|IMAGES IN RHEUMATOLOGY
|Year : 2018 | Volume
| Issue : 4 | Page : 284-286
Juvenile eosinophilic fasciitis presenting with polyarthritis and koebner's phenomenon
Zakiya Saleh Al Mosawi1, Muneera Jassim Al Hammadi2, Batool Abdul Karim Ebrahim1, Sara Mathew George3
1 Department of Pediatric, Salmaniya Medical Complex, Manama, Bahrain
2 Department of Dermatology, Salmaniya Medical Complex, Manama, Bahrain
3 Department of Pathology, Salmaniya Medical Complex, Manama, Bahrain
|Date of Web Publication||18-Nov-2018|
Dr. Zakiya Saleh Al Mosawi
Department of Pediatric, Salmaniya Medical Center, P. O. Box: 12, Manama
Source of Support: None, Conflict of Interest: None
Keywords: Children, eosinophilic fasciitis, Koebner's phenomenon, polyarthritis
|How to cite this article:|
Al Mosawi ZS, Al Hammadi MJ, Karim Ebrahim BA, George SM. Juvenile eosinophilic fasciitis presenting with polyarthritis and koebner's phenomenon. Indian J Rheumatol 2018;13:284-6
|How to cite this URL:|
Al Mosawi ZS, Al Hammadi MJ, Karim Ebrahim BA, George SM. Juvenile eosinophilic fasciitis presenting with polyarthritis and koebner's phenomenon. Indian J Rheumatol [serial online] 2018 [cited 2020 Jan 24];13:284-6. Available from: http://www.indianjrheumatol.com/text.asp?2018/13/4/284/235733
A 5-year-old Arab girl presented to our outpatient clinic with a history of the inability to bear weight for 3 months, accompanied with swollen hands and feet, along with morning stiffness which usually lasted for 2–3 h daily. Pink skin rashes were seen on the face and abdomen. There was no history of fever, redness of eye, oral ulcers, hair loss, or weight loss. Physical examination of the skin revealed bilateral nonpitting edema involving the dorsum of hands, proximal and distal phalanges, extending to mid-forearms with tethering of skin to the underlying tissue. There was difficulty in flexion and extension of her fingers, as well as an inability to adopt a praying position [Figure 1]a. Koebner's phenomenon (linear erythema in the arms after applying blood pressure cuff) was positive [Figure 1]b. Similar findings were noted on the feet, with nonpitting edema and taut skin which involved the metatarsophalangeal joints and ankles. Musculoskeletal examination (MSK) showed contracted knuckles, warm wrists with tenderness, and restricted range of movement (ROM). There was also tenderness of both tibialis and quadriceps muscles, both hip and knee joints appeared contracted with restricted movements, and she had toe-walking gait. On further MSK examination, there was remarkable tenderness over the cervical and thoracolumbar spine with significant restriction in the ROM.
|Figure 1: Patient's clinical features – (a) A child with contracted knuckles edematous skin, swollen MCPs, proximal phalanges and wrists, (b) Koebner's phenomenon, (c) improvement in edema and knuckle contractures and positive prayer sign|
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Laboratory investigations showed a hemoglobin of 10.9 g/l, white blood cell (WBC) count of 8.9 109/l, polymorphs were 32%, lymphocytes were 42%, monocytes were 4.6%, eosinophils were 20.1%, total eosinophil count of 1798 cu/mm, and platelets were 235 109/l. Investigations also showed an erythrocyte sedimentation rate of 5 mm/h, C-reactive protein of 0.7 mg/L (N:<3), and creatinine kinase (CK) of 21 u/l (N: 21–215). The serum protein electrophoresis was normal, and liver function tests, urea and electrolytes, creatinine, calcium, magnesium, and phosphorus were within normal limits as well. Urine analysis and protein/creatinine ratio were normal. Rheumatology screening resulted in antinuclear antibody, rheumatoid factor, anti-cyclic citrullinated peptide, antineutrophil cytoplasmic antibody, and Extractable nuclear antigen (ENA) antibody were all negative and C3 and C4 were normal. Plain X-rays of hands and knees showed soft-tissue swellings and normal bones.
A full-thickness skin biopsy was performed which showed collagenization of the subcutaneous fat dividing it into lobules with broad collagen bands in between [Figure 2]a. In addition, there was diffuse chronic inflammatory cell infiltrate composed of mainly lymphocytes, plasma cells, and few eosinophils in the thick fascia [Figure 2]b.
|Figure 2: (a) Scanner view of the skin biopsy shows collagenization of the subcutaneous fat dividing it into lobules with broad collagen bands between (H and E, ×40). (b) High-power view shows the diffuse chronic inflammatory cell infiltrate composed of mainly lymphocytes, plasma cells, and few eosinophils in the thick fascia (H and E, ×400). (c) Scanner view of the muscle biopsy showing inflammatory cell infiltrates in the fascia, fat, and in the skeletal muscle bundles (H and E, ×40)|
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Due to significant muscular pain, a muscle biopsy was carried out from the flexor carpi ulnaris which revealed chronic inflammatory cell infiltrates in the fascia, fat, and in-between the skeletal muscle bundles [Figure 2]c.
The patient was started on pulses of methylprednisolone, 10 mg/kg/day was given for 3 days, along with occupational therapy sessions. Within 48 h, both the edema and skin thickening improved significantly. With the treatment, morning stiffness and joint pain regressed dramatically. At this phase of her course of management, a repeat complete blood count (CBC) showed WBC of 10.5 109/l with a differential count of polymorphs: 41%, lymphocytes: 49%, eosinophils: 1.6%, monocytes: 7.7%. The CBC revealed marked reduction in the absolute eosinophil count to 168 cu/mm and improvement in hemoglobin to 11.2 g/dl. Her symptoms and overall condition continued to improve while she was on prednisolone 1.2 mg/kg/day divided 12 hourly. After 6 months of administration, the prednisolone dose was tapered gradually. Subsequently, she was on a low dose of prednisolone for 9 months. Our patient had great clinical improvement over the course of treatment, with soft skin and no joint contractures, but with residual widening of proximal phalanges [Figure 1]c.
| Discussion|| |
Eosinophilic fasciitis (EF) is a rare connective tissue disorder, which was first reported by Lawrence Shulman in 1974. EF is characterized by thickening of the muscular fascia and subcutaneous tissue, with variable infiltration by eosinophils. Hypergammaglobulinemia and peripheral eosinophilia can be seen as well. The incidence and prevalence of this rare disease is unknown, with only approximately 32 cases of juvenile EF published up to 2010.,
The pathophysiology of EF is obscure. Dysregulated immunity in EF has been proposed by detecting abnormal T-cells and increased cytokine interleukin 5 (IL-5) level in a patient with EF and eosinophilic cellulitis. IL-5 causes the peripheral eosinophilia.
The clinical presentation of EF mimics scleroderma but differs in skin lesions which have the tendency to involve the forearms, sparing fingers, and the absence of Raynaud's phenomenon. However, our case manifests hard skin which involves the fingers and toes, in addition to Koebner's phenomenon. Furthermore, polyarthritis was reported as an initial presentation in one female child with EF, whereas the case presented had polyarthritis at the same time of the manifestation of her other clinical symptoms, which differs from other reported cases in literature.
Remarkable eosinophilia in peripheral blood test and eosinophils deposited in fascia and muscle biopsies should confirm a diagnosis of EF. However, our patient had normal inflammatory markers, CK, and gamma globulins. Inconsistency of clinical and laboratory consequences could highlight the need to question the fundamental nature of EF and classify the disease as a distinct entity with different phenotype, which suggests the necessity to undertake genetic studies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's mother has given consent for her child's images and other clinical information to be reported in the journal. The patient's mother understands that name and initials of the child will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Shulman LE. Diffuse fasciitis with hypergammaglobulinemia and eosinophilia. A new syndrome? J Rheumatol 1974;1 Suppl 1:46.
Ortega-Loayza AG, Merritt BG, Groben PA, Morrell DS. Eosinophilic fasciitis in a female child. J Am Acad Dermatol 2008;58:S72-4.
Loupasakis K, Derk CT. Eosinophilic fasciitis in a pediatric patient. J Clin Rheumatol 2010;16:129-31.
French LE, Shapiro M, Junkins-Hopkins JM, Wolfe JT, Rook AH. Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: Increased production of interleukin 5 and inhibition by interferon Alfa. J Am Acad Dermatol 2003;49:1170-4.
Shulman LE. Diffuse fasciitis with eosinophilia: A new syndrome? Trans Assoc Am Physicians 1975;88:70-86.
Olson NY, Lindsley CB, Kepes JJ. Eosinophilic fasciitis presenting as inflammatory polyarthritis. Pediatrics 1986;78:512-4.
Limpers A, van Royen-Kerkhof A, van Roon JA, Radstake TR, Broen JC. Overlapping gene expression profiles indicative of antigen processing and the interferon pathway characterize inflammatory fibrotic skin diseases. Expert Rev Clin Immunol 2014;10:231-41.
[Figure 1], [Figure 2]