Nicolò Rivetti

Hair depigmentation and vitiligo-like lesions in a leukaemic paediatric patient during chemotherapy with dasatinib.

© 2012 The Authors. doi: 10.2340/00015555-1289 Journal Compilation

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.

Effectiveness of photodynamic therapy in refractory plaque‐stage mycosis fungoides associated with Bowen's disease

Dear Editor, Bowen’s disease (BD) is a form of squamous cell carcinoma in situ that can develop into invasive carcinoma if not treated, clinically characterized by an erythematous and scaly plaque that can occur both on sunexposed and non-exposed areas of the skin (Idriss, Misri, & B€ oer-Auer, 2016). Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), and it is characterized by erythematous patches distributed in not-sun exposed areas, which can evolve to

Pseudoepitheliomatous changes in a case of vegetating Darier–White disease: a unique histopathological finding.

Abstract:Darier–White disease (DWD) is a rare autosomal dominant genodermatosis, characterized by constant and typical histopathological findings, such as hyperkeratosis, dyskeratosis with corps ronds and grains and papillary microvilli formation with suprabasal clefting. Despite its nearly constant histopathological presentation, unusual clinical variants are reported, such as the vegetating and cornifying ones. These variants share the same histopathological features of the classic type, except for the striking hyperkeratosis and acanthosis. Here, unreported pseudoepitheliomatous features are described in an elderly male patient with a long history of vegetating and verrucous papules and nodules of DWD, associated with typical nail involvement. These unique histolopathological changes were closely in conjunction with the characteristic microscopic features of DWD. Differential diagnosis with other pseudoepitheliomatous and acantholytic conditions such as reticulated seborrheic keratosis, inverted follicular keratosis, and acantholytic squamous cell carcinoma is also considered. Pseudoepitheliomatous features, in this case of vegetating DWD, could be regarded as a reactive epidermal phenomenon because of different stimuli, i.e. maceration, bacterial superinfection, and chronic scratching.

Long-term remission of erythrodermic mycosis fungoides after persistent control of hepatitis B infection

Editor Mycosis fungoides (MF) is the most frequent form of primary cutaneous T-cell lymphomas. Diffuse erythroderma can accompany each stage of MF, but it can also represent a peculiar clinical variant of the disease that arises de novo as ‘erythrodermic MF’. We report the case of a 54-year-old man who presented at our Department with diffuse erythroderma, hyperkeratosis of palms and soles and onychodystrophy with onychogryphosis (Fig. 1a, b), from which he had been affected since the previous 4 months. Furthermore, he complained of diffuse pruritus, fever, chills and fatigue. His family physician had prescribed 50 mg of oral prednisone daily for about 4 weeks, before our observation, without any improvement. We performed a skin biopsy from the back of the patient. Histological examination showed a dermal patchy and band-like cellular infiltrate of smallto medium-sized lymphocytes, mostly showing hyperchromatic and irregularly contoured nuclei. Focal epidermotropism, mostly of single lymphocytes, was noticed (Fig. 2a, b). The epidermis showed acanthosis, mild spongiosis and some scattered CD1a+ Langerhans cells. The lymphoid infiltrate positively stained for CD2, CD3 and CD5 antigens, with partial loss of antigen CD7 (Fig. 2c, d, e), and displayed a CD4+/CD8phenotype. CD30+ cells were rare, as well as only scattered residual CD20+ B cells were observed. On such basis, a diagnosis of MF was made and the patient was staged according to the ISCL/EORTC staging system, resulting in stage IIIA. A blood sample showed alterations of the liver function, and the patient was screened for HBV and HCV infections. High levels of HBV-DNA (90.067.500 IU/mL) were detected, suggesting the diagnosis of severe active hepatitis B infection. The presence of active hepatitis strongly influenced the treatment choice. Prednisone was gradually reduced and, according to the infectivologists, the patient started a combined treatment of subcutaneous natural interferon-alpha (IFN-a), (starting dose of 3.000.000 UI 3 times/week) and oral entecavir 0.5 mg twice/ daily, associated with NB-UVB phototherapy. The patient received NB-UVB phototherapy (311 nm) because this therapy does not require the assumption of a systemic photosensitizer (psoralen), a drug with hepatic metabolism. Moreover, we decided to use IFN-a because it is an appropriate drug option for both MF and active HBV infection. The therapy with natural IFN-a was continued for 12 months (from 3.000.000 UI 3 times/week to 6.000.000 UI every 2 days). The patient experienced a progressive improvement of MF and, after 7 months of combined therapy, he obtained a complete MF clinical remission (Fig. 1c, d). Moreover, we observed a progressive normalization of liver enzymes and, after 12 months of therapy, the HBV-DNA viral load was undetectable. After 6 years of follow-up, the patient is still maintaining a complete clinical and serological remission of both MF and HBV infection. In order to keep the HBV viral load undetectable, the patient is still receiving therapy with entecavir. The aetiology of MF remains to be clarified. In literature, infectious agents have been suspected as potential trigger agents. In particular, it has been proposed that MF may result from chronic antigenic stimulation in genetically susceptible individuals and that Human Leukocyte Antigen (HLA) system can influence the susceptibility and prognosis of the disease. In conclusion, at least in our patient, the progressive clinical remission of MF and the serological remission of HBV viral load suggests the possible role of HBV as a lymphoma trigger, according to Dulmage et al. and Mirvish et al. This observation suggests the need to investigate the possible role of HBV in the aetiology of MF and stresses the potential effectiveness of combined therapeutic modalities. However, further studies on larger series of HBV-infected MF patients are needed to elucidate the exact role of HBV in the pathogenesis of MF. (a) (c)

Effectiveness of surgical treatment of severe macrocheilia in a patient with orofacial granulomatosis

Orofacial granulomatosis (OFG) is the term given to a group of diseases characterized by the presence of non‐necrotizing granulomatous inflammation affecting the soft tissues of the orofacial region. Treatment of OFG is often challenging and unsatisfactory. We report on a 32‐year‐old man with a 2‐year history of oedema and swelling of the upper lip without systemic symptoms. The history, clinical features and histopathological findings led to the diagnosis of cheilitis granulomatosa (CG), a disease included in the spectrum of OFG. The patient was treated with oral diaminodiphenyl sulfone (DDS) and clofazimine without success. Oral doxycycline led to a slight improvement of the disease. Because the volume of the upper lip was twice normal size, surgical reduction was performed, followed by administration of oral doxycycline for 3 months. This therapeutic approach led to complete remission, with no recurrence after 3 years.

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.

Recurrence of Mycosis Fungoides on Multiple Melanocytic Nevi: A Case Report and Review of the Literature

Melanocytic nevi represent a widespread cutaneous finding. Nevertheless, the presence of mycosis fungoides and melanocytic nevi in the same location is an extremely rare event. We report the case of a patient affected by mycosis fungoides and treated with PUVA therapy, with complete remission of the disease. Eight years after therapy discontinuation, he presented epidermal scaling and an erythematous perinevic halo on 3 old melanocytic lesions, the clinical aspect of which was highly suggestive for Meyerson nevi. The histological and immunohistochemical examination of an excised melanocytic lesion revealed histological features consistent with the diagnosis of mycosis fungoides superimposed on junctional melanocytic nevi. The finding of patches of mycosis fungoides superimposed on melanocytic nevi is a rare event; the confounding clinical appearance with eczematous changes around a pre-existing nevus may recall the halo dermatitis known as Meyerson phenomenon; this highlights the importance of clinical and histological examination to make the correct diagnosis of dermatological diseases.

La terapia fotodinamica: indicazioni e controindicazioni. Esperienza della Clinica Dermatologica

La terapia fotodinamica (PDT) e una forma di trattamento non chirurgico applicabile a diversi tipi di tumori non cutanei (tumori gastrointestinali, cerebrali, broncopolmonari, endometriali e vescicali) e ad alcuni tumori e lesioni pretumorali della cute. Il principio su cui si basa la PDT e quello di una reazione fotodinamica in grado di distruggere selettivamente le cellule tumorali, ovvero un processo chimico mediato dalla luce (processo foto-chimico) che prevede l’assorbimento della luce da parte di una sostanza fotosensibile e la successiva formazione di specie reattive dell’ossigeno (ROS), in grado di distruggere la cellula nella quale si sono formati. Dalla fine degli anni ’90 la PDT cutanea e divenuta una valida opzione terapeutica per il dermatologo, e dopo il 2000 e entrata a far parte delle linee guida internazionali per il trattamento dei tumori cutanei non melanocitari (NMSC) quali la cheratosi attinica (AK), il carcinoma basocellulare superficiale (BCC), e recentemente anche il morbo di Bowen (BD). La tollerabilita e i vantaggi per il paziente in confronto alle altre terapie ablative chirurgiche e non chirurgiche sono notevoli. La selettivita per le cellule malate con risparmio del tessuto sano permette di avere una guarigione della lesione ulcerativa post-trattamento piu rapida, di mantenere la funzione della cute, e di ottenere un risultato esteticamente molto piu accettabile. Questi vantaggi sono particolarmente evidenti per le lesioni di ampie dimensioni o per lesioni multiple soprattutto al volto. Inoltre e utile per il trattamento di pazienti anziani o in scadenti condizioni generali o in terapia anticoagulante. In questo lavoro, presentiamo l’esperienza della Clinica Dermatologica della Fondazione IRCCS Policlinico San Matteo di Pavia a distanza di circa un anno dall’introduzione della PDT per la terapia dei tumori cutanei non melanocitari: sono stati trattati 29 pazienti e, tenendo conto che alcuni soggetti presentavano piu di una lesione e che in uno stesso paziente potevano essere contemporaneamente presenti lesioni di natura diversa (BD, BCC e/o AK), sono stati nel complesso trattati 4 BD, 21 BCC e 52 AK. L’esperienza della Clinica Dermatologica conferma i dati riportati in letteratura sull’efficacia e i vantaggi di que-sto tipo di terapia: i risultati ottenuti sono piu che soddisfacenti con una remissione clinica delle lesioni trattate maggiore del 90% del totale delle lesioni. Gli eventi collaterali sono stati scarsi e caratterizzati da dolore o bru-ciore localizzato all’area trattata e da aumento della pressione arteriosa durante la procedura. In conclusione, la PDT si e rivelata una terapia efficace, nonche una valida alternativa nel trattamento dei NMSC.