www.medigraphic.org.mx Medicina Cutánea Ibero-Latino-Americana Localizador: 17057 Ledderhose’s disease associated with Dupuytren’s disease in a patient with acquired immunodeficiency syndrome: first-time report Doença de Ledderhose associada à doença de Dupuytren em paciente com síndrome da imunodefi ciência humana: primeiro relato Raquel Nardelli de Araujo,* Renata Marcarini,* Aaron Alarcon Novillo,‡ Daniela Alves Pereira Antelo§ Key words: Plantar fibromatosis, ABSTRACT RESUMO Ledderhose’s disease, acquired The plantar fibromatosis (PF), also known as Ledderhose’s A fibromatose plantar (FP), também conhecida como doença de immunodeficiency disease, is characterized by affecting the plantar aponeurosis. Ledderhose, é uma doença caracterizada por acometer a syndrome. In a general way, it is a benign fibrous condition still quite aponeurose plantar. Trata-se de uma condição fibrosa benigna unclear in its various aspects, such as its etiology and treatment. ainda pouco clara nos seus diversos aspectos, como a etiologia Palavras-chave: To date, it has not been described in association with AIDS. e o tratamento. Descrevemos o caso de um paciente de 45 anos Fibromatose Our patient presented PF previously hospitalization due to do sexo masculino portador de síndrome da imunodeficiência plantar, doença de occurrence of many opportunistic infections due to AIDS at an adquirida em fase avançada, associado à doença de Dupuytren Ledderhose, síndrome advanced stage. We have described the case of a 45 year-old (DD) que evoluiu com o surgimento da FP bilateral. O tratamento da imunodeficiência male patient with acquired immunodeficiency syndrome at a foi conservador, pois as lesões plantares eram assintomáticas adquirida. late stage, associated with Dupuytren’s disease (DD) and the e o risco de recorrência com medidas invasivas é elevado. Em emergence of bilateral PF. Treatment was conservative as the torno de 15% dos acometidos com FP possuem contratura plantar lesions were asymptomatic and the risk of recurrence de Dupuytren concomitante. Pacientes com HIV com doença with invasive measures is high. About 15% of people affected avançada apresentam associação com DD em até 36% dos with PF have concomitant Dupuytren’s contracture. HIV casos. Para o nosso conhecimento, esse é o primeiro caso em patients with advanced disease have DD in combination in up que se observa a associação dessas três condições em um único to 36% of cases. To our knowledge, this is the first case in which paciente. Apesar da notória necessidade de pesquisas voltadas * Resident in Dermatology. ‡ Resident in Radiology. there is association of these three conditions in a single patient. para a FP, acreditamos que este artigo possa corroborar para § Master’s and PhD in Despite the evident need for research on PF, we believe that this futuros esclarecimentos sobre a doença. Dermatology - Associate paper may contribute to further elucidation about the disease. Professor of Dermatology Department. Pedro Ernesto University Hospital, University of the INTRODUCTION rarity of the disease. The incidence increases State of Rio de Janeiro, Rio with advancing age, although it can occur in de Janeiro-RJ, Brazil. lantar fibromatosis (PF), Ledderhose’s childhood. In addition, man are affected twice Confl ict of interest: Pdisease, is an uncommon benign fibrous as often as females.4 None. disorder involving the plantar aponeurosis, In this article, we report the case of a and was first reported in 1894 by Dr. Georg male patient of 45 years old, with human Received: Ledderhose.1 It is one of the superficial immunodeficiency syndrome at an advanced 01/November/2017. fibromatoses alongside Dupuytren’s disease, Accepted: stage, with PF and DD clinical settings. To our 11/June/2018. KnucklePads,www.medigraphic.org.mx the Infantile Digital Fibromatosis knowledge, there is no reported case involving and Peyronie’s disease.2 The exacerbated these three conditions in the same patient. proliferation of connective tissue can form irregular masses or nodules in the plantar CASE REPORT of the aponeurosis, especially in the central band.2,3 Its incidence, as well as its etiology and A 45 year-old male patient, born in Rio de treatment, are not clearly defined yet due to the Janeiro, has had confluent firm nodules in the Med Cutan Iber Lat Am 2018; 46 (3): 209-212 www.medigraphic.com/medicinacutanea Nardelli de Araujo R, et al. Ledderhose’s disease associated with Dupuytren’s disease CLINICAL CASE / THERAPEUTIC plantar surface of both feet for about 10 years (Figure 1). Conservative measures to treat PF were adopted since He reports that the injuries began in his left foot and two the patient did not complain of pain on the injuries. He years later, similar injuries were also noticed in the right was referred to the orthopedics for surgical treatment of foot. He denies similar family cases, trauma, pain or other the contracture due to the limitations resulting from this manifestations on the lesion’s site. clinical setting. Seven years ago, the patient needed several hospital admissions due to opportunistic infections, when he DISCUSSION was diagnosed with acquired human immunodeficiency syndrome. He presented neurotoxoplasmosis, The fibromatoses represent a group of diseases neurosyphilis, pneumocystosis, cytomegalovirus colitis, characterized by fibroblast proliferation with similar pseudomembranous colitis, hepatitis B, Kaposi sarcoma, histological appearance and are classified as superficial pulmonary tuberculosis and Dupuytren’s contracture of and deep.2 Superficial fibromatosis include hand injuries the right hand with palpable nodule between the fourth (Dupuytren’s contractures, knuckle pads, Infantile digital and fifth fingers and retraction of the fifth finger (Figure 2). He used various medications in the period, among them phenytoin to control recurrent tonicoclonic seizures. He underwent ultrasound examination with a 14 MHz linear transducer on the plantar surface of both feet which revealed massive expansive heterogeneous and solid formation located in the middle of the left plantar aponeurosis without significant vascular flow to the amplitude Doppler, with measurements of 67 x 12 x 35 mm in larger diameters (Figure 3). The radiological aspect was compatible with plantar fibroma. On the right side, hypoechoic thickening of the middle third of the plantar aponeurosis was also observed, which measures 28 x 4 mm in larger diameters, also consistent with fibromatosis. Histopathological analysis showed presence of dense collagen fibers proliferation in the dermis, as seen in the description of PF. Figure 2. Dupuytren’s contracture of the right hand. www.medigraphic.org.mx Figure 1. Firm nodules and well-defi ned limits on the plantar surface Figure 3. Presence of fi rm nodules, well-defi ned limits with large fi broid of both feet. and mixed echogenicity located in the plantar aponeurosis. Med Cutan Iber Lat Am 2018; 46 (3): 209-212 210 www.medigraphic.com/medicinacutanea Nardelli de Araujo R, et al. Ledderhose’s disease associated with Dupuytren’s disease CLINICAL CASE / THERAPEUTIC fibromatosis), feet injuries (plantar fibromatosis), and penis located in or on the plantar fascia. Larger dimensions are injuries (Peyronie’s disease). The palmar fibromatosis is unusual and tend to move the fascia profoundly. In our the most common of them, occurring in 1 to 2% of the case, the patient had 67 mm lesions on the left plantar population with male predominance, usually bilaterally.2 region and 29 mm on the right one. It was suggested that The plantar fibromatosis (PF) affect a younger age group, the development stage of the lesion may influence the affecting men twice as often as women and are bilateral in physical and sonographic appearance. The superficial 20 to 50% of cases.2,4 The PF are well defined slow-growing location and appearance should strongly suggest the lesions and are usually located in the medial surface of the diagnosis, but should not be considered pathognomonic. plantar fascia.4,5 The nodules may be multiple in 33% of Although MRI is effective in assessing the infiltration cases and are generally asymptomatic. However, they can of surrounding tissues, and, thus, is important in the be locally aggressive and produce pain when the lesion preoperative period, its high cost and low availability involves the neurovascular bundles, muscles, or tendons. restrain its use. The advent of transducers with frequencies Approximately 15% of patients with PF have Dupuytren’s over 7.5 MHz with better resolutions, make the ultrasound disease in association.4,6 a suitable method to study PF.4,10 There are numerous conditions associated with Microscopically, there is fibroblast proliferation superficial fibromatoses. Among them, DD is the most organized in parallel fascicles, with elongated and studied one, having alleged connections with heredity, hyperchromatic nucleus, dense deposit of collagen fibers alcohol, epilepsy, trauma, diabetes, rheumatoid arthritis, and occasional mitoses. The old and well-developed gout and HIV.7,8 lesions are hypocellular, possessing greater amount of Concerning epilepsy, it was noted that the actual link dense collagen and mature fibrous tissue.11 would be through anticonvulsants rather than the genetic Microscopically, there is fibroblast proliferation association. The suggested mechanism is the stimulation of organized in parallel fascicles but usually in nodules, with tissue growth factors by anticonvulsants, causing its users elongated and hyperchromatic nucleus,