Document downloaded from http://www.elsevier.es, day 26/02/2019. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. r e v c o l o m b r e u m a t o l . 2 0 1 8;2 5(1):63–68 www.elsevier.es/rcreuma Case report ଝ Eosinophilic fasciitis: A case report a,∗ a b,c Amalia Londono˜ Correa , Ángela Londono˜ , Ana Cristina Ruiz , d,e Miguel Mesa a Departamento de Dermatología, Facultad de Medicina, Universidad CES, Medellín, Colombia b Departamento de Dermatopatología, Facultad de Medicina, Universidad CES, Medellín, Colombia c Dermatopatología Clínica CES y Hospital Pablo Tobón Uribe, Laboratorio de Patología, Medellín, Colombia d Departamento de Reumatología, Clinica Universitaria Bolivariana, Medellín, Colombia e Departamento de Reumatología, Facultad de Medicina de Escuela de Ciencias de la Salud, Universidad Pontificia Bolivariana, Medellín, Colombia a r t i c l e i n f o a b s t r a c t Article history: Eosinophilic fasciitis is a rare connective tissue disease. It is characterized by a progressive Received 29 September 2016 and symmetrical induration and thickening of the skin and soft tissues of the limbs. In Accepted 22 March 2017 addition to the skin manifestations, the joints and muscles are also involved, and in rare Available online 5 October 2018 cases there can be systemic involvement. The diagnosis of EF is based on clinical findings, the presence of peripheral blood eosinophilia, and a full-thickness biopsy that should include Keywords: the deep fascia in order to show the inflammatory infiltration that is mostly composed of Eosinophilic fasciitis lymphocytes and eosinophils. Shulman disease Systemic corticosteroids remain the treatment of choice and may be combined with an Dermal sclerosis immunosuppressive drug. © 2016 Asociacion´ Colombiana de Reumatologıa.´ Published by Elsevier Espana,˜ S.L.U. All rights reserved. Fascitis eosinofílica: reporte de un caso r e s u m e n Palabras clave: La fascitis eosinofílica es una enfermedad rara del tejido conectivo que se caracteriza por Fascitis eosinofílica induración y engrosamiento progresivo y simétrico de la piel y del tejido celular subcutáneo Síndrome de Shulman localizado, principalmente, en las extremidades. Además de las manifestaciones cutáneas Esclerosis localizada hay compromiso articular, muscular y, en casos excepcionales, compromiso sistémico. PII of original article: S0121-8123(17)30042-7 ଝ Institución donde se realizó el trabajo: Clínica CES y Clínica UPB, Medellín Colombia. Rev Columb Reumatol. 2018;25:63–68. ∗ Corresponding author. E-mail address: [email protected] (A. Londono˜ Correa). 2444-4405/© 2016 Asociacion´ Colombiana de Reumatologıa.´ Published by Elsevier Espana,˜ S.L.U. All rights reserved. Document downloaded from http://www.elsevier.es, day 26/02/2019. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. 64 r e v c o l o m b r e u m a t o l . 2 0 1 8;2 5(1):63–68 Su diagnóstico se basa en los hallazgos clínicos, eosinofilia en sangre periférica y la toma de una biopsia profunda de piel, que incluya la fascia donde se evidencia un infiltrado com- puesto por linfocitos y eosinófilos. El tratamiento de elección son los esteroides sistémicos acompanados˜ de medicamentos inmunosupresores. © 2016 Asociacion´ Colombiana de Reumatologıa.´ Publicado por Elsevier Espana,˜ S.L.U. Todos los derechos reservados. Introduction Eosinophilic fasciitis (EF) is a rare disease of the connective tis- sue characterized by edema, induration, and thickening of the 1 skin and soft tissues. It affects men and women equally and 2,3 its cause is unknown. The definitive diagnosis is made with a skin biopsy that includes subcutaneous cellular tissue and a biopsy of muscle fascia. Below we present the clinical case of a 57-year-old female patient with extensive involvement con- sisting of edema, induration, and functional limitation with a refractory response to treatment. Case report A 57-year-old female patient with a clinical picture of a month and a half of evolution consisting of induration of the skin in the proximal region of the extremities, abdomen, and neck, associated with pain on palpation, which progresses to func- tional limitation. Additionally, the patient presented hair loss, arthralgias. and edema in the lower limbs. As relevant patho- logical antecedents, the patient had a diagnosis of hereditary Figure 1 – Marked induration in lower hemiabdomen and livedo reticularis spherocytosis which required splenectomy at 17 years of age, in thighs. being in remission since then. Physical examination showed livedo reticularis in the lower limbs (Fig. 1), as well as induration, which affected the inner side of the thighs, lower hemiabdomen, and upper limbs, being the right upper limb the site where it appeared with the highest intensity with “orange peel skin” changes (Fig. 2) and a longitudinal depression in the ventral surface (Fig. 3) but without affecting the region of the skin distal to the wrist. In view of the generalized commitment and the suspi- cion of a systemic disease, the patient was hospitalized. Among the laboratory tests performed, the blood count at 3 admission showed a leukocytosis of 17 430 leukocytes/mm at the expense of eosinophils with an absolute value of 4183 (25.8%), which increased until reaching an absolute value of 3 5670 eosinophils/mm (32.8%) (Fig. 4). In addition, the patient Figure 2 – Induration and “orange peel” changes are had an erythrocyte sedimentation rate of 18 mm/h, antin- observed in the right upper limb. uclear antibodies by indirect immunofluorescence in HEP 2 cells of 1:320 homogeneous pattern, and negative extractable nuclear antigens (Ro, La, Sm, RNP) by ELISA technique. The imaging studies included an abdominal tomography which the patient underwent an initial skin biopsy that included reported hepatomegaly, as well as a tomography of the chest epidermis and dermis without alterations. Subsequently, a and a Doppler ultrasound exam, both normal. To better char- deeper biopsy was performed, in this occasion with represen- acterize the compromise, it was requested an MRI of the tation of subcutaneous cellular tissue and muscular fascia, right forearm, which reported soft tissue edema affecting which demonstrated thickening of the fascia, fibrin deposi- the entire limb and thickening of the fascia with increased tion, and inflammatory infiltrate composed of lymphocytes uptake of the contrast agent at this level. Given these findings, and eosinophils (Fig. 5) establishing the diagnosis of EF. Document downloaded from http://www.elsevier.es, day 26/02/2019. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. r e v c o l o m b r e u m a t o l . 2 0 1 8;2 5(1):63–68 65 methylprednisolone 500 mg/day for 3 days and later continu- ing with prednisolone 50 mg/day for 1 month with subsequent reduction, accompanied by monthly intravenous cyclophos- phamide at doses of 750 mg to 1 g monthly, for a cumulative dose of 5 g and, after this, management was continued with mycophenolate at a dose of 2 g/day. At this moment, the patient is in remission of the disease with significant improve- ment of the functional limitation and reduction of the skin hardening after 2 years of follow-up. Discussion Figure 3 – Longitudinal depression known as groove sign in the distal region of the right upper limb. EF is a rare disease of the connective tissue, the first two reports were made in 1974 by Shulman, who described a syn- Eosinophil percentage drome characterized by skin changes, similar to those found in 35 systemic sclerosis (SS), consisting of induration of the extrem- ities associated with an increase in the number of eosinophils, 30 which he named Shulman syndrome; since then, more than 1,4 25 300 cases have been reported so far. This disease affects both sexes equally, between 40 and 50 20 years of age, predominantly in Caucasian people; even it can 2 affect children, but this is considered to be exceptional. 15 Although its cause is unknown, there are triggering fac- 10 tors which are identified in 30–40% of patients. Among these triggers, we can find a severe trauma, extreme 5 3 1 exercise, splenectomy, as well as the use of some drugs 5 6 7 such as statins, phenytoin, chemotherapeutic agents, and 0 infliximab.8 Admission Control In addition, various infectious agents have been related, 9 Eosinophil percentage from intestinal parasitosis going through bacterial infections 10,11 with Mycoplasma arginini and Borrelia burgdorferi. Another group of diseases with which EF has been Figure 4 – Evidence of increase in eosinophils. associated are hematological alterations which can be found in up to 10% of patients, such as paroxysmal 12 13 nocturnal hemoglobinuria, aplastic anemia, and 14 15,16 agammaglobulinemia, as well as leukemias and 17–22 lymphomas. EF has also been described after bone mar- 23,24 row transplantation, being the graft-versus-host disease its main differential diagnosis. The pathophysiology is unknown but it is believed that the increase in eosinophils generates an increase in the trans- forming growth factor ␤ that will stimulate the fibroblasts to produce a greater amount of collagen; associated with this it is known that patients with EF have increased levels of metallo- proteinase inhibitor protein type 1 (TIMP-1), which inhibits the degradation of collagen leading to fibrosis of the tissues, sec- 1,25 ondary to an increased immune response. Although there is only one study in this regard, in the cytokines profile there seems to be an increase in the levels of IL-5 and transforming Figure 5 – Thickening of the fascia, fibrin deposit, and growth factor ␤ (TGF-␤), which was documented in a 3-year- inflammatory infiltrate composed of lymphocytes and old child with EF, and reversed with the clinical improvement 26 eosinophils.