Maria da Luz Duarte

A twenty-year survey of dermatophytoses in Braga, Portugal

Background  Modifications in social habits together with the increase of emigration have contributed not only to increased dermatophytoses but also to an altered etiology. During the last few years, Braga has suffered a radical change from a rural to a cosmopolitan life‐style.

Indeterminate cell histiocytosis in association with acute myeloid leukemia.

Indeterminate cell histiocytosis (ICH) is a rare proliferative disorder, in which the predominant cells share morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytosis. We describe a 62-year-old man presenting a 2-month history of firm nodular lesions on the upper lip. Histopathology, immunohistochemical, and ultrastructural analysis showed typical findings of ICH. The patient was treated with thalidomide and almost complete regression of the lesions was reached within 7 months. Nevertheless, one month after remission, he developed an acute myeloid leukemia of the subtype monocytic leukemia (M5). The patients condition rapidly worsened and he died due to a respiratory failure four weeks later. We present this case because apart of being rare it joins the effectiveness of thalidomide and the association with an acute monocytic leukemia. A review of the literature is made.

Cutaneous Phaeohyphomycosis Caused by Alternaria alternata Unresponsive to Itraconazole Treatment

Cutaneous alternariosis is an opportunistic infection that has been described mainly in patients treated with corticosteroids. We report a case of dermal alternariosis occurring in a woman treated with corticosteroids after a neurosurgery that was unresponsive to itraconazole. Treatment with intravenous voriconazole was initiated, followed by oral protocol, with marked improvement of the lesions.

Colloid milium successfully treated with MAL-PDT

1 Raj S, Calonje E, Kraus M, et al. Cutaneous pilar leiomyoma: clinicopathologic analysis of 53 lesions in 45 patients. Am J Dermatopathol 1997; 19: 2–9. 2 Akay BN, Boyvat A, Heper AO, et al. Congenital pilar leiomyoma. J Am Acad Dermatol 2008; 59: S102–S104. 3 Gokdemir G, Altunay IK, Koslu A, et al. A case of multiple facial painless leiomyomata. J Eur Acad Dermatol Venereol 2000; 14: 144–145. 4 Tomlinson IP, Alam NA, Rowan AJ, et al. Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer. Nat Genet 2002; 30: 406–410. 5 Alam NA, Barclay E, Rowan AJ, et al. Clinical features of multiple cutaneous and uterine leiomyomatosis: an underdiagnosed tumor syndrome. Arch Dermatol 2005; 141: 199–206. 6 Badeloe S, van Geel M, van Steensel MA, et al. Diffuse and segmental variants of cutaneous leiomyomatosis: novel mutations in the fumarate hydratase gene and review of the literature. Exp Dermatol 2006; 15: 735–741. 7 Stewart L, Glenn GM, Stratton P, et al. Association of germline mutations in the fumarate hydratase gene and uterine fibroids in women with hereditary leiomyomatosis and renal cell cancer. Arch Dermatol 2008; 144: 1584– 1592. 8 Sifaki MK, Krueger-Krasagakis S, Koutsopoulos A, et al. Botulinum toxin type A – treatment of a patient with multiple cutaneous piloleiomyomas. Dermatology 2009; 218: 44–47. 9 Gravvanis A, Kakagia D, Papadopoulos S, et al. Dermal skin template for the management of multiple cutaneous leiomyomas. J Cutan Med Surg 2009; 13: 102–105.

Port-wine stain as a clue for two rare coexisting entities

Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities.1 Its pathogenesis is not elucidated, and the prognosis is mainly determined by the presence of extracutaneous manifestations, such as Klippel-Trenaunay syndrome (KTS), that is defined by the triad of a port-wine stain (PWS), anomalous veins and progressive overgrowth of the affected extremity. Herein, we report a case of an adult patient, who presented with a large PWS, nevus of Ota, ocular melanosis, and limb hypertrophy and varicosities. These findings represented a rare association of PPV type IIb and KTS.

Cutaneous nocardiosis by a new pathogenic species: Nocardia grenadensis

Nocardiosis is a rare, predominantly opportunistic, suppurative disease caused by bacteria of the order Actinomycetales. There are currently more than 100 species of Nocardia described, less than half are pathogenic to humans. Cutaneous nocardiosis can be caused by direct inoculation from a contaminated material or by secondary dissemination. The authors present a 70-year-old man with an autoimmune haemolytic anaemia treated with prednisolone and azathioprine. The patient presented multiple erythematous tender nodules with linear distribution and proximal progression along the left upper limb with 2 months of evolution. The skin biopsy revealed non-specific inflammation with areas of abscess. Culture was positive for bacteria of the genus Nocardia, and molecular techniques revealed Nocardia grenadensis. The patient was treated with minocycline with good response, but the disease recurred. N. grenadensis was first identified in 2012 in a bioprospecting process. The authors now describe the first case of cutaneous nocardiosis caused by N. grenadensis.

DERMATOFITIAS NO DISTRITO DE BRAGA – ESTUDO RETROSPECTIVO DOS ÚLTIMOS 11 ANOS (1999 – 2009)

Objectivos : Avaliar a prevalencia das micoses superficiais, agentes etiologicos envolvidos e caracteristicas epidemiologicas da populacao da consulta de Dermatologia do Hospital de Braga, observada por dermatofitia, nos ultimos 11 anos; analisar a distribuicao de especies em relacao ao grupo etario e area cutânea envolvida; comparar os resultados com estudos anteriores referentes a mesma area geografica. Material e Metodos : Exame micologico directo e cultura de 5825 amostras colhidas em doentes observados na consulta do Servico de Dermatologia do Hospital de Braga, entre Janeiro de 1999 e Dezembro de 2009, com suspeita clinica de dermatofitia. Resultados : Durante o periodo em estudo, das 5825 colheitas realizadas em doentes com suspeita clinica de infeccao fungica, foi identificada a presenca de dermatofito em 1143 (19,6%). A apresentacao clinica da infeccao por dermatofito mais frequente foi a tinea corporis (44,8%). Globalmente, o agente etiologico mais frequentemente isolado foi o Trichophyton rubrum (43%), tendo sido tambem o principal agente na tinea cruris (73,5%), tinea ungueum (74,8%), tinea pedis (72,7%) e tinea manuun (57,7%). O T. mentagrophytes var. granulare foi o segundo agente mais frequentemente isolado, e a principal causa de tinea corporis (38%) e tinea barbae (76,9%). O Mycrosporum canis foi o agente mais frequente na tinea capitis (67,1%). Discussao : As infeccoes por dermatofitos sao frequentemente observadas na pratica clinica. Varios trabalhos tem constatado diferencas etiologicas em diferentes regioes e ao longo dos anos, provavelmente relacionadas com habitos de vida distintos. Os nossos resultados sao globalmente concordantes com os resultados relatados noutros trabalhos efectuados em Portugal.

TINHA DO COURO CABELUDO POR TRICHOPHYTON VIOLACEUM EM ADULTO COM LÚPUS DISCÓIDE

A tinha do couro cabeludo e uma infeccao causada por dermatofitos. Os fungos mais frequentemente implicados no Hospital de Braga sao o Microsporum canis e o Trichophyton mentagrophytes var. granulare. Trata-se de uma infeccao frequente em criancas mas rara nos adultos. O Trichophyton violaceum e um dermatofito antropofilico raro nos paises desenvolvidos. Descreve-se o caso clinico de uma mulher adulta, com historia de lupus discoide, com tinha do couro cabeludo por Trichophyton violaceum.

Kikuchi´s disease of the xanthomathous type with cutaneous manifestations

Kikuchis disease is a benign, self-limiting disease, whose pathogenesis remains unknown. Patients most often present with cervical lymphadenopathy, sometimes associated with fever and leukopenia. It has been reported that up to 40% of patients with Kikuchis disease have also cutaneous eruptions, but no specific skin changes have been described. Kikuchis disease can be subclassified into three histologic subtypes: a proliferative type, a necrotizing type and a xantomathous type. Most patients with Kikuchis disease require no specific treatment, because the disease regresses spontaneously, within a few weeks to months. We report a case of a 31-year-old woman with xanthomatous type of Kikuchis disease, whose first manifestation was the onset of erythematous papules with central suppuration on her face and on her left hand.

Imunoglobulinas endovenosas em dermatologia – experiência clínica de 7 anos no Hospital de Braga

Introduction: In recent years, the clinical experience with the use of intravenous immunoglobulin (IvIg) in Dermatology has increased. Despite limited information in literature, off-label use of IvIg has shown efficacy in the treatment of several refractary dermatosis to conventional treatments. Material and methods: We conducted a retrospective study of patients with dermatological pathology treated with IvIg between January 2004 and October 2011 in the Department of Dermatology, Hospital de Braga. We analyzed the demographic and clinical characteristics, therapies performed, clinical response and safety profile. Results: 21 patients were treated with IvIg in 10 different dermatological conditions: four patients with pemphigus vulgaris [2 with complete response (CR), one with partial response (PR) and another who interrupted treatment due to severe side effects]; two patients with bullous pemphigoid (one with CR and one with PR); three patients with Toxic Epidermal Necrolysis (TEN) with CR; two patients with dermatomyositis (both PR); four patients with Chronic Urticaria (one with CR, one with PR, one who did not respond and another who discontinued treatment for side effects); two patients with livedoid vasculopathy with PR, a patient with CREST syndrome that has not improved; a patient with Scleromyxedema with PR; a patient with Gangrenous Pyoderma with CR and one patient with Atopic dermatitis who discontinued treatment due to pregnancy. With the exception of three patients with TEN, all other patients had refractory disease to conventional systemic treatments. Conclusions: Although our experience is limited, treatment with IvIg may be beneficial in certain pathologies that do not improve with conventional treatment. Given its high cost and variable therapeutic effect, their use should be cautious until more studies define the risk-benefit ratio.