Stefania Barruscotti

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

Bleomycin-induced flagellate dermatitis: Report of four paediatric cases

References 1 Holzberg M, Baran R. The nail in dermatologic disease. In Baran R, de Berker DAR, Holzberg M, Thomas L, eds. Diseases of the Nails and Their Management. Wiley-Blackwell, Oxford, 2012: 257. 2 De Jong EM, Seegers BA, Gulinck MK et al. Psoriasis of the nail, associated with disability in a large number of patients: results of a recent interview with 1728 patients. Dermatology 1996; 193: 300–303. 3 Baran R. The burden of nail psoriasis: an introduction. Dermatology 2010; 221(suppl 1): 1–5. 4 Reich A, Szepietowski JC. Health related quality of life in patients with nail disorder. Am J Clin Dermatol 2011; 12: 313–320. 5 Ortonne JP, Baran R, Corvest M et al. Development and validation of nail psoriasis quality of life scale (NPQ10). J Eur Acad Dermatol Venereol 2010; 24: 22–27. 6 McGonagle D. Enthesitis: an autoinflammatory lesion linking nail and joint involvement in psoriatic disease. J Eur Acad Dermatol Venereol 2009; 23 (S1): 9–13. 7 Aydin SZ, Castillo-Gallego C, Ash ZR et al. Ultrasonographic assessment of nail in psoriatic disease shows a link between onychopathy and distal interphalangeal joint extensor tendon enthesopathy. Dermatology 2012; 225: 231–235. 8 Wortsman X, Jemec GBE. Ultrasound imaging of nails. Dermatol Clin 2006; 24: 323–328. 9 Soscia E, Sirignano C, Catalano O et al. New developments in magnetic resonance imaging of the nail unit. J Rheumatol Suppl 2012; 89: 49–53. 10 Namey TC, Rosenthall L. Periarticular uptake of 99mtechnetium diphosphonate in psoriatics: correlation with cutaneous activity. Arthritis Rheum 1976; 19: 607–612.

Ponatinib-induced widespread ichthyosiform eruption

Ponatinib is a new potent third-generation tyrosine kinase inhibitor (TKI), developed for the treatment of chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphocytic leukaemia (ALL) resistant to first (imatinib) and second generation (dasatinib and nilotinib) TK-inhibitors. Aimed at wild type and mutant BCR-ABL, ponatinib demonstrates significant anti-leukemic activity and holds much promise in treating other malignancies, including gastrointestinal stromal tumors (GIST)(1;2). This article is protected by copyright. All rights reserved.

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.

Pediatric Old World cutaneous leishmaniasis treated with oral fluconazole: A case series

Cutaneous leishmaniasis has an incidence of 0.7–1.2 million cases per year and represents a growing concern in the outpatient dermatologic practice in Europe because of imported cases due to increased travel to risk areas and to immigration phenomena. When dealing with children, the treatment can be challenging because of side effects and pain of classic antimonial therapy leading to poor rates of course completion and requirement of sedation for several children.

Zoon's plasma cell balanitis: clinical and dermoscopic features in pediatric patients

test turned out to be negative at 3 months after the treatment. Syphilis is a prevalent sexually transmitted disease caused by TP. Secondary syphilis is a notorious “Great Mimicker”, because its clinical features have a very extensive diversity. Physicians are increasingly challenged by unusual presentations from secondary syphilis. The most common cutaneous presentation for secondary syphilis is a generalized, non-pruritic, papulosquamous eruption varying from pink to violaceous or brown, with mucosal involvement. The presentation of palmoplantar lesions is a helpful diagnostic clue for secondary syphilis. Pustular lesions or impetigo-like lesions in secondary syphilis are less common. Immunocompromised patients infected by syphilis could have pustular lesions more commonly than immunocompetent individuals. Although our present patient had a negative result of HIV assay, his low Body Mass Index (16.46) suggested he was malnourished. Thus, the poor nutrition status could be another risk for syphilis patients to develop impetigo-like lesions. In summary, we reported a case with secondary syphilis in a malnourished patient with unusual impetigo-like lesions. It is worth testing for syphilis in patients with perplexing rashes, especially those that failed the standard therapy.

Transient symptomatic zinc deficiency in a breast-fed African infant: case report and literature review

A three-month-old African girl was born prematurely at 32 weeks’ gestation. She presented a standard height-weight growth and was exclusively breastfed since birth. At 3 months of age, she developed a periorificial dermatitis characterized by epidermal disepithelialization with erosions located on the face (Fig. 1), on the neck, and on the diaper area (Fig. 2). A week after the onset of symptoms, the eruption spread to the scalp and the perianal region, accompanied by diarrhea, and the patient was therefore hospitalized. The routine laboratory investigations gave negative or normal results, except for a reduction of the alkaline phosphatase (88 mU/ml, normal range 108– 345 mU/ml). Culture swabs from the diaper area grew a Pseudomonas Aeruginosa and Candida Parapsilosis, therefore systemic therapy with vancomycin 240 mg daily and fluconazole 4 ml daily was started. Considering the clinical features and the history of the patient, a zinc deficiency was considered, and serum zinc concentration revealed low concentration (20 mcg/ dl; normal range 50–120 mcg/dl). The maternal serum zinc level resulted within the normal range, but maternal breast milk zinc level was low (21 mcg/dl; normal 50-120 mcg/dl). A diagnosis of zinc deficiency was made. An oral zinc supplementation was started (zinc sulfate 4 mg/kg/daily). A rapid improvement of skin lesion was obtained, and a complete resolution was observed, in 12 weeks (Fig. 3). Zinc supplementation was discontinued, and relapse was not observed at weaning. At the age of 1 year, the patient is in complete clinical remission. Based on clinical features, laboratory findings, low zinc concentration in mother’s milk, and clinical remission at weaning, a diagnosis of transient symptomatic zinc deficiency (TSZD) was made, and genetic investigations have not been carried out.

Human Herpesvirus-7 Papular Rash in a Healthy Adult Patient

A 50-year-old man was referred to the department of dermatology with a diffuse erythemato-papular rash, spreading from the trunk to the limbs and neck, accompanied by intense pruritus (Fig. 1). The patient’s medical history was unremarkable, no prodromal symptom was present, and he denied drug use, both routinely and in the days before the eruption. Laboratory investigations were negative or within normal ranges, except for mild lymphocytosis (3.9 × 103/mm3; normal range 1.5–3 × 103/mm3). A diagnosis of urticaria papulosa/insect bites was suspected. The patient returned 3 days later due to persistent lesions. Skin biopsies were performed and the histological findings showed an interface dermatitis, with rare lymphocytes along the dermo-epidermal junction, and a scarce, superficial perivascular lymphocytic infiltrate. The epidermis showed a mild spongiosis, lymphocytes exocytosis and rare single necrotic keratinocytes. No eosinophils were evident. An orthokeratotic stratum corneum with a preserved basket-weave aspect was seen, demonstrating the acute nature of the process (Fig. 2). The most common infectious agents associated with cutaneous rashes (secondary syphilis, HSV 1–2, VZV, CMV, EBV, HHV-6, HCV, HBV, HIV, parvovirus B19, Coxsackie and echovirus) were excluded by serological testing or count of DNA copies in blood samples. The only notable finding was the detection of circulating HHV-7 DNA (800 copies/ml) in the blood, which is considered significant for active infection. A diagnosis of HHV-7 related papular rash was suggested. Four weeks after the beginning of the eruption, the rash cleared completely without scaling and HHV-7 DNA was undetectable in blood samples.

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.