Daniele Torchia

Tumor necrosis factor-α in vitiligo: direct correlation between tissue levels and clinical parameters.

Background: Experimental evidences have shown that tumor necrosis factor (TNF)-α may play a role in the pathogenesis of nonsegmental vitiligo, and successful cases of vitiligo treated with TNF-α inhibitors have been recently reported. Materials and methods: Two cases of refractory generalized vitiligo, which showed high tissue levels of TNF-α, were commenced anti-TNF-α antibody etanercept 50 mg weekly. A retrospective study, considering chart review and immunohistochemical staining for TNF-α, was then carried out on eight additional patients affected by untreated vitiligo. Results: Etanercept achieved improvement of vitiligo in two patients at 6-month follow-up. Five out of eight specimens showed a strong cytoplasmic staining for TNF-α. Considering all 10 cases, patients with a strong TNF-α staining were characterized by a higher vitiligo disease activity score than patients with a weak staining. Discussion: These findings, albeit limited in significance by the low number of cases and the retrospective nature of the study, confirm a probable role of TNF-α in the pathogenesis of vitiligo. The intensity of TNF-α staining in vitiligo lesions may be worth to be further studied as a biomarker for potentially successful anti-TNF-α treatment of nonsegmental vitiligo in cases refractory to conventional treatment.

Papuloerythroderma 2009: Two New Cases and Systematic Review of the Worldwide Literature 25 Years after Its Identification by Ofuji et al.

Background: Even after the description of papuloerythroderma of Ofuji (PEO) in 1984, little is known about this clinical entity. Objective:To report on 2 new cases of PEO and review of the worldwide literature on this topic. Methods: Article citations were searched on several biomedical search engines (PubMed, EMBASE, SCOPUS, Google Scholar). Papers were retrieved either online or in print. Results: A grand total of 170 PEO cases were identified. Most patients were older than 55 years and of Asian or white descent, with an overall male/female ratio of 4.0. Itch and the deck-chair sign were observed in all patients. Peripheral eosinophilia, lymphocytopenia and increased serum IgE were common findings. Histopathology mostly showed aspecific inflammation, while 17 showed histological features of cutaneous T-cell lymphoma (CTCL). Atopy, malignancies, infections and drugs were rarely linked to PEO. Conclusion: PEO represents a rather monomorphous entity both clinically and, with the remarkable exception of CTCL, also histologically. Nonetheless, no causative factor could be identified in the vast majority of cases. An etiological classification and diagnostic criteria are proposed in the attempt to contribute framing this puzzling clinical entity.

Treatment of anogenital condylomata acuminata with topical photodynamic therapy: report of 14 cases and review

Photodynamic therapy (PDT) with 5-aminolevulinic acid (ALA) is an emerging technique for the treatment of genital human papillomavirus (HPV)-induced benign and premalignant lesions. We report herein a case series of anogenital CA successfully treated with ALA-PDT and review the literature available to date on this topic. Thirteen out of 14 cases of anogenital condylomata acuminata were successfully treated with topical PDT. We conclude that PDT can be considered a highly effective and safe treatment option for anogenital condylomata acuminata. Also, considering the available literature, the best results are likely to be achieved with a 16-20% gel formulation of 5-ALA and a red light dose of 100-150J/cm(2).

Eruptive Vellus Hair Cysts A Systematic Review

We report the results of the first systematic review of the worldwide literature on eruptive vellus hair cysts (EVHC). It is likely that EVHC are less rare than it may appear from the scarcity of related publications in the literature. EVHC may be present at birth and may appear at any age, although they show a clear trend towards occurring during the first 3 decades of life. A strong clue to the heavy influence of genes on the occurrence of EVHC is provided by the numerous reports of families in whom two or more members were affected. EVHC lesions present clinically in a rather monomorphous fashion, i.e. round, dome-shaped, skincolored, asymptomatic, soft-tender papules with a smooth surface and grouped or disseminated in a symmetric pattern. EVHC may affect any cutaneous area, even if the upper part of the body and some distribution patterns are particularly frequent and recognizable, i.e. cephalic, upper trunk around the midline, upper limb including axillae, and proximal lower limb. Such a distribution is likely not random and seems to grossly overlap with that of pilosebaceous and apocrine units. Like clinical morphology, the histologic features of EVHC papules are rather monomorphous, indeed, the diagnostic hallmark being the presence of vellus hair shafts within the cystic space. Peculiar subgroups (familial, late-onset, unilesional, and associated with steatocystoma multiplex) are also identified and discussed.In conclusion, EVHC are basically a cosmetic concern to patients but represent a chronic and difficult-totreat condition. On the basis of our review, future studies are warranted, mainly concerning (i) further nosographic framing involving genetic and tissue analysis, (ii) implementation of non-invasive diagnostic procedures, and (iii) therapeutic trials of interventions shown to achieve some effectiveness.

S100-Negative Indeterminate Cell Histiocytosis in an African American Child Responsive to Narrowband Ultraviolet B

Abstract:  Indeterminate cell histiocytosis is a rare cutaneous disease characterized by the presence of dendritic cells that lack Birbeck granules and immunophenotypically shares features of both Langerhans cells and macrophages. We describe a case of a 4‐year‐old African American boy affected by a disseminated, exclusively mucocutaneous form of indeterminate cell histiocytosis. The eruption was successfully treated with narrowband ultraviolet B. The peculiar negativity of the Langerhans cell marker S100 is also discussed.

Segmental Acne versus Mosaic Conditions with Acne Lesions

Acne rarely presents in segmental patterns, which are encountered only in cutaneous mosaicism. We report herein two cases of segmentally arranged acne and systematically review the literature on the topic. Beside already known mosaic conditions which may show primary lesions typical of acne, i.e. nevus comedonicus, Happle-Tinschert syndrome, acne superimposed on epidermal nevi and mosaic Apert syndrome, we introduce the possibility that acne itself may present in a mosaic form. As from the extremely small casuistics retrieved, segmental acne is not present at birth, follows Blaschko lines, is polymorphous in nature and occurs on locations typical of common acne.

Allopurinol-induced palisaded neutrophilic and granulomatous dermatitis

We describe a 64-year-old man with past chronic myeloid leukemia. Palisading neutrophilic granulomatous dermatitis of the hands was diagnosed and related to recent allopurinol intake. Allopurinol is known to rarely cause granulomatous reactions, but this appears to be the first case of palisading neutrophilic granulomatous dermatitis induction. Possible mechanisms include immune complex deposition, an immune response directed against the metabolites of allopurinol, or allopurinol hypersensitivity exclusively localized to the skin.

Photodynamic Therapy for the Treatment of Endoanal Condylomata Acuminata

Lise E. Nigrovic, Richard Malley, Dewesh Agrawal, and Nathan Kuppermann, for the Pediatric Emergency Medicine Collaborative Research Committee of the American Academy of Pediatrics Divisions of Emergency Medicine and Infectious Diseases, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts; Division of Emergency Medicine, Children’s National Medical Center and George Washington University School of Medicine, Washington, DC; and Departments of Emergency Medicine and Pediatrics, University of California, Davis, Medical Center and School of Medicine, Davis

Bilateral Treatment for Alopecia Areata

Abstract:  A 4‐year‐old, otherwise healthy white girl was referred for a 15‐month history of alopecia areata. Anthralin 0.1% cream was prescribed for the left side of the scalp, while corticosteroids for the right side. After 4 months, only the right side of the scalp showed hair regrowth. Half‐side strategy, that is, treating one side and managing the other – divided by the mid sagittal suture – as an internal control for no treatment, placebo or other treatment, has been commonly used in clinical studies for decades. In everyday practice, bilateral treatment is useful to evaluate the responsiveness to two topically delivered interventions and diminishes the time necessary to identify an effective one.

More on Speckled Lentiginous Nevus Syndrome

Speckled lentiginous nevus (SLN) syndrome is a condition characterized by a segmental papular SLN associated with ipsilateral neurological or musculoskeletal disturbances. Framed retrospectively in 2002 by Happle [1] and Vidaurri-de la Cruz [2] on the basis of a handful of cases published for other reasons [3–8] , only further 2 affected individuals have been reported ever since [9, 10] . I wish to propose herein an additional striking case of SLN syndrome, published in the literature 1 30 years ago but remained unrecognized so far. Heine and Poppele [11] described a 14-yearold boy who presented since the age of 2 a checkerboard-arranged papular SLN, mainly involving the upper trunk and right arm, and eventually developed homolateral hyperhidrosis, dysesthesia and musculoskeletal abnormalities ( table 1 ).