamic-pituitary-adrenal axis suppression. The discontinuation of Mometasone treatment and supplement treatment with oral Hydrocotisone three times per day proved successful in this patient. For this case, the serious side effects of topical glucocorticosteroid treatment should be explained to the family and their long-term therapy should be refrained. Iatrogenic Cushing syndrome in childchood caused by topical treatment is a rare event.

StreSzczenieWprowadzenie. Zespół Comèla-Nethertona jest rzadką, dziedziczoną autosomalnie recesywnie genodermatozą charakteryzującą się występowaniem wrodzonej erytrodermii ichtiotycznej, rybiej łuski linijnej okrążającej, obecnością włosów bambusowatych i skazy atopowej. Zespół ten spowodowany jest mutacją w genie SPINK 5, kodującym inhibitor proteazy serynowej LEKTI. Cel pracy. W pracy przedstawiono trudności diagnostyczne i terapeutyczne u pacjenta z rozpoznaniem zespołu Comèla-Nethertona. Opis przypadku. Przedstawiamy przypadek 3-letniego chłopca, u którego od pierwszej doby życia obserwowano uogólnioną erytrodermię ichtiotyczną i rozlane, grubopłatowe złuszczanie naskórka. Diagnostyka różnicowa obejmowała zespół Omenna, atopowe zapalenie skóry, łojotokowe zapalenie skóry oraz niepęcherzową wrodzoną erytrodermię ichtiotyczną. Ostatecznie na podstawie badania mikroskopowego, w którym wykazano obecność włosów bambusowatych, oraz całokształtu obrazu klinicznego rozpoznano zespół Comèla-Nethertona. Wnioski. Ze względu na podobieństwo do innych erytrodermii rozpoznanie zespołu Comèla-Nethertona w pierwszych miesiącach życia stwarza problemy, a u starszych dzieci nawracające infekcje skóry oraz nasilenie skazy atopowej są przyczyną trudności terapeutycznych i wymagają współpracy wielu specjalistów. AbStrActIntroduction. Comèl-Netherton syndrome is a rare autosomal recessive genodermatosis characterized by congenital ichthyosiform erythroderma, ichthyosis linearis circumflexa, trichorrhexis invaginata, and atopic diathesis. Comèl-Netherton syndrome is caused by mutations of the SPINK5 gene, which encodes the serine protease inhibitor LEKTI. Objective. We present diagnostic and therapeutic difficulties in a patient with Comèl-Netherton syndrome. Case report. We present the case of a 3-year-old boy, in whom from the first day of life generalized ichthyosiform erythroderma and diffuse exfoliation of the skin were observed. The differential diagnosis included Omenn syndrome, and atopic and seborrheic dermatitis. Finally, based on the overall clinical picture and microscopic examination of the hair, which showed the presence of bamboo hair, Comèl-Netherton syndrome was diagnosed. Conclusions.Because of similarity to other erythroderma, diagnosis of Comèl-Netherton syndrome in the first months of life creates diagnostic zespół comèla-nethertona -opis przypadku comèl-netherton syndrome -case report izabela błażewicz, Alicja rustowska, Aleksandra Wilkowska, roman J. nowicki Katedra i Klinika Dermatologii, Wenerologii i Alergologii Gdańskiego Uniwersytetu Medycznego