Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that manifests as sharply demarcated, telangiectatic, brownish-red plaques with atrophic yellowish centers prone to ulceration and occurs predominantly on the shins. In children, NL is extremely rare, but resistance to therapy, troublesome cosmetic appearance, painful ulcerations, and possible development of squamous cell carcinoma in long-persisting lesions are challenges during treatment. Our review includes 29 reports of NL in patients aged <18 years published from 1990 on PubMed, EMBASE, and Medline. The mean age of patients was 14.3 years, with a female predominance of 2 : 1 and a high prevalence of diabetes mellitus (80%). Data showed that potent topical steroids up to twice daily is the first-line treatment. For refractory cases, therapy can be switched to tacrolimus. Ulcerations benefit from phase-adapted wound care and anti-inflammatory medical dressings such as medical honey. Adding hyperbaric oxygenation to local or systemic therapy in difficult-to-treat ulcerated lesions can be considered. Refractory cases may be switched to topical photochemotherapy or systemic treatment with TNF-α inhibitors, systemic steroids (preferably in non-diabetic patients), pentoxifylline, or hydroxychloroquine. Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.

Complete recovery of a wide local reaction by the use of dexrazoxane 72 hours after epirubicin extravasation: case report and review of the literature.

BACKGROUND: Accidental extravasation of anthracyclines might result in devastating side effects and complications, including necrosis of tissue, with impairment of neighboring structures - most of them requiring surgery. Following recent approach guidelines, intravenous administration of dexrazoxane within 6 h after the event is recommended. CASE REPORT: We report on a 63-year-old female patient with breast cancer treated with epirubicin combination therapy. She experienced extravasation on the distal right arm at the end of the very first cycle, being initially treated with dimethylsulfoxide 99% topically. Clinical symptoms became worse. Despite the interval between the event and referral, dexrazoxane was given once daily for 3 days. She recovered completely without any sequelae. CONCLUSION: To our knowledge, this is the first case report of a successful treatment and complete recovery by the use of dexrazoxane even 3 days after extensive epirubicin extravasation. Additionally, a review of the literature is given.

Prevalence of nevi, atypical nevi, and lentigines in relation to tobacco smoking.

BACKGROUND: Melanocytic nevi have a complex evolution influenced by several endogenous and exogenous factors and are known risk factors for malignant melanoma. Interestingly, tobacco use seems to be inversely associated with melanoma risk. However, the association between tobacco use and nevi and lentigines has not yet been evaluated. METHODS: We investigated the prevalence of nevi, atypical nevi, and lentigines in relation to tobacco smoking in a cohort of 59 smokers and 60 age- and sex-matched nonsmokers, using a questionnaire and performing a total body skin examination by experts. RESULTS: No significant differences were detected between smokers and nonsmokers in the numbers of nevi, atypical nevi, and lentigines in sun-exposed areas (p = 0.966, 0.326, and 0.241, respectively) and in non-sun-exposed areas (p = 0.095, 0.351, and 0.546, respectively). CONCLUSION: Our results revealed no significant differences in the prevalence of nevi, atypical nevi, and lentigines between smokers and nonsmokers in sun-exposed and non-sun-exposed areas.

CXCL13 is an activity marker for systemic, but not cutaneous lupus erythematosus: a longitudinal cohort study.

Serum levels of the IFN-regulated cytokine CXCL13 have been found to correlate with SLEDAI and renal involvement in systemic lupus erythematosus. This study investigates whether CXCL13 can also be a marker of disease activity in patients with subacute cutaneous or chronic cutaneous lupus erythematosus (SCLE, CCLE). We analysed CXCL13 levels in 60 patients' sera (18 SLE, 19 SCLE, 23 CCLE) at five time points within 1 year and correlated these levels with disease activity scores and laboratory markers. Clinical scores with no/mild, moderate or high/severe disease activity were categorized by SLEDAI in SLE, by CLASI in SCLE/CCLE. CXCL13 levels were significantly higher in SLE (median 122.5, IQR 88.0-239.0 pg/ml) than in CCLE patients (median 69.0, IQR 60.0-102.0 pg/ml) (p = 0.006). CXCL13 levels were elevated in 59% (41/70) of SLE patient visits with mild or no disease activity, but in 90% (9/10) with high disease activity. CXCL13 levels correlated with ECLAM, dsDNA-antibodies, and inversely with complement factors C3 and C4 in SLE, and with IgA and ESR in SCLE. In CCLE CXCL13 did not correlate with CLASI or laboratory markers. One SCLE and two CCLE patients with CXCL13 levels > 500 pg/ml had conversion to SLE or an underlying autoimmune disease. CXCL13 seems to be a useful marker of disease activity in SLE, but not in SCLE and CCLE. Conversion from normal to elevated CXCL13 may indicate a flare of SLE. Whether high CXCL13 levels in cutaneous LE indicate the development of SLE should be further investigated.