Annalucia Virdi

Autoantibodies in psoriatic patients treated with anti-TNF-α therapy.

TNF‐α inhibitors have been associated with induction of autoantibodies and autoimmune diseases. We retrospectively evaluated the incidence of autoantibodies ANA, ENA, anti‐dsDNA, the occurrence of clinical symptoms and possibly related treatment failure.

Cutaneous collagenous vasculopathy: report of a case

an itchy facial rash. His medical history was unremarkable apart from acne vulgaris as a teenager. On physical examination, striking tumid erythematous papules, plaques and nodules were seen predominantly on the lower cheeks, and scaly erythematous plaques over the central forehead, upper cheeks, chin and neck (Fig 1a). The eyebrows, beard and scalp appeared normal with no obvious hair loss, and the cutaneous findings were limited to the head and neck. The differential diagnoses considered included granulomatous rosacea, sarcoidosis and lupus erythematosus. Pending histological confirmation, twicedaily topical 0.1% tacrolimus ointment was commenced. On histological examination of an incisional biopsy taken from the left pre-auricular area, a prominent perifollicular inflammatory cell infiltrate was seen, comprised mainly of lymphocytes, with mucinous degeneration of the follicular epithelium (Fig. 2a,b). There was no significant lymphocyte atypia, and the lymphoid immunoprofile was normal. Follicular destruction and granulomatous inflammation were absent. Fungal, bacterial and mycobacterial cultures were negative, as was direct immunofluorescence. Alcian blue staining confirmed the presence of intrafollicular mucin deposits (Fig 2c). Findings were consistent with a diagnosis of benign FM. On review 10 days later, the patient’s facial eruption had dramatically improved (Fig 1b). Full blood count, erythrocyte sedimentation rate, lactate dehydrogenase, human T cell lymphotropic virus (HTLV)1 and HTLV2 serology, and lymphocyte immunophenotyping were all normal or negative. T-cell gene rearrangement studies confirmed a polyclonal population, and a final diagnosis of primary idiopathic FM was made. Treatment with topical 0.1% tacrolimus ointment was tapered over the following 4 weeks, and remission has been maintained for > 1 year. A single case of recalcitrant FM successfully treated with 1% pimecrolimus cream has previously been reported. The response to tacrolimus ointment in our case was unusually rapid, and spontaneous resolution of lesions cannot be excluded. The risk of activating what may be considered a premalignant condition remains a concern, and further studies evaluating the efficacy, safety and tolerability of topical tacrolimus ointment in FM are required before it can be recommended.

Novel p.Glu519Gln missense mutation in ST14 in a patient with ichthyosis, follicular atrophoderma and hypotrichosis and review of the literature.

[1] Jemec G.B, Clinical practice Hidradenitis suppurativa, N. Engl. J. Med. 366 (2012) 158–164. [2] G.R. Vinding, I.M. Miller, K. Zarchi, et al., The prevalence of inverse recurrent suppuration: a population-based study of possible hidradenitis suppurativa, Br. J. Dermatol. 170 (2014) 884–889. [3] I.M. Miller, C. Ellervik, G.R. Vinding, et al., Association of metabolic syndrome and hidradenitis suppurativa, JAMA Dermatol. 150 (2014) 1273–1280. [4] C.T. Esmon, Basic mechanisms and pathogenesis of venous thrombosis, Blood Rev. 23 (2009) 225–229. [5] J. Zacho, A. Tybjaerg-Hansen, B.G. Nordestgaard, C-reactive protein and risk of venous thromboembolism in the general population, Arterioscler. Thromb. Vasc. Biol. 30 (2010) 1672–1678. [6] O. Ahlehoff, G.H. Gislason, J. Lindhardsen, et al., Psoriasis carries an increased risk of venous thromboembolism: a Danish nationwide cohort study, PLoS One 6 (2011) e18125. [7] C. Schlapbach, T. Hanni, N. Yawalkar, et al., Expression of the IL-23/ Th17 pathway in lesions of hidradenitis suppurativa, J. Am. Acad. Dermatol. 65 (2011) 790–798. [8] H.K. Bergholdt, L. Bathum, J. Kvetny, et al., Study design, participation and characteristics of the Danish general suburban population study, Dan. Med. J. 60 (2013) A4693. [9] I.M. Miller, M.E. Johansen, U.B. Mogensen, et al., Coagulation status in hidradenitis suppurativa: a Danish populationand hospital-based crosssectional study, Dermatology (2015) . [10] F. Marongiu, G.G. Sorano, C. Bibbo, et al., Abnormalities of blood coagulation and fibrinolysis in psoriasis, Dermatology 189 (1994) 32–37.

Familial anetoderma: a report of two families

Anetoderma is a rare cutaneous disorder where a localized dermal defect of elastic fibers determines depressed areas and often herniated saclike skin. Primary anetoderma is an idiopathic phenomenon while secondary anetoderma is related to various conditions. The term primary anetoderma implies that the lesions occur in clinically normal skin although they may be associated with another dermatological or systemic disease or condition, without a well established relationship. The term secondary anetoderma implies that anetoderma occurred on the same site as another skin lesion. Familial anetoderma is a very rare condition that can be associated with bony, neurological and ocular anomalies. Recently some families with familial anetoderma have been described, where the disease seems to be limited to the skin. The pathogenesis for familial anetoderma is still unclear. It has been reported in only 10 families and in the first 4 reported families, anetoderma was always associated with extra-cutaneous abnormalities, while in the remaining 6 families, all described in last three decades, anetoderma was limited to the skin. We report here another two families with anetoderma without any associated disease and we review the literature on familial anetoderma.

Topical corticosteroids versus “wait and see” in the management of solitary mastocytoma in pediatric patients: a long‐term follow‐up

Management of patients affected by mastocytoma (MS) includes avoiding triggering factors of mast cell degranulation, and administration of symptomatic treatment. We evaluated topical steroid treatment efficiency on the clinical course of MS in a group of patients, comparing the results with another untreated group.We retrospectively evaluated clinical data of 176 patients under 15 years of age, affected by MS and referred to our Dermatological Pediatric Service from 1996 to 2010. Ninety‐one of 176 children were treated with topical steroids. Follow‐up was possible in 130 of 176 patients and lasted for 56.3 months on average. We compared 62 treated and 68 untreated patients. There was not statistic difference between the two groups: (i) in the number of healed or partially improved cases; and (ii) in the time of partial regression, although it is quicker with therapy. The time of healing is 16.4 months (on average) with treatment, and 34.7 months (on average, p = 0.001) without any treatment. The resolution of MS is independent of therapy administration, but the time of healing is statistically faster using the local steroids. An appropriate treatment with them is effective and safe, considering the long time needed for spontaneous resolution.

Hand, foot and mouth disease: an overview of clinical features in adult patients.

Editor Hand, foot and mouth disease (HFMD) mainly affects children aged <5 years and is caused by several serotypes of enteroviruses, more frequently Enterovirus 71 (EV71) and Coxsackie A16 (CA16). The disease presents with vesicles in the mouth, hands, feet and buttocks. Only 1% of infected adults develop clinical manifestations, probably as a result of immunological memory. Nevertheless, in the atypical HFMD caused by Coxsackie virus A6 (CVA6), the involvement of immunocompetent adults has been recently described. Adult cases (18 years of age and older) of atypical HFMD, referred to the Dermatology Unit in Bologna from October 2014 to January 2016, were enrolled for the study, if they met three inclusion criteria (Table 1). We identified 10 atypical HFMD lesions in immunocompetent adults transmitted by children: seven men and three women with a mean age of 39 12.59 years. Epidemiological, clinical and laboratory findings are summarized in Table 1. Seven of 10 patients presented prodromal symptoms, most frequently fever and sore throat. A similar frequency of prodromal symptoms was reported in a paediatric study. The cutaneous findings of our adult patients were similar to the atypical diffuse vesiculobullous forms of childhood, which is one of the four main patterns of atypical HFMD described in children. In our cluster, the extent of cutaneous lesions was mild in five of 10 patients, moderate in four of 10 patients and severe in one of 10 patients. A severe extension seems more common in children: at least 50% could present an involvement >25% BSA. All patients presented reddish pseudo-purpuric macules on the palms and soles. The lesions were vesiculobullous with an erythematous halo or pseudo-purpuric macules associated with vesicles on the palms and soles (Fig. 1a,b). Similar pseudo-purpuric lesions of palms and soles were reported in 78% and 17% of subjects, respectively, in two recent paediatric studies. The pseudo-purpuric pattern is the clinical expression of vesicles more deeply located in the epidermis and is more frequent in adults, probably due to the greater thickness of the palmoplantar epidermis. In addition, five of 10 patients manifested perioral involvement and four of these also perinasal (Fig. 1c,d). Perianal involvement was absent, this site being more prone to irritation in childhood. The lower limbs were involved in three of 10 patients (Fig. 1e), the upper limbs and abdomen in one of 10 patients and the scalp in three of 10 patients (Fig. 1f). Curiously in one patient affected by psoriasis, the HFMD lesions first appeared on the psoriasis plaques (Fig. 1g). As is known, Coxsackie virus tend to diffuse on areas with an altered cutaneous barrier, such as on eczematous skin (eczema coxsackium), more frequent in paediatric age. One patient presented desquamation of the palms and soles 6 weeks after onset, while another presented orchiepididymitis 7 days after CVA6 infection. Onychomadesis was seen in three of 10 patients within 3–8 weeks after diagnosis and was associated (a) (b)

Nodular scabies in infants: dermoscopic examination may avoid a diagnostic pitfall

infantile scabies sometimes manifests with atypical clinical presentation, leading to misdiagnosis. Monomorphic primary lesions or different coexisting lesions may be present. Specific pediatric localizations are scalp and face1, but in infants the most common are palms, soles and axillae. Irritability and poor feeding are more often referred by parents than itching. This article is protected by copyright. All rights reserved.

A perineal infantile haemangioma presenting as early ulcerations

A 20-day-old, otherwise healthy female presented with a 2-week history of small perianal ulcers. These lesions appeared on the fourth day of life as some discrete ulcers overlying an area of macular erythema on the right buttock. The ulcers enlarged slowly, despite two courses of topical antibiotics for impetigo. She was the product of a full-term pregnancy. Birth weight was 3870 g. Physical examination showed multiple perianal ulcerations coalescing …

Cutaneous Mastocytosis Exacerbated by Pinworms in a Young Boy

Abstract:  Cutaneous mastocytosis in children has an indolent course and undergoes spontaneous regression. Many triggering factors may cause mast cell degranulation and clinical manifestations. Knowledge of these factors is important for patients and their families. We report a case of exacerbation of urticaria pigmentosa due to mast cell degranulation caused by Enterobius vermicularis, which has not been reported before as a triggering factor.

Comment on “Scabies in babies”

To the Editor, We read with great interest the article by Hill and colleagues, which confirms that infantile scabies sometimes manifests with atypical clinical presentation, leading to misdiagnosis. We would like to comment on four issues. All infants included in this study initially presented with polymorphic scabies; intensely inflammatory papulovesicles and nodules, pustules, hemorrhagic crusting, and serpiginous or J-shaped burrows were described. In contrast, in our case series, nodular scabies was the primary and monomorphic presentation in nine of 15 infants. In our group, the most commonly involved body area in infants (≤2 years old) was the armpits, confirming the results reported by Boralevi et al. Urticaria pigmentosa could be considered in the differential diagnosis, although the Darier sign may be positive also in scabietic nodules because of a local increase in mast cells. The recognition of age-related variations in morphology and clinical pattern is important to diagnose scabies, although in doubtful cases, dermatoscopy permits a rapid, noninvasive examination of many suspicious sites, differentiating reactive from active nodules with sensitivity of 91% and specificity of 86%. These levels of accuracy are comparable with those of microscopic examination, but dermoscopy is a more-rapid, noninvasive examination, especially in infants. A handheld dermatoscope, at a magnification of 910, shows mites as typical small, brown pigmented triangular structures (hang-glider sign), as well as S-shaped white burrows or jet-liner sign (Figure 1). In conclusion, in infants, a careful family history and a precise clinical examination are useful for the diagnosis of typical scabies. In the case of atypical monomorphic nodular eruption, more frequent in infants, handheld dermatoscopy is a rapid, noninvasive tool for diagnosis and to facilitate the differential diagnosis with urticaria pigmentosa, Langerhans and non-Langerhans cell histiocytosis, juvenile xanthogranuloma, and leukemia cutis.