Andrea Carugno

Narrowband UVB phototherapy for pediatric generalized pityriasis lichenoides

To the Editor, Pityriasis lichenoides (PL) is an uncommon acquired skin disease of unknown etiology characterized by a spectrum of clinical manifestations ranging from the onset of acute papular lesions that evolve into pseudovesicles with central necrosis (pityriasis lichenoides et varioliformis acuta, PLEVA) to the presence of small scaly brown papules (pityriasis lichenoides chronica, PLC), including forms with intermediate and overlapping characteristics (1–3). The incidence of PL is estimated at about 1 in 2000 people, and 20% of the cases affect children, with peak incidence around the 5th and 10th years (2, 3). The etiology and pathogenesis are not well defined; it is believed that the mechanism lies in a possible atypical immune response in genetically susceptible individuals (2, 3). The most cited pathogenetic hypothesis proposes a hypersensitivity reaction triggered by infectious agents (Toxoplasma gondii, Epstein–Barr virus, HIV, adenovirus, Staphylococcus, Mycoplasma, varicella zoster virus, cytomegalovirus, parvovirus B19, Streptococcus, hepatitis C virus), and it is supported by the finding that some cases show benefit from antibiotic therapy or have positive serology for infectious agents (2, 3). PL lesions may present autoresolution over several weeks or months and may benefit from the use of topical corticosteroids. Antibacterial agents such as tetracycline and erythromycin have shown reasonable utility in PL, as foreign antigen is one of the suspected triggers and because of their antiinflammatory properties (2). Erythromycin is a better choice in the pediatric population because of the possible adverse dental effects of tetracycline (2). For more extensive and/or resistant forms, it may be useful to use systemic steroids, immunosuppressants (methotrexate, cyclosporine), retinoids, or photo/photochemotherapy (2–4). In particular, photo/photochemotherapy is by far the most successful therapy, and is considered the first-line therapy for generalized or recurrent PL (2). It is used in various modalities: broadband UVB phototherapy (BBUVB; 290–320 nm), narrowband UVB phototherapy (NB-UVB; 311 nm), psoralen–UVA photochemotherapy (PUVA; 320–400 nm), and UVA-1 (340–400 nm) (4). NB-UVB phototherapy is a therapeutic modality with a well-known efficacy and safety profile, which makes it particularly suitable for the treatment of childhood skin diseases. The current knowledge about its use in pediatric PL patients is limited but encouraging (1–5). We describe our experience with five cases of pediatric patients with generalized PL resistant to common therapies who were treated with NB-UVB phototherapy. Pediatric patients with generalized PL, unsuccessfully treated with topical steroids and/or antibiotics and referred to the photo/photochemotherapy unit at the Institute of Dermatology of the Fondazione IRCCS Policlinico S. Matteo in Pavia, Italy, from 1 January 2010 to 31 December 2012, were included in the study. Five patients with generalized PL were studied (1 boy and 4 girls; mean age 10.4 years, range 7–15) (Table 1). Two patients were affected by PLEVA and three by PLC. Each patient underwent a skin biopsy for histological examination, which confirmed the clinical suspicion. All the patients were treated with NB-UVB phototherapy. Photodermatology, Photoimmunology & Photomedicine

Risk of acute postoperative hypertension after topical photodynamic therapy for non-melanoma skin cancer

Topical photodynamic therapy with methyl aminolevulinate (MAL‐PDT) is a non‐surgical treatment for actinic keratoses, Bowens disease and basal cell carcinoma. MAL‐PDT is particularly useful in elderly patients, who often present co‐morbidities and/or in whom surgical excision could be contraindicated. MAL‐PDT is generally well tolerated; the most frequent acute adverse events include pain and burning sensation localized to the treatment area. We describe our observation of the occurrence of acute postoperative hypertension (APH) and hypertensive crisis, after a MAL‐PDT.

Cutaneous blastic plasmacytoid dendritic cell neoplasm: successful palliative treatment with oral prednisone in an elderly patient.

no specific reactivity for epidermal proteins. These findings may mean that autoimmunity mediated by a monoclonal IgA-j antibody against an unknown epidermal antigen may exhibit an affinity that is not enough to be detected by ELISA or immunoblotting. Another possibility is that the unknown antigen may be modified during the molecular procedure, giving rise to negative results. Further investigations are needed to define the specificity and significance of these results.

Ciclosporina-A e week-end therapy nella psoriasi a placche: la nostra esperienza

L’efficacia della Ciclosporina-A (CsA) nel trattamento della psoriasi e nota da 30 anni. La sua maggiore limitazione e pero correlata al rischio di eventi avversi quali l’ipertensione arteriosa e l’alterazione della funzionalita renale che sembrerebbero non solo dose-dipendenti ma anche proporzionali alla durata della terapia. Pertanto, la possibilita di formulare uno schema di trattamento in grado di controllare efficacemente le manifestazioni cliniche nel lungo termine con un profilo di sicurezza accettabile risulta sempre piu importante al fine di migliorare la qualita della vita dei pazienti affetti da psoriasi. Scopo del nostro studio e la valutazione di una terapia con CsA somministrata solo due giorni alla settimana da protrarre su lunghi periodi per il controllo delle recidive di psoriasi. Il nostro studio prende spunto dalla week-end therapy che si e dimostrata efficace nel mantenimento della remissione a lungo termine ed e stata ben tollerata da tutti i pazienti considerati.

Usefulness of in vivo photodiagnosis for the identification of tumor margins in recurrent basal cell carcinoma of the face

Basal cell carcinoma (BCC) is the most frequent malignant tumor of the skin. The high prevalence of BCC, the risk of local recurrence, and the difficult clinical identification of the excision margins emphasize the importance of studying new approaches, ensuring complete surgical excision that allows preservation of normal tissue, especially for BCCs located on cosmetically important areas such as the mid face. Photodiagnosis (PD) is a pre‐operative technique that allows a more accurate distinction of neoplastic lesions from surrounding healthy skin in vivo.

Pompholyx of the hands after intravenous immunoglobulin therapy for clinically isolated syndrome: A paediatric case

Pompholyx is a common eruption of small vesicles on the palms, soles, and/or lateral aspects of the fingers. It has a multifactorial etiology, including genetic determinants, allergy to metals, and id reaction; rarely it is a drug-related side effect. We report a paediatric case of pompholyx of the hands related to the intravenous immunoglobulin (IVIG) therapy for Clinically Isolated Syndrome (CIS). A 10-year-old boy, received an IVIG therapy (Venital®, Kedrion Spa, Italy) at a dose of 400 mg/kg daily for five days. The fifth day of IVIG infusion, a symmetrical vesicular eruption appeared on the palms of the hands and on lateral aspects of the fingers. The lesions improved with application of topical steroids in few days. The mechanism of induction of pompholyx by IVIG therapy is unknown. A review of the Literature suggests the hypothesis that dyshidrotic eczematous reactions may be related not only to the type of IVIG, to the dose and the rates of infusion, but also to an allergic response to excipients and preservatives contained in the drug, probably elicited by an underlying neurological disease in some cases.

Efficacia della terapia con propranololo negli emangiomi infantili: studio di un caso difficile

The infantile hemangiomas are the most common tumor of childhood, occurring in 4-5% of children under the age of one year and in 10% of Caucasian children. Most of them appear in the first four weeks of life, in a minority of cases the lesions are already present at birth. The disease is more frequent in females than in males, with a ratio of 3:1. The lesions are characterized by a rapid neonatal growth, followed by a phase of slow regression, with disappearance of the lesions within the first few years of life. Although the benign capillary hemangiomas have a tendency to spontaneous involution their management should be carefully considered. In most cases the preferred approach is wait and see, but in cases of problematic hemangiomas with complications risk, a therapeutic approach should be taken into consideration. For over forty years corticosteroids have been used as first-line therapy, with vincristine and interferon alpha. However all these drugs had many side effects. After the discovery of the usefulness of propranolol in the treatment of infantile hemangiomas numerous studies have confirmed the efficacy of the drug in inducing regression of hemangiomatous lesions, with no evidence of significant side effects. We present the case of a patient with a problematic hemangioma treated with propranolol, showing a steady regression of the lesion after 12 months of total treatment.

Acne Fulminans e sindrome SAPHO: studio di un caso

L’acne fulminans (AF) e una rara grave forma di acne vulgaris associata a sintomi sistemici. Essa colpisce prin-cipalmente adolescenti di sesso maschile. Sebbene l’eziologia dell’AF rimanga sconosciuta, molte teorie sono state avanzate: ipersecrezione androgenetica, presenza di immunocomplessi e predisposizione genetica. La ma-lattia si presenta con l’insorgenza acuta di noduli dolenti, ulcerati sul viso, petto e dorso e franche raccolte asces-suali. Le manifestazioni sistemiche associate sono febbre e dolore muscolo-scheletrico, di solito presenti gia al momento dell’esordio con perdita di peso. Il trattamento dell’AF e difficile poiche la risposta alle tradizionali terapie per l’acne e scarsa. Le linee guida raccomandano un trattamento aggressivo con una combinazione di steroidi orali e isotretinoina. Presentiamo il caso di un giovane adolescente che ha sofferto di AF ed e stato trat-tato efficacemente con associazione di isotretinoina e prednisone. Successivamente ha sviluppato una sacroileite con grosse difficolta alla deambulazione e con intenso dolore, interpretata come sindrome SAPHO (sinovite, acne, pustolosi, iperostosi e osteite). L’associazione tra AF e sindrome SAPHO e molto rara e comunque sono entrambe considerate nello spettro delle patologie autoimmuni reumatologiche; e pertanto richiesto un approccio multidisciplinare per una corretta gestione diagnostico-terapeutica.

Dalla terapia con emollienti e idratanti ai tessuti dedicati alla dermatite atopica: revisione della letteratura

La dermatite atopica (DA) e una malattia cronica infiammatoria della cute che colpisce il 20% dei bambini e quasi il 3% degli adulti, caratterizzata da secchezza cutanea e prurito intenso. Nella fisiopatologia della dermatite atopica rivestono un ruolo fondamentale le anomalie strutturali della barriera epidermica, le alterazioni immunitarie (in particolare la risposta anomala da parte dei linfociti T-helper 2 con conseguente iperproduzione di Ig E) e l’anomala colonizzazione microbica da parte di microrganismi patogeni. In base alla gravita clinica della DA possono essere utilizzate diverse terapie: terapie di base, come gli emollienti e gli idratanti (nelle forme lievi), corticosteroidi topici, farmaci antistaminici e antimicrobici, immunosoppressori sistemici, farmaci biologici, terapie adiuvanti come la fototerapia (nelle forme piu severe). In questo articolo si parlera delle terapie utilizzate nelle forme lievi di DA, dei metodi di detersione e degli ultimi tessuti sviluppati allo scopo di ridurre gli stimoli irritanti e migliorare il decorso clinico della patologia.

Pityriasis lichenoides in età pediatrica: quale terapia? Eccellente risposta alla fototerapia: studio di un caso

La Pityriasis Lichenoides (PL) e una patologia cutanea rara, acquisita, ad eziologia sconosciuta, caratterizzata da un ampio spettro di manifestazioni cliniche che vanno dall’insorgenza acuta di lesioni papulari che evolvono in pseudovescicole con necrosi centrale (Pityriasis Lichenoides et Varioliformis Acuta, PLEVA) alla presenza di piccole papule brunastre desquamanti (Pityriasis Lichenoides Cronica, PLC) includendo forme con caratteristi-che intermedie. In eta pediatrica risultano piu frequenti le forme generalizzate e caratterizzate da frequenti reci-dive. La fototerapia con UVB a banda stretta (311nm) (UVB-nb) rappresenta una modalita terapeutica con un noto profilo di efficacia e sicurezza, che la rende particolarmente indicata nel trattamento di alcune patologie dermatologiche dell’eta pediatrica, tra cui psoriasi, dermatite atopica, vitiligine, sclerodermia. Le conoscenze attuali riguardo il suo impiego nella terapia della Pityiriasis Lichenoides, soprattutto nei pazienti pediatrici, sono limitate ma incoraggianti. Con questo lavoro presentiamo un caso di un paziente pediatrico affetto da PLEVA resistente alle comuni terapie trattato con fototerapia con UVB-nb con remissione clinica completa della sinto-matologia.