024 Thick and fast: two cases of eosinophilic fasciitis

Abstract

Background: Eosinophilic fasciitis (EF) is a rare inflammatory condition, characterised by skin thickening and induration. Data are limited to case reports and small case series in contrast to the more extensively studied scleroderma. We describe two cases that presented within two weeks. Methods: 57-year-old South-Asian male presented with a threemonth history of weight loss, leg oedema and pruritus affecting the legs and forearms. Bloods showed raised inflammatory markers, significant eosinophilia and raised IgG. Extensive investigation by the haematology and infectious diseases teams, including PET-CT, revealed no cause. 55-year-old Caucasian male presented with a one-week history of worsening swelling in his right forearm and hand. This was preceded by a similar episode on the contralateral arm which resolved spontaneously two weeks prior. He was initially started on coamoxiclav for presumed cellulitis. Bloods showed raised inflammatory markers and significant eosinophilia. CT chest/abdomen/pelvis was unremarkable. Results: MRI of the arms and legs showed widespread fascial thickening and oedema affecting all muscle compartments and intermuscular fascial planes, in keeping with fasciitis. Full thickness skin biopsy revealed marked thickening, fibrosis and eosinophilic hyalinsation of the collagen in the superficial fascia and deep dermis alongside chronic inflammatory cell infiltration of plasma cells, lymphocytes and eosinophils. Upper limb MRI showed pronator quadratus and flexor pollicus longus oedema suggesting a neuropathy of the anterior interosseus nerve. Nerve conduction of the arms showed patchy motor and sensory axonal loss in the left median nerve with mild denervation in the forearm muscles and a severe left median lesion around the carpal tunnel. Skin biopsy results are pending. Both patients responded rapidly to corticosteroids with normalisation of eosinophil count, reduction in swelling and resolution of pruritus. Conclusion: The hallmarks of EF including induration and thickening of the skin (usually the limbs), eosinophilia, hypergammaglobulinaemia and a raised erythrocyte sedimentation rate were seen in both cases. Our second patient presented with an uncommon form of EF with a spontaneously resolving and relapsing episode as well as carpal tunnel syndrome. Additionally, the muscle oedema adjacent to the fascia noted on his MRI is a recognised feature of EF. There are no accepted international diagnostic criteria for EF. Criteria for inclusion in case series often involve clinical features and inflammatory cell infiltrate in the hypodermis on full thickness skin biopsy. It is suggested that where biopsy is not possible, a diagnosis can be made based on clinical findings and radiological (ultrasound or MRI) evidence of fasciitis. There are no randomised controlled trials comparing treatment but success with steroids and steroid-sparing agents are reported. In these two cases, we correlate clinical images, blood tests, radiographic findings and histopathological images with our diagnosis. This is of importance as the differentials, including scerloderma and infection, are not steroid responsive.