Ana Brasileiro

Cutaneous leukemic infiltration following varicella - a case of Wolf's isotopic response

Wolfs isotopic response designates the appearance of two subsequent unrelated dermatoses in the same anatomic location. We report the case of a 51-year-old man with a medical history of chronic lymphocytic leukemia without known extra-hematopoietic involvement. The patient developed a disseminated papulo-vesiculous eruption, diagnosed as varicella. Few days after recovering, an erythematous and violaceous papular dermatosis with histopathological examination compatible with leukemic infiltration appeared on the scars of previous herpetic lesions. Complete remission was obtained under systemic corticotherapy, without cutaneous recurrence or blastic transformation. Wolfs isotopic response is attributed to a localized immunologic imbalance following a certain stimulus. In this patient, herpetic infection acted as a local spur for inaugural cutaneous leukemic infiltration, with no impact on the prognosis for the underlying disease.

Facial Lesions in Children: Often a Challenging Diagnosis

CASE REPORT An 11-year-old girl was observed with mildly pruritic facial lesions consisting of well-demarcated symmetric erythematous scaly plaques involving both eyelids, isolated erythematous and scaly papules on the forehead, base of the nose and malar regions and a crusted-yellowish plaque with less well-defined borders on the tip of the nose (Fig.1). Lesions began 3 weeks before as mildly pruritic papules around the eyes that progressively increased. No other skin lesions were observed and systemic symptoms were absent. The child was otherwise healthy and parents denied personal or family history of atopy. No other dermatoses were known in the family. The dermatitis was initially diagnosed as eczema and a mid-potency topical corticosteroid cream was prescribed. Due to lesional extension with the occurrence of papules and pustules within the next week, an oral beta-lactam antibiotic was prescribed without improvement. On reviewing the history, the child reported she used to play with a pet rabbit. Revista SPDV 76(3) 2018; Lesões faciais em criança; Borges AS, Brasileiro A, Matos Pires E, Baptista J, Galhardas C, Apetato M.

Tinea faciei in a central Portuguese hospital: A 9-year survey

Tinea faciei is a relatively uncommon dermatophytosis that affects the glabrous skin of the face. The aim of this study was to analyse the epidemiologic, clinical and mycological features of tinea faciei cases diagnosed at the Dermatology and Venereology Department of Hospital Santo António dos Capuchos (Lisbon, Portugal). Consecutive cases diagnosed between 2008 and 2016 were studied retrospectively. A total of 72 tinea faciei cases have been diagnosed, involving 37 male and 35 female, aged between 8 months and 86 years. The majority were observed in patients younger than 12 years of age (59.72%). Anthropophilic isolates (mainly Microsporum audouinii, Trichophyton soudanense and Trichophyton rubrum) accounted for 75.7% of the identified dermatophytes. One quarter of the patients were also affected by dermatophytosis in other areas, such as the scalp. Only 10 cases were previously treated with topical steroids due to misdiagnosis. Most patients were treated with topical and systemic antifungal therapy with total resolution of skin lesions, without relapse or side effects. In contrast to other European studies, anthropophilic dermatophytes were the main causative agents of tinea faciei. As previously described to tinea capitis, this result is probably due to changes in the epidemiology of dermatophytes worldwide.

Systemic allergic dermatitis caused by sodium metabisulfite in rectal enemas: SYSTEMIC CONTACT DERMATITIS CAUSED BY RECTAL ENEMAS

Sulfites are widely used as preservatives and antioxidants in food and beverages, but also in cosmetic and pharmaceutical products (1) and medical devices (2). Allergic contact dermatitis caused by sodium metabisulfite has been increasingly recognized in recent years, and positive patch test reactions seem to be relevant in most patients if carefully investigated (3, 4). Furthermore, systemic allergic reactions to sulfites have also been described following oral and/or parenteral exposure (5–7).

Mucosal and Periungual Telangiectasia as Signs of Systemic Disease

Telangiectasias are a typical feature of systemic sclerosis, mostly in localized variant, and often involve lips and palms.1 However, they can be present in other connective tissue diseases or familiar disorders as hereditary hemorrhagic telangiectasia and Peutz-Jeghers syndrome.2 Nail fold capillary abnormalities, sclerodactyly and digital pitting scars are useful in supporting the diagnosis.3 The frequent internal involvement, with pulmonary disease being the leading cause of death, justifies the importance of high clinical suspicion and close monitoring of these patients. Mucosal and Periungual Telangiectasia as Signs of Systemic Disease

Pseudoainhum numa Doente com Doença Mista do Tecido Conjuntivo: Uma Associação Incomum

The term pseudoainhum is used to describe constricting band(s) around one or more digits in relation to several congenital or acquired diseases. Systemic sclerosis, keratoderma, psoriasis, Reynolds syndrome among others, has been reported to be associated with pseudoainhum development. We report a case of a 54 years old woman with annular constricting bands and micronychia on the fourth and fifth digits of her both hands associated with bilateral distal acrosclerosis. Raynaud’s phenomenon with some years of evolution and, more recent, fatigue and recurrent inflammatory polyarthritis and also antinuclear and anti-U1 nuclear ribonucleoprotein antibodies were present. Therefore, the diagnoses of pseudoainhum with a likely association with mixed connective tissue disease were proposed. Whenever in the presence of pseudoainhum, a detailed workup, including autoimmune disorders screening, is mandatory in order to rule out possible associated syndromes or underlying diseases. The authors present this case as the association between pseudoainhum and mixed connective tissue disease has not been previously reported in the literature.

Implicações Terapêuticas dos Novos Conhecimentos Sobre a Fisiopatologia da Dermite Atópica

A dermite atopica e uma dermatose inflamatoria cronica que afeta principalmente criancas, podendo tambem manifestar- -se em idade adulta. Apesar de estar associada a um forte impacto na qualidade de vida, os avancos terapeuticos sao ainda muito precarios. Novos conhecimentos no âmbito da fisiopatologia da dermite atopica, realcando o papel crucial da disfuncao da barreira epidermica e sua relacao com a desregulacao imunitaria, tem permitido o desenvolvimento de possiveis armas terapeuticas.

Switching biologics in severe pediatric psoriasis: a retrospective analysis

References 1 Thomas V, Dobson R, Mennel R. Primary cutaneous large Bcell lymphoma, leg type. Proc (Bayl Univ Med Cent) 2011; 24: 350–353. 2 Jurisi c V, Ple ci c M, Colovi c N, et al.MUM-1 and bcl-2 positive primary diffuse large B cell non-Hodgkin’s lymphoma of the colon. Arch Iran Med 2016; 19: 297–299. 3 Swerdlow SH, Campo E, Harris NL, et al., eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press, 2008. 4 Senff NJ, Noordijk EM, Kim YH, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008; 112: 1600–1609. 5 Hallermann C, Niermann C, Fischer RJ, et al. Survival data for 299 patients with primary cutaneous lymphomas: a monocentre study. Acta Derm Venereol 2011; 91: 521–525. 6 Suarez AL, Pulitzer M, Horwitz S, et al. Primary cutaneous Bcell lymphomas: part I. Clinical features, diagnosis, and classification. J Am Acad Dermatol 2013; 69: e1–e13. 7 Onoyama I, Nakayama KI. Fbxw7 in cell cycle exit and stem cell maintenance: insight from gene-targeted mice. Cell Cycle 2008; 21: 3307–3313. 8 Gokdemir G, Ari S, Altunay I, et al. Primary cutaneous diffuse large B-cell lymphoma of the leg, with an atypical clinical picture of verrucous plaques associated with stasis dermatitis. Clin Exp Dermatol 2010; 35: e87–e89. 9 Belousova IE, Vanecek T, Skreg SV, et al. Unusual clinicopathological presentation of primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple nodules and widespread garland-like lesions. Am J Dermatopathol 2009; 31: 370–374. 10 Tessoulin B, Eveillard M, Lok A, et al. p53 dysregulation in Bcell malignancies: more than a single gene in the pathway to hell. Blood Rev. 2017. Available at: https://doi.org/10.1016/j.blre. 2017.03.001 (last accessed 17 April 2017). 11 Song JH, Schnittke N, Zaat A, et al. FBXW7 mutation in adult T-cell and B-cell acute lymphocytic leukemias. Leuk Res 2008; 32: 1751–1755. 12 Oniyama I, Nakayama KI. Fbxw7 in cell cycle exit and stem cell maintenance. Cell Cycle 2008; 7: 1–7.

Livedo reticularis after intra-articular hyaluronic acid injection

A 74‐year‐old man presented with a 10‐day history of painful skin eruption on the left knee. The eruption developed 1 week after intra‐articular hyaluronic acid injections on both knees for treatment of osteoarthritis. Examination revealed a blanchable erythematous reticulate patch on the left knee with a violaceous center [Figure 1]. The right knee was normal. Doppler ultrasonography and blood analysis revealed no pathological findings. A punch biopsy of the skin was performed. Histopathological examination showed an amorphous basophilic foreign material consistent with hyaluronic acid present within the vessels of the dermis and hypodermis with associated thrombus formation [Figures 2 and 3]. With a clinical and histological correlation, a diagnosis of livedo reticularis due to intra‐articular hyaluronic acid injection entering the cutaneous vasculature was confirmed. Since there was no evidence of necrosis, the patient was advised rest and treated conservatively with analgesic. The lesion resolved after 4 weeks.

SAT0412 Biological Therapy in Psoriatic Arthritis (PSA): Differences between Switchers and Non-Switchers

Background Switching biological therapies is becoming increasingly common in routine management of PsA patients. However, evidence in this topic is still scarce. Predictive markers for an optimal approach to the sequential prescription of biologics are lacking. Objectives In a population of PsA patients, we aimed to determine differences in baseline clinical and laboratory features between switchers and non-switchers. Methods We conducted a retrospective analysis of the PsA patients followed at our outpatient clinic in the last 24 months. Demographic, clinical and laboratory data were collected. Severity of skin manifestations and peripheral arthritis at PsA onset were recorded using PASI and DAS28 score. Patients who changed biological therapies due to therapeutic failure (primary or secondary) or toxicity were defined as switchers. For comparison of clinical and laboratory features we used chi-square test for categorical variables and Mann-Whitney or T-Student test for continuous variables. Results 58 PsA patients were included, 60.3% were women; the mean age at PsA and psoriasis (PsO) onset was 46.4 (±15) and 34.8 (±15) years, respectively. At PsA onset, 24.1% of patients had a PASI score>10 and a mean DAS of 3.76 (±1.0). Laboratory features, arthritis and extra-articular manifestations were assessed (Image). 94.8% of patients were prescribed with non-biologic DMARD therapy and 55.2% with biological therapy, after failing to respond to a classic DMARD treatment alone. Median time from diagnosis until biological therapy prescription was 25.9 (±56,5) months. Out of 32 patients prescribed with biologics, 15 (46.9%) switched to another biologic agent, mainly due to secondary failure (63.6%), primary failure (18.2%) and adverse events (13.6%). 15.65% and 3.1% of patients required 2 or 3 switches. Etarnacept was the first line agent prescribed in the majority (78.8%) of patients. Adalimumab (73.3%) and ustekinumab (50.0%) were the most used agents as second and third line biological therapies, respectively. Switchers were significantly younger at psoriasis onset (25.6 (±13.8) vs 37.4 (±13.3) years, p=0.020) and also at PsA onset (33.3 (±9.6) vs 50.0 (±13.1) years, p<0.0001). They had higher PASI (p=0.011) levels comparing to non-switchers. Uveitis and axial involvement were significantly more common in switchers (p=0.047 and p=0.034, respectively). All patients starting biological therapy on the first 12 months after disease onset switched to another biological agent. Conclusions Age at PsO and PsA onset is a well-established clinical factor with significant impact in disease severity and in skin and arthritis manifestations. Our work suggests that it might also play a role in first biological treatment failure determining a need to switch. Higher PASI score at PsA onset, axial involvement and uveitis also seem to be associated with more likelihood of switching. Our results should be considered exploratory. Larger studies are required to determine the role of these clinical features in predicting biological treatment switching. Disclosure of Interest None declared