7-year-old girl with swollen, thickened, indurated skin on the right wrist, inability to clench fist and flex elbow for 3 months. No fever or other systemic features.
Vitals were stable. Subtle hyperpigmentation, non-pitting oedema and warm right upper and lower limb.
Clinically, differentials were morphea and lymphoedema.
Laboratory investigations revealed raised absolute eosinophilic count (>1500/cumm) with normal TLC. Mildly elevated ESR (43mm).
MRI of both forearms and screening MRI scans of both arms and lower limbs were performed.
MRI of both forearms revealed right-sided involvement more than left side with marked superficial as well as deep fascial and intermuscular septal thickening and oedema involving both the anterior and posterior compartment. There was also mild adjacent muscle oedema in the dorsal compartment (Fig. 1a, b). No evidence of fatty infiltration in the muscles (Fig. 1c).
MRI of both lower limbs also showed significant right-sided and minimal left-sided fascial and deep intramuscular septal thickening and oedema in thighs as well as calves (Fig. 2a, b).
Right arm had mild fascial thickening and oedema with relative sparing of the left arm (Fig. 3a, b).
Background:
Eosinophilic fasciitis, a rare disease entity, is also referred to as Shulman’s syndrome (1974) [1, 2]. The disease causes inflammation of the fascia, predominantly involving the distal peripheral extremities. The aetiopathogenesis is not clear [1].
Clinical Perspective:
Eosinophilic fasciitis clinically presents with skin manifestations, which may mimic scleroderma [2]. Classically, it is acute in onset and presents with cutaneous oedema and induration. It predominantly affects the distal parts of the extremities and is usually symmetric [1]. Laboratory findings include hypergammaglobulinaemia, elevated erythrocyte sedimentation rate and peripheral eosinophilia (raised absolute eosinophilic count) [2]. Imaging helps in confirmation of clinical diagnosis, guidance for optimal location for biopsy and evaluation of response to therapy. The triad of clinical features, peripheral eosinophilia and hypergammaglobulinaemia are diagnostic. Biopsy is not pathognomonic but suggestive and can be avoided.
Imaging perspective:
MRI can help identify superficial, deep fascia and intermuscular septa separate from the muscle belly. The oedema centred around the former would suggest fasciitis and the latter myositis. The oedema from one can extend to involve the other, however, the epicentre is important to identify.
In eosinophilic fasciitis, predominant fascial oedema and thickening is best appreciated on fluid sensitive sequences, oedema can extend into adjacent hypodermic fat and muscle [1, 2]. Fascial enhancement can also be seen on post-gadolinium acquired images.
Myositis [1] (related to polymyositis, dermatomyositis and inclusion body myositis) is a close differential with predominant edema in the muscles and relative sparing of the fascia. Other differentials include stasis edema, cutaneous scleroderma in which the edema and involvement is usually limited to the hypodermic soft tissue [1]. Necrotising fasciitis is commonly associated with abscess and air foci may be seen [1].
Biopsy can help in further confirmation [2], however, is not pathognomonic but suggestive of diagnosis. Clinical and laboratory investigations with radiological correlation can confirm diagnosis and avoid a biopsy.
Outcome:
The treatment is medical with administration of steroids and methotrexate. The prognosis is better compared to morphea and scleroderma.
Take-home message, teaching points:
If imaging reveals predominant fascial involvement (rather than muscle) affecting the extremities, the suspicion for eosinophilic fasciitis should be raised. Further correlation with clinical presentation and laboratory investigations should be recommended to confirm the diagnosis.
The triad of clinical features, peripheral eosinophilia and hypergammaglobulinaemia are diagnostic. Radiological findings support the diagnosis and may also prompt the clinician to think about it retrospectively if not thought of prospectively as it is a rare entity, and thus avoid biopsy.
Eosinophilic fasciitis
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Based on the provided MRI images, the child’s right forearm (and the bilateral forearms for comparison) shows significant thickening and edematous signals in the fascial layer, especially in the superficial and deep fascia, as well as in the intermuscular septa. The muscle tissue itself appears largely normal or only mildly involved. On T2-weighted images, the fascia displays high signal intensity indicative of edema; contrast enhancement shows varying degrees of fascial enhancement. No obvious abscess, gas formation, or significant bone destruction or bone marrow lesions are noted. Compared to the adjacent soft tissues, the swelling and thickening of the fascia are particularly prominent.
The child presents with skin thickening, stiffness, and a firm, woody feel on palpation of the limbs (especially the right side), with a marked elevation of eosinophils in peripheral blood (>1500/μL). Imaging reveals swollen and thickened fascia, consistent with the classic features of eosinophilic fasciitis.
Initially, localized scleroderma (Morphea) or other scleroderma subtypes may be considered. However, imaging often shows predominant subcutaneous thickening rather than fascial involvement, and peripheral eosinophilia is not common in scleroderma.
In cases that primarily involve the muscle belly (muscle tissue) with symmetrical muscle edema, polymyositis or dermatomyositis would be more likely. However, in this case, the imaging findings center on fascial involvement, with only mild spread to the muscle itself, making this diagnosis less likely.
Imaging typically shows subcutaneous edema and thickening, with less pronounced inflammatory changes in the fascia. Clinically, these conditions usually develop slowly and lack significant peripheral eosinophilia.
Usually accompanied by gas formation, significant soft tissue necrosis, and signs of severe systemic infection, which are not consistent with the clinical scenario here.
Taking into account the child's clinical features (skin swelling, hardening, and restricted joint motion), blood test results showing significant eosinophilia, mildly elevated ESR, and MRI findings of fascial-centered edema and thickening, the most likely diagnosis is:
Eosinophilic Fasciitis (also known as Shulman’s syndrome).
This condition is relatively rare. If clinical and imaging findings are classic, biopsy may be partially avoided. If uncertainty remains, a pathological biopsy can be conducted for further confirmation.
Management focuses on both medical therapy and functional rehabilitation.
Under the guidance of physicians and rehabilitation therapists, a planned regimen of joint movement and muscle strengthening exercises is recommended, following the FITT-VP principle (Frequency, Intensity, Time, Type, Volume-Progression):
Example: 5–10 minutes of hand and elbow range-of-motion exercises (stretching, gentle opening and closing of the fist) every morning and evening. Gradually add exercises such as squeezing a stress ball to improve grip strength and flexibility.
Avoid excessive fatigue or high-intensity exercises to prevent triggering new inflammation or causing local injury.
This report is a preliminary analysis based on the current examinations and information, and is for reference purposes only. It cannot replace a hospital diagnosis or professional face-to-face evaluation by a physician. If you have any questions, please seek consultation at a qualified medical institution, and proceed with further examinations and treatment under the guidance of a specialist.
Eosinophilic fasciitis
