Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.

Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue of unknown aetiology and poorly understood pathogenesis. We present the case of a 60-year-old man diagnosed with eosinophilic fasciitis with extensive cutaneous involvement and severe functional repercussion, which appeared weeks after suffering from pneumonia due to Legionella pneumophila. The patient did not experience any clinical response with high-dose corticosteroids, subcutaneous methotrexate, and intravenous immunoglobulins. Consequently, tocilizumab was initiated at 8 mg/Kg monthly achieving clinical response measured by a control MRI at the fifth dose. Response in terms of cutaneous thickness has been slower however favourable, therefore, more months of follow-up are necessary to assess the complete remission at skin level. EF treatment still constitutes a challenge, and experience with tocilizumab in the management of the disease is very limited. Through a systematic search of medical literature, we retrieved two cases describing EF treated with tocilizumab and several cases using another monoclonal antibody or Janus kinase inhibitor. We report the third case to our knowledge of the efficacy of tocilizumab in a refractory EF to corticosteroids and other immunosuppressive drugs.

Scleroderma is a rare complication of carcinoid syndrome and is usually encountered in the setting of a metastatic primary neuroendocrine tumour of the distal ileum. Associated endocardial fibrosis is a frequent finding and the condition carries a poor prognosis. We report a case of scleroderma occurring in a 72-year-old female with metastatic neuroendocrine carcinoma and associated pericardial fibrosis. The use of an alternative nomenclature such as \"scleroderma-like\" or \"sclerodermoid\" disease is proposed in order to emphasise its distinction from true idiopathic scleroderma, despite the histopathological similarities on skin biopsy.

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud\'s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.

BACKGROUND: Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis.
OBJECTIVE: There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis.
CONCLUSIONS: These are termed as \"scleroderma variants\" or \"scleroderma like disorders\". This review discusses the various \"scleroderma variants\" e.g. scleromyxedema, scleredema, nephrogenic systemic fibrosis, and eosinophilic fasciitis.

The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated with protein deposits. The lack of Raynaud\'s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies should question the diagnosis of scleroderma and trigger the search for a scleroderma-like disorder, for which treatment and prognosis differ. This article gives a review of these disorders and their main characteristics.