EF, or diffuse fasciitis with eosinophilia, was first described by Shulman3 in 1974. EF is a rare connective tissue disease with characteristics different from systemic sclerosis (SSc). Skin hardening is not only common in EF, a variant of scleroderma, but also the hallmark of SSc and a reason why conditions are often confused.1 It is important to distinguish between EF and SSc because treatment and prognosis differ. Since tissue fibrosis causes progressive organ damage and contributes to mortality due to SSc, drugs that block the fibrotic process represent a rational therapeutic approach. D-penicillamine has been widely used as an antifibrotic agent. In retrospective studies, d-penicillamine stabilized and improved skin hardening, prevented further involvement of internal organs, and improved SSc survival. However, in a randomised controlled clinical trial with early active SSc, there was no difference in the extent of skin involvement between patients treated with a standard dose (750 mg/day) or a very low dose (125 mg every other day) of d-penicillamine. Minocycline, bosentan, recombinant relaxin, interferon γ and tumor necrosis factor inhibitors are suspected antifibrotic agents that have shown no significant benefit in SSc clinical trials. Small molecule tyrosine kinase inhibitors successfully used to treat malignancies, such as imatinib, nilotinib and dasatinib, block TGF-β and PDGF signaling, thereby preventing fibrotic reactions in vitro and in vivo. These compounds are currently in clinical trials for the treatment of SSc. In cases not associated with diabetes, women outperform men by 2:1. The onset can be from childhood to adulthood. Tension and hardening of the skin begins in the neck and/or face and spreads symmetrically to include the arms, shoulders, back, and chest.
The distal extremities are spared. The patient may have difficulty opening their mouth or eyes and have a mask-like expression following infiltration. The affected skin, waxy and woody in consistency, gradually changes to normal skin without clear demarcation. Associated outcomes occur in a variable number of patients and may include dysphagia caused by involvement of the tongue and upper esophagus, cardiac arrhythmias and associated paraproteinemia, usually IgG. Myeloma may be present. There may be pleural, pericardial or peritoneal effusion. Subcutaneous interferon alpha sometimes causes local erythema and hardening of the skin, which can be avoided by regularly changing the injection site. Isolated reports have described more severe local reactions, with inflammatory painful nodules, purple papules, and vasculitis, local ulceration, and necrosis at the injection site [394–398]. Despite previous results, even patients receiving low doses of interferon alpha may have severe injection site reactions.
A localized intradermal bullous rash that recurred after each injection of interferon alfa has also been reported . At the time of the presentation, the patient was feverish. Physical examination was notable for mild wrist contractures without synovitis. Firm, hardened woody skin was observed on the upper and lower limbs on both sides, but spared by the fingers, chest, feet, back, face and neck. This classic find can be seen in the photo (left). What term is used to describe it? Skin hardening is a deep thickening of the skin that can result from edema, inflammation, or infiltration, including cancer. Treatment for skin hardening varies greatly depending on the underlying cause. Skin involvement in early SSc is inflammatory and can be controlled with systemic antihistamines or short-term low-dose corticosteroids. Due to the increased risk of scleroderma kidney attack, blood pressure should be monitored carefully.
Cyclophosphamide, methotrexate, d-penicillamine and mycophenolate were associated with modest improvement in skin hardening in the early stages of SSc. Dryness of the skin can be treated with hydrophilic ointments and emollient bath oils. Fingertip ulcerations should be protected with an occlusive bandage to promote healing and prevent infection. Infected skin ulcers are treated with topical or oral antibiotics and may require surgical debridement. No drug treatment has been shown to be effective in preventing or promoting soft tissue calcification, and surgical treatment is only occasionally effective. The main causes of skin hardening are: Britannica.com: Encyclopedia articles on hardening Studies have shown that hardening of the soft tissues of the feet can lead to an increased risk of recurrent foot ulcers in people with diabetes, as it leads to a decrease in the foot`s ability to absorb shock when a person carries weight. Scleroderma (systemic scleroderma) is a rare disease characterized by inflammation and fibrosis of the skin and internal organs. The disease has three phases and the second phase involves hardening of the skin. The disease is associated with significant morbidity and mortality rates. Different types of skin infections can manifest as symptoms of hardening of the skin.
Examples: Scledema is a skin disease characterized by stiffening and hardening of the subcutaneous tissue, as if infiltrated with paraffin. It comes in two forms: with and without diabetes mellitus. In more general non-diabetic diseases, a sudden onset may occur after infection, usually streptococci. This reactive variant can also present itself as a drug epidemic. In other cases, the onset is insidious and chronic and has no previous infection. In the most common disease associated with diabetes, a lasting hardening of the upper back is characteristic. This case shows a classic representation of a rare connective tissue disease. Patients with skin hardening, myalgia, arthralgia, and peripheral eosinophilia should be diagnosed with EF, and a full-thickness biopsy is essential to rule out other diagnoses. In the second group, which is the most common type in the experience of most dermatologists, there is an association with late insulin-dependent diabetes.
Men are 10:1 higher than women. Affected men tend to be obese. The lesions are gradual and long-lasting and present as woody hardening and thickening of the skin of the middle of the upper back, neck and shoulders (Fig. 9.7). There is a sharp step from the affected skin to normal skin. Persistent erythema and folliculitis can affect the affected areas. Associated diabetes is long-lasting and difficult to control. In addition, patients often experience complications from their diabetes, such as nephropathy, atherosclerotic diseases, retinopathy and neuropathy. Control of diabetes does not affect the course of scledema. No paraproteins are detected and no visceral involvement is observed.