Treatment with pimecrolimus cream 1% clears irritant dermatitis of the periocular region, face and neck.

BACKGROUND: Irritant dermatitis of the face and neck is particularly prevalent in women > or = 30 years old, who typically present with periocular cutaneous symptoms. Current therapies are limited, indicating a need for rapid, effective alternatives. Pimecrolimus cream 1%, a nonsteroid, cell-selective inhibitor of inflammatory-cytokine release, is effective in the treatment of inflammatory skin diseases, such as chronic irritant dermatitis of the hands, and thus offers a potential therapeutic option for this indication. This study reports on the safety and efficacy of pimecrolimus treatment in patients with irritant periocular dermatitis, extending to the face and neck in some patients. METHODS: Twenty-seven patients with periocular irritant dermatitis (extending onto the face and neck in eight) were treated twice daily with pimecrolimus cream 1% for 7 d, followed by once-daily application for a further 7 d. Erythema, swelling, and pruritus were assessed at baseline, weeks 1-4 using a 4-point clinical score (0, absent; 1, mild; 2, moderate; and 3, severe). RESULTS: All patients showed marked improvement within 2-3 d of treatment with disease clearance in 23 of 27 patients within 14 d. In the remaining four patients, mild relapse occurred at weeks 3-4, but improvement was observed following a further 10-d treatment. Side-effects were mild and transient. CONCLUSION: Pimecrolimus cream 1% provides a new potential option for treatment of irritant dermatitis of the periocular region, head and neck. Further double-blind, controlled studies are required to confirm the efficacy and safety of pimecrolimus cream 1% for this indication.

[Necrolytic migratory erythema in glucagonoma syndrome]. / Erythema necroticans migrans bei Glucagonomsyndrom.

The glucagonoma syndrome is a rare disease in which a typical skin lesion, necrolytic migratory erythema, is often one of the presenting symptoms. A 68-year-old woman developed erythematous polycyclic migratory lesions with advancing scaling borders and crusts over several years. Skin biopsies, laboratory studies and imaging confirmed the diagnosis of necrolytic migratory erythema as part of a glucagonoma syndrome.

[Skeletal and dermatological manifestations of the nevoid Basal cell carcinoma syndrome (Gorlin-Goltz syndrome). Results of 8 patients in 12 years]. / Skelettale und kutane Charakteristika des nävoiden Basalzellkarzinomsyndroms (Gorlin-Goltz-Syndrom). Ergebnisse von 8 Patienten aus 12 Jahren.

PURPOSE: In 1960 Gorlin and Goltz defined the nevoid basal cell carcinoma syndrome (NBCCS, Gorlin-Goltz Syndrome) as a syndrome comprising multiple basal cell carcinoma, odontogenic keratocysts, and skeletal anomalies. NBCCS is an autosomal dominantly inherited disease with an estimated prevalence of 1:150,000 and diagnosis of this syndrome is often an accidental finding of radiological investigations. The purpose of this study was to report the varied radiological and dermatological manifestations of our patients affected with NBCCS and to present this rare syndrome as a differential diagnosis of skeletal anomalies. MATERIALS AND METHODS: Between 1994 and 2005 the demographic, clinical, radiological and histological data of 8 patients with NBCCS were retrospectively analyzed. Nevoid basal cell carcinoma syndrome was diagnosed in the event of two major or one major and two minor criteria. The major criteria are more than 2 basal cell carcinoma, odontogenic keratocysts, three or more palmar pits, and calcification of the falx cerebri. RESULTS: Between 1994 and 2005 8 patients (3 females and 5 males) with NBCCS were treated in our departments. The average age at the time of diagnosis of NBCCS was 49.9 years. All patients had a minimum of two major criteria. The major criteria with the most frequency were the basal cell carcinoma (6 patients) and the odontogenic keratocysts (5 patients), followed by the calcification of the falx cerebri and palmoplantar pits (4 patients). There was no gender-related or age-related predilection and only one patient was affected with pain in his fingers which radiologically correlated to small cystic bone lesions ("flame-shaped lucencies"). CONCLUSION: Due to limitations in identification of mutations in the PTCH1 gene, clinical and radiological examination still remains a very important factor in the treatment of patients suffering from NBCCS. The knowledge of the varied skeletal manifestations and constellations is therefore essential and correlates with therapeutic consequences. Often chest, rib, spine, skull, and jaw X-rays show the way. Due to the risk of the development of an associated medulloblastoma, neurological surveillance in 6-month intervals in addition to an annual MRI of the cerebrum up to an age of 7 is strongly recommended.

Reactive angioendotheliomatosis: is it 'intravascular histiocytosis'?

We report the case of a 68-year-old female with reactive angioendotheliomatosis (RAE). This case highlights the benign course of this condition and suggests that this entity might be an intravascular histiocytosis.