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BACKGROUND: General skin care recommendations such as the use of moisturisers and products with adequate photoprotection are important components of management for acne patients to complement the medical regimen. This study aimed to evaluate the real-life clinical effects of a novel UV-selective face cream (Acne RA-1,2) on acne, epidermal barrier function, sebum production, adherence and tolerability when used together with pharmacological acne treatment. METHODS: 40 patients receiving pharmacological acne treatment applied Acne RA- 1,2 once-daily for 3 months. Investigator's Global Assessment of acne, trans- epidermal water loss, sebum production and tolerability were assessed after 1 and 3 months. RESULTS: After 3 months, there was a 38% significant clinical improvement in mean Investigator Global Assessment score (3.4 to 2.1), a 29% significant reduction in trans-epidermal water loss (13.2 to 9.4 g/h/m2), and a 17% significant decrease in sebum production vs baseline (234.6 to 195.6 μg/cm2; all p

Distal to tyrosinase activity, eumelanin synthesis is governed by the rearrangement of dopachrome which may proceed with or without decarboxylation giving 5,6-dihydroxyindole (DHI) and/or 5,6-dihydroxyindole-2-carboxylic acid (DHICA), that exhibit different chemical and biological properties. In melanocytes the reaction occurs under strict enzymatic assistance directing the course of melanogenesis toward DHICA and preventing the spontaneous decarboxylation to DHI. To inquire into the role of the carboxyl group as determinant of the chemical properties of the 5,6-dihydroxyindole system, DHICA and its methyl ester were compared for both their antioxidant activity and their conversion to eumelanin chromophores via oxidative polymerization. The results revealed that DHICA ester oligomers are stronger antioxidant compared to the free acid ones. Moreover, whereas the spectrophotometric course of DHICA oxidation proceeds via a purple chromophoric phase followed by band broadening to give a dark brown eumelanin, DHICA ester is converted to light brown species devoid of the typical euemelanin properties. These results suggest that the carboxyl group plays a more complex role than simply blocking the 2-position of the indole ring. The actual biological significance of these observations and their possible exploitation for dermocosmetic applications are currently under assessment in our laboratory.

Recent advances in the chemistry of melanins have disclosed the importance of the monomer composition in determining significant differences in the pigment properties of crucial relevance to their biological role, including skin (photo)protection and UV-susceptibility. In this scenario, a key role seems to be played by carboxyl groups. Depending on the extent of decarboxylation during the rearrangement of dopachrome different contents of 5,6-dihydroxyindole (DHI) and 5,6-dihydroxyindole-2-carboxylic acid (DHICA) can be incorporated into eumelanin pigments. Rearrangement of 5-S-cysteinyldopa o-quinoneimine likewise can lead to 1,4-benzothiazine (BTZ)or its 3-carboxylic acid (BTZCA) derivative. The DHICA-to-DHI ratio markedly affects the antioxidant and chromophoric properties of eumelanins: in particular, high amounts of DHICA-derived units decrease visible light absorption relative to DHI-based melanins, but markedly enhance antioxidant properties. In the same way carboxylated benzothiazines can confer pronounced visible and UVA absorption features to pheomelanins, accounting for light-dependent reactive oxygen species (ROS) production, whereas BTZ-related units seem to be more effective in inducing ROS production by redox cycling mechanisms in the dark. The possible biological consequences of carboxyl group retention in the eumelanin and pheomelanin pathways will be discussed.

Maddalena Napolitano,1 Matteo Megna,2 Elena A Timoshchuk,3 Cataldo Patruno,2 Nicola Balato,2 Gabriella Fabbrocini,2 Giuseppe Monfrecola2 1Dipartimento di Medicina e Scienze della Salute “Vincenzo Tiberio”, Università degli Studi del Molise, Campobasso, 2Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy; 3Department of Dermatovenereology, Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia Abstract: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease primarily affecting apocrine gland-rich areas of the body and presenting with painful nodules, abscesses, sinus tracts, and scarring. HS is a multifactorial disease in which genetic and environmental factors play a key role. The primary defect in HS pathophysiology involves follicular occlusion of the folliculopilosebaceous unit, followed by follicular rupture, and immune responses (perifollicular lympho-histiocytic inflammation), finally leading to the development of clinical HS lesions. HS has a destructive impact on the patient’s quality of life, being a very challenging disease. Available treatments are limited, mostly off-label and with high variability in the reported efficacy. Fortunately, a monoclonal antibody against tumor necrosis factor alpha has been recently approved for treatment of moderate to severe HS, offering patients a promising new option. This review focuses on the main features of HS, including epidemiology, clinical aspects, pathogenesis, severity classifications, comorbidities, and currently available treatments. Keywords: hidradenitis suppurativa, pathogenesis, diagnosis, treatment

Hyaluronic acid (HA) is a linear non-sulphated glycosaminoglycan, used in dermatology as a biomaterial for bioengineering purposes, temporary dermal filler, stimulation of wound healing as well as drug vehicle in topical formulations. In addition to the well-characterized structural properties, extensive research on HA has revealed a range of vastly immunemodulatory effects, dependent on its size. In this in vitro study we investigated the ability of HA-S3, a small fragment HA (MW, molecular weight: 68 kDa) with degree of sulphatation of 3 and of HA fraction (MW:210 kDa) to reduce the bacterial induced inflammatory response in spontaneous immortalized keratinocytes. To this purpose, HaCaT cells were treated for 24 hours with 25 µg/ml of E. Coli derived bacterial lipopolysaccharide (LPS) in absence or presence of small fragment HA-S3 or HA. Cell viability was thereafter assessed using trypan blue stain and interleukin (IL)-8, IL-1β and tumor necrosis factor alpha (TNF-α) concentrations were determined in cell supernatants by single enzyme-linked immunoadsorbent assay (ELISA). Our results showed that cell viability was not affected either by HA-S3 or HA which in turn were able to reduce LPS-induced mortality. HA and especially HA-S3 were able to significantly reduce LPS-induced pro-inflammatory cytokines. Our observation might suggest new perspectives in the development of HA-S3 containing topical products able to modulate cutaneous inflammatory response.

L'acne è una dermatosi ad eziopatogenesi multifattoriale in cui differenti fattori contribuiscono al mantenimento del processo infiammatorio. Il ruolo del P. acnes è stato, infatti, rideterminato in quanto trigger principale della risposta infiammatoria, essendo in grado di attivare la liberazione di numerose citochine proinfiammatorie. Non a caso, gli antibiotici sono tra i farmaci cardine della terapia topica dell'acne. Il loro uso è, tuttavia, limitato dallo sviluppo di resistenze batteriche. Ne deriva la necessità di utilizzare nuove molecole, le cui proprietà antibatteriche non siano suscettibili di fenomeni di resistenza. Tra le molecole che hanno destato maggiore interesse, per l'azione antibatterica non antibiotico-dipendente, e, pertanto, non soggetta allo sviluppo di resistenze, annoveriamo l'acido laurico, l'argento micronizzato e lo zinco acetato. Il nostro studio si propone di valutare l'efficacia nel trattamenti dell'acne lieve-moderata di una terapia topica a base di argento micronizzato, acido laurico e zinco acetato attraverso una valutazione sia oggettiva, mediante GAGS e Sebutape, che soggettiva, mediante l'ausilio di un nuovo strumento di valutazione della componente psicologica del paziente acneico: l'acne radar.

Traditionally, the positive correlation between red hair and melanoma has been attributed to both the poor antioxidant and photoprotective properties of pheomelanins compared to the dark eumelanins, and the capacity of pheomelanin to act as photosensitizer inducing generation of reactive oxygen species upon UV irradiation. Recently, the occurrence of UV-independent pathways of carcinogenesis was provided by a series of experiments showing that induction of an activating mutation of BRAF into red mice resulted in a high incidence of invasive melanomas in the absence of UV stimulation . In addition, the skin of pheomelanic mice contained higher levels of oxidative DNA and lipid damage with respect to albino-Mc1re/e mice (Mitra et al, Nature 2012, 491, 449-453). These data clearly showed that the pheomelanin pathway could mediate oxidative stress and melanomagenesis ; however the mechanisms have not so far addressed at molecular level. To get an insight into these processes we investigated the reactivity of pheomelanin pigment isolated from human red hair (RHP) and synthetic pheomelanins from cysteinyldopa (CD-Mel) toward critical cellular antioxidants such as GSH and NAD(P)H. Data showed a remarkable increase in the oxidation rate of both compounds in the presence of RHP compared to the controls, whereas much less marked variations were noticed with the eumelanin extracted from black hair. HPLC analysis consistently indicated a rise in GSH disulfide (GSSG) levels with GSH decrease, confirming a redox reaction. Formation of NAD(P)+ was similarly observed in the reaction mixture of NAD(P)H with RHP. In the absence of oxygen, GSH and NAD(P)H depletion was not observed while the presence of enzymes as superoxide dismutase and catalase did not modify the effect of pheomelanin suggesting a ROS independent mechanism. Similar effects were obtained with CD-Mel confirming that the prooxidant effects are due to the pheomelanic component. The mechanism of GSH oxidation by RHP was investigated by EPR spectroscopy. Besides inducing antioxidant depletion, RHP, like CD-Mel, proved also capable of promoting autoxidation of melanin precursors, such as 5-S-cysteinyldopa and dopa, under conditions of exclusion of light, with formation of pheomelanin or eumelanin pigments. This effect is oxidative in character, since it depends on oxygen, revealing a remarkable prooxidant capacity of the pigment serving as a biocatalyst for non-enzymatic melanogenesis (Panzella et al., PCMR 2014, 27,244-252; Greco et al. Chem Comm. 2011,47, 10308-10310). A mechanism has been proposed to account for the observed effects in which GSH, NAD(P)H and melanin precursors oxidation is not mediated by ROS, but is the result of a direct redox exchange with oxidized 1,4-benzothiazine units within the pigment scaffold. Re-oxidation of reduced pheomelanin by oxygen with formation of ROS would ensure shuttling of pheomelanin between the redox states. Overall these results provide a unifying chemical framework into which to explain the mechanisms underlying the relationships between pheomelanin pigmentation and oxidative stress.

Acne vulgaris is a common skin condition with substantial cutaneousand psychologic disease burden. The emotional impact of acne iscomparable to that experienced by patients with systemic diseases, likediabetes and epilepsy. Its pathophysiology is multifactorial and complex,including obstruction of the pilosebaceous unit due to increased sebumproduction, abnormal keratinization, proliferation of Propionibacteriumacnes and inflammation. Topical agents (retinoids, benzoyl peroxide andantibacterials) are the most commonly used therapy for acne. Newertopical therapy, such as photodynamic therapy, has been proposed toincrease efficacy when conventional therapies are ineffective. Thischapter discusses the etiology, pathogenesis, treatment and psychologicalimpact of acne vulgaris. © 2013 by Nova Science Publishers, Inc. All rights reserved.

Introduction. Skin is a specific target of new cancer treatments. Xerosis, inflammatory lesions and radiodermatitis are the most frequent skin reactions. They can decries patients quality of life and compromise their compliance with cancer therapy. Vitamin E is well known for its antioxidant action, for which it is used in many inflammatory skin diseases. We evaluated the efficacy and safety of a medical device based on vitamin E for the treatment of xerosis, of consequent effects of papulopustular rash and radiodermatitis. Materials and methods. We selected 20 women between 35 and 60 years old, affected by xerosis, consequent effects of papulopustular rash and radiodermatitis. The protocol provided for the application of a topical product consisting of tocopherols and tocotrienols to 10% to 0.3% twice a day to the affected area. The evaluations were carried out at the beginning of treatment (TO) and after 40 days (T1) using the following methods: photographic assessment; clinical evaluation; evaluation of Trans Epidermal Water Loss (TEWL) by corneometry; assessment of skin color by colorimeter X-Rite̊ 968 according to the L *a*b* system; assessment of tolerability with a questionnaire; assessment of adherence to therapy through the completion of a daily diary. Statistical analysis was performed by calculating the Student's t. Results. Photographic evaluation shows a significant improvement of the lesions from T0 to T1. Clinical evaluation showed a good improvement in 13 of 20 patients (65 %). TEWL in patients with xerosis went from a value of 19.86 at T0 to a mean value of 14.62 at T1. The outcomes of patients with papulopustular rash presented at T0 an average value of TEWL of 17,25, which was of 15.42 at T1. In patients with previous radiodermatitis, the TEWL was 35.06 at T0 and it was 13.23 at T1. Colorimetry showed a L * value of 58,06 at T0 while it was 60.04 at T1 in patients with xerosis. Patients with previous papulopustular rash presented at T0 an average value of L* of 57.33 while at T1 this was 59.67. In patients with an outcome of radiodermatitis the average went from 53.12 at T0 to 59.91 T1. Tolerability was excellent in 100% of cases. The analysis of the diaries of application of the product showed a good adherence to therapy. Discussion and conclusions. The preparation based on tocopherol and tocotrienols showed an excellent efficacy profile, a good tolerability and safety profile and an excellent profile of adherence.

In medical practice, almost every clinician may encounter patients with skin disease. However, it is not always easy for physicians of all specialties to face the daily task of determining the nature and clinical implication of dermatologic manifestations. Are they confined to the skin, representing a pure dermatologic event? Or are they also markers of internal conditions relating to the patient’s overall health? In this review, we will discuss the principal cutaneous conditions which have been linked to metabolic alterations. Particularly, since insulin has an important role in homeostasis and physiology of the skin, we will focus on the relationships between insulin resistance (IR) and skin diseases, analyzing strongly IR-associated conditions such as acanthosis nigricans, acne, and psoriasis, without neglecting emerging and potential scenarios as the ones represented by hidradenitis suppurativa, androgenetic alopecia, and hirsutism.

Le fotodermatosi in età pediatrica rappresentano un vasto capitolo delle patologie dermatologiche. La luce solare è in grado di produrre malattie caratterizzate da fotosensibilità. Il capitolo focalizza l'attezione soprattutto su le fotodermatosi autoimmuni e quelle mediate da sostanze chimiche. Fra le prime vengono trattate soprattutto la dermatite polimorfa solare, l'orticaria solare e l'hydroa vacciniforme; fra le seconde la gran parte è rappresentata da reazioni fototossiche a vegetali (fitofotodermatiti, cosmetici e farmaci sia topici che sistemici.

Hidradenitis suppurativa (HS) is a chronic, recurrent, debilitating inflammatory disease of unknown aetiology. The current standard of care for HS includes antibiotics (oral/topical), retinoids (oral/topical) and intralesional steroids. However, the recurrence of the disease and the unsuccessful of the different treatments stimulate findings of different therapy using light source. Among these, photodynamic therapy with 5-aminolevulinic acid (ALA-PDT) has been proposed by some authors recently. In this study we report the experience of 15 patients affected by HS resistant to conventional therapy treated with ALA-PDT.

BACKGROUND/PURPOSE: Polymorphous light eruption (PLE) heterogeneity has been postulated, but the existence of benign summer light eruption (BSLE) is controversial. We studied the prevalence of the clinical patterns, criteria distinguishing BSLE from PLE, and diagnostic usefulness of phototest. METHODS: Five Italian Photodermatology Centres recruited retrospectively 346 patients with typical clinical history and/or presentation of PLE. Age, gender, skin type, family history and presence of atopy were considered. UVA and UVB MEDs and provocative phototests with UVA and UVB were obtained with a standardized procedure. Photopatch tests were applied according to the IRCDG rules. ANA were assessed by indirect immunofluorescence. RESULTS: Four criteria (predominance of women, shorter latency, uninvolvement of the face and absence of relapse during summer) identified BSLE in only 6.1% of cases. All had positive phototests, mostly with UVA. Uninvolvement of face, short latency and no seasonal relapses identified 11.7% patients, mostly with positive phototests to UVA. Short latency and no seasonal relapses in women identified 11.2% patients. Uninvolvement of face and no seasonal relapses in women identified 8.1% of patients. Uninvolvement of face and short latency in women identified 17.6% of patients. CONCLUSION: Criteria diagnosed BSLE in only a minority of patients, who were positive at phototesting, mostly with UVA.

The possible role of melanocyte as a modulator of the inflammation and keratinocyte hyperproliferation in psoriasis has been hypothesised but never demonstrated on experimental basis. Aim of the present study was to assess whether plasma levels of 5-S-cysteinyldopa (CD), a metabolite reflecting melanocyte activity, undergo changes in association with psoriasis together with those of typical lipid peroxidation markers thiobarbituric acid reactive substances (TBARS). A group of 16 patients with psoriasis at different stage as indicated by the psoriasis area and severity index (PASI) were enrolled against an age and sex matched control group. Both TBARS (P

Objective: The aim of this work was to assess whether psoriasis is associated with phenotype prevalence and altered activity of Haptoglobin (Hpt). Background: Hpt is a plasma acute phase glycoprotein, displaying in humans three phenotypes. Phenotype prevalence or structure modification of Hpt was associated with several diseases. The Hpt main function is to bind and carry to the liver free hemoglobin for degradation and iron recycling. Hpt was recently found able to bind the apolipoprotein A-I (ApoA-I), thus impairing its stimulation on the activity of the enzyme lecithin-cholesterol acyl-transferase (LCAT) Study design: Hpt was isolated from patients with psoriasis vulgaris, and its activity in hemoglobin or ApoA-I binding and LCAT inhibition was compared with that of normal protein. Methods: Two affinity chromatography steps, the first using resin-coupled hemoglobin and the second anti-Hpt antibodies, were used to purify Hpt. The protein phenotype was assessed by electrophoresis. Binding experiments were performed by ELISA with stationary hemoglobin or ApoA-I, Hpt in solution, and anti-Hpt antibodies for detection of bound Hpt. Standard LCAT assays were carried out in the presence of Hpt purified from patients or healthy subjects. Results: Phenotype prevalence of Hpt in psoriasis was not found. After affinity chromatography by hemoglobin, albumin and ApoA-I were routinely found heavily contaminating only Hpt from normal subjects. Isolated Hpt from patients had lower activity than normal protein in both hemoglobin binding and LCAT inhibition. Conclusions: In psoriasis, Hpt displays some structure modification(s) which might be associated with the protein function in the disease.

Pheomelanin photochemistry is commonly regarded as reflecting the behavior of benzothiazine chromophores biogenetically derived from 5-S-cysteinyldopa. This notion, which has become a central dogma in pheomelanin research despite the lack of direct experimental support, has in part been built upon the identification many decades ago in pheomelanin-containing tissues of a peculiar group of low molecular weight compounds, the trichochromes, featuring the peculiar 2,2'-bi(2H-1,4-benzothiazine) skeleton. Although commonly regarded as primary determinants of the red colorations of hair in the true “pheomelanic” phenotype, the origin and even the actual occurrence of trichochromes in human hair and tissues has remained a subject of debate. In an attempt to address this issue, the chromophoric features of synthetic pheomelanins and of a red hair pheomelanin were compared with those of some putative precursors prepared under biomimetic conditions. All pheomelanin samples in phosphate buffer at pH 7.4 showed a definite maximum around 300 nm and a monotonic decrease of the absorption in the range 350-500 nm with inflections around 259 and 360 nm. The chromophoric changes accompanying the formation of synthetic pheomelanin by peroxidase/H2O2 oxidation of CD in the presence or in absence of zinc ions were monitored with concomitant LC/UV and MS analysis. At the monomer stage benzothiazine intermediates were detected which changed into benzothiazine dimers and trichochrome related species. At the later stage, in the absence of chromatographically defined species, the oxidation mixture showed a distinct absorption around 300 nm which was evidently due to chromophoric units belonging to the pigment backbone. These features were superimposable to those of 3-oxo-3,4-dihydro-1,4-benzothiazine and benzothiazole species suggesting that these previously overlooked structural units play in fact a role in determining the pigment chromophore. The possible impact of these observations on the phtotochemical and photoprotective properties of pheomelanins will be discussed.

Pigmentary traits such as red hair, fair skin and the lack of tanning ability are generally recognized as risk factors for melanoma and other skin cancers. However different response to UV exposure are observed among individuals exhibiting similar phenotypes. The association between loss of function mutations at the melanocortin-1 receptor (MC1R) and high risk red hair phenotypes has now been firmly established but a relationship of such genetic traits and the type and amounts of cutaneous melanins have not so far been extensively investigated, nor has the presence of pheomelanins, the typical red hair pigments, been assessed in those individuals bearing the most common allelic variants. We reported that pheomelanic tissues may be identified by analysis of two highly specific structural markers namely 1,3-thiazole-2,4,5-tricarboxylic acid (TTCA) and 6-(2-amino-2-carboxyethyl)-2-carboxy-4-hydroxybenzothiazole (BTCA) obtained by chemical degradation of the pigments. Most of the red human hair examined showing different hues afforded TTCA in variable yields, while BTCA was obtained only from a restricted number of samples. BTCA positive individuals exhibited a marked tendency to actinic damage as evidenced by MED values. These preliminary data have now been confirmed on a larger group of red haired individuals. The lowest MED values and 5-days delayed pigmentation was associated with BTCA-positive subjects while TTCA-positive subjects gave statistically significant higher MED values. The potential of pheomelanin markers associated with determination of photobiogical parameters and quantitation of other metabolic markers for predicting individuals at high risk for skin cancer and melanoma in large populations will be discussed.

Aim. treatment currently employed for plantar warts (PW) are often painfl and poorly effective. This study evaluates the effect of photodynamic therapy (PDT) with δ-aminolevulinic acid (ALA) on PW. Methods. Before treatment, the superficial hyperkeratotic layer of warts was removed by the application, for 7 days, of an ointment containing 10% urea and 10% salicylic acid. Then, after gentle curettage, a cream containing 20% ALA was applied under occlusive dressing for 3h on 3 patients with 84 warts, while 30 patients with 62 warts (controls) receveid only base cream. Both groups were irradiated using a visible light lamp (range 400-700 n, peaking at 630 nm). The light dose was 50 J/cm2 each session. Patients were followed-up for 12 months. During the treatemtn some patients referred mild burning sensation or slight pain. The absorption of ALA in warts was investigated and demonstrated by in vivo fluorescence spectroscopy. Results. Two months after the last irradiative session, 84.5% of the ALA-PDT treated lesions and 22.5% of controls had resolved. Conclusions. The results of this study suggest that topical ALA-PDT can be considered as alternative treatment for PW.

Objective: aim of this study was been the evaluation of daily and monthly changes of solar short ultraviolet (UVB) in a central Mediterranean area (Naples city, 40.8623° North) from 1997-2002. Materials and methods: the measurements were automatically collected and expressed as physical units (mW/cm2) and biological units (Minimal Erythema Dose/hour = MED/h). The irradiance unit may be converted to UV Index (UVI) by the conversion factor: 1 [MED/h] = 2.3 on the UVI scale. These values were processed with a special software Solar Light IDL 2.0 that informed us about current UV dose, accumulated UV dose, current UVI, peak UVI, time until sunburn, expressed in mins. Moreover, we studied these parameters by means of a statistical data analyser software called SPSS version 10.0 to obtain information about average values in this last 5 years of measurements. Results: the results showed that: i) the sporadic peaks of maximal irradiance occurred in June; ii) the maximal value of the monthly mean irradiance was observed in June-July, while the minimal one in December-January; iii) the amount of solar UVB that reaches the ground and therefore our skin during the last 5 years has been unmodified. About 20-30% of total daily UVB is received during the interval of time between 60 mins both before and after midday in summer, with 75% between 9 am and 3 pm. In Naples during the summer, half an hour of midday sun exposure of the unacclimatized skin of Caucasian subjects is normally sufficient to result in a subsequent mild reddening of the skin. We report that in Naples the average UVB sunstroke is equal to about 2 MED/day in winter; 7 MED/day in spring and in autumn, 12 MED/day in summer. According to Diffey algorithm, in Naples annual MED is about 2600, and these values have been confirmed by our measurements (2676 MED in 1998, 2591 MED in 1999, 2724 MED in 2000, 2612 in 2001, 2634 in 2002). Conclusions: our results show that erythema could appear, in individuals with skin type II and III living in a touristic Mediterranean area, after about 30 mins of sun exposure, in July; ii) the sunburn risk is higher just during the months when skin hasn't yet acquired the natural defences to respond to the peak of UVB. This monitoring may give more information about photodermatitis, photocarcinogenesis and some preventative devices of exposure to sun rays.

Atopic dermatitis (AD) is a complex and heterogeneous disease whose incidence and prevalence is increasing in developed countries. The management of patients with AD is sometimes difficult, and includes proper recognition of trigger factors. Here guidelines for the diagnosis and therapy of AD that are based on scientific evidence are proposed. The development of guidelines should avoid useless intervention, reduce disease costs and promote a better patient care.

A 66 year-old man with neurofibromas occurring only on the left upperarm is described. This rare form of neurofibromatosis (of which only 15 cases have been reported) can be ascribed to type V according to Riccardi's classification, improved by Roth.