Assessment of Sclerodermoid Chronic Graft-versus-host Disease with Colour Doppler Ultrasound.

Sclerodermoid chronic graft-versus-host disease (scGVHD) is a severe complication of allogeneic haema-- topoietic stem cell transplantation. The aim of this study was to investigate the usefulness of high-frequency ultrasound of the skin in assessing the inflammatory patterns and prognosis of patients with scGVHD. A prospective study was carried out with patients who developed scGVHD in the period June 2016 to April 2018. Clinical and ultrasound examinations were performed on the first visit and at 6-month follow-up. A total of 24 patients were included in the study. A 6-month follow-up high-frequency ultrasound of the skin was performed on 20 of the 24 patients. Abnormal B-mode findings in high-frequency ultrasound of the skin consisted of hypoechogenic dermis, hypoechogenicity of septa and hyperechogenicity of lobules in hypodermis. No differences were observed in these basal parameters between treatment progressive/non-responding and inactive/responding scGVHD groups of patients. Basal Doppler showing increased vascular flow with a systolic peak ≥10 cm/s and a vascular resistance index ≥ 0.70 was observed only in those patients who developed progressive/non-responding scGVHD (62.5% vs 0% p = 0.006). In conclusion, Doppler ultrasound is a useful tool to assess the inflammatory activity and outcome of scGVHD. These findings could enhance patient management and help to guide treatment decisions.

[Scleromyxedema]. / Skleromyxödem.

Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy. The pathogenesis of scleromyxedema is unknown. The clinical course is chronic and progressive and can lead to marked morbidity or death. The skin findings consist of multiple waxy papules and indurated plaques. Progressive skin involvement can lead to decreased mobility of the mouth and joints. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, or in the kidneys. There are no approved or evidence-based treatment options available for scleromyxedema. High-dose immunoglobulins are considered the treatment of choice, followed by lenalidomide (or thalidomide) and systemic glucocorticosteroids, or in severe cases even autologous hematopoetic stem cell transplantation. Long-term maintenance treatment is usually required and close clinical follow-up is necessary as recurrence of scleromyxedema is common after withdrawal of an effective therapy.

A rare case of palmar congenital nevus with sclerodermoid reaction.

Palmar congenital nevus with sclerodermoid reaction has not been reported. It has the potential of deep extension following the fibrous bundle. The utilization of slow Mohs or frozen sections with immunohistochemistry staining was recommended.

Inflammatory cutaneous metastases originating from gastric cancer: A case report.

BACKGROUND: Cutaneous metastasis with gastric cancer (GC) origin is extremely rare and associated with poor prognosis. Nodular type is the most common type, while other forms are extremely rare. CASE SUMMARY: This study describes severe skin redness, swelling, pain, and fever in a 65-year-old man diagnosed with GC, whose left chest wall, left upper limb, and left back were mainly affected. Firstly, the patient was diagnosed with "lymphangitis" and treated to promote lymphatic return. However, the symptoms were constantly deteriorating, and skin thickening and scattered small nodules gradually appeared. Finally, the skin biopsy confirmed cutaneous metastases, and the patient died 7 d later. CONCLUSION: Our case highlights that cutaneous metastasis should be considered when skin lesions appear in patients with GC.

Evaluating differential expression of fibrosis-related genes and their correlation with blood vessel density in chronic cutaneous graft-versus-host disease.

BACKGROUND: Sclerodermoid graft-versus-host disease (GVHD) is the most severe form of chronic GVHD (cGVHD) and represents a considerable therapeutic challenge. Due to the scarcity of human studies on sclerodermoid cGVHD, the pathogenesis of this entity is not fully understood. OBJECTIVE: To identify the differential expression of fibrosis-related genes in skin lesions of human lichenoid and sclerodermoid cGVHD and to assess the expression of their corresponding proteins. METHODS: PCR array analysis was performed on RNA extracted from three skin biopsies of sclerodermoid cGVHD patients and three normal skin samples, for fibrosis-related gene expression profiles followed by evaluation of their corresponding protein expressions. The expressions of Tissue inhibitor of metalloproteinase 3 (TIMP3), matrix metalloproteinase 1 (MMP1), TIMP1, and TIMP2 were further studied by immunohistochemistry. Demographic, clinical and immunohistochemical parameters of the two cGVHD groups and the control group were compared. The Pearson correlation coefficient was used to assess the correlation between data among the study groups. RESULTS: We identified 44 upregulated and 14 downregulated genes in the skin samples of sclerodermoid cGVHD compared to the control group. TIMP3 was positive in 13/21 biopsies of cGVHD and in one biopsy of the control group. The average staining intensity was significantly higher in the cGVHD group compared to the control group. TIMP3 was expressed mainly in dermal blood vessels. cGVHD specimens with positive TIMP3 staining had a statistically significantly higher total microvascular area than the negative specimens. CONCLUSION: TIMP3 levels are increased in both subtypes of cGVHD and are associated with increased dermal vascularity.

Case report: Scleromyxedema associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins.

Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly documented. High-dose intravenous immunoglobulin (IVIG), used either alone or in conjunction with systemic steroids and/or thalidomide, has been suggested as a first-line treatment. We report the case of a 45-year-old woman diagnosed with scleromyxedema with paraproteinemia that initially did not respond to systemic steroids, retinoids, and thalidomide but greatly improvement in terms of systemic and cutaneous symptoms after treatment with IVIG.

Carcinoid Syndrome-Induced Scleroderma-Like Disease.

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.

Cutaneous Chronic Graft Versus Host Disease in a Symmetric Distribution.

Graft-versus-host disease (GVHD) is a common complication following patients who have undergone allogenic hematopoietic stem cell transplantation (allo-HSCT). While GVHD has been previously sub-categorized through a temporal relationship upon transplantation, revisions from the National Institutes of Health have modified the diagnosis criteria to be more involved with specific signs and symptoms. Chronic classifications of GVHD include non-sclerotic and sclerotic forms, and the sclerotic form can be further classified based on morphologies such as lichen-sclerosis-like, sclerodermoid or morphea-like plaques. Generalized morphea can have similar histopathological findings but in order to be diagnosed, certain diagnostic criteria must be met. Herein, we report a patient with linear and inflammatory morphea morphology of chronic GVHD, which presents symmetrically on both lower extremities.

New insights on scleromyxedema.

Scleromyxedema is a rare fibromucinous disorders, with several clinical and pathological overlaps with scleroderma and scleredema. Etiopathogenesis remains uncovered, and no explanation has been provided either for the origin of mucin deposition or for the paraprotein role. The disease does not show gender predilection and affects mainly middle-age adults. The course is unpredictable, and prognosis remains guarded for renal, cardiac, and neurologic complications, especially in the setting of dermato-neuro syndrome. A valuable recent progress is the consensus definition of diagnostic criteria and lines of treatment, which hold the promise to improve the early recognition and management of this rare condition worldwide. High-dose intravenous immunoglobulin has been suggested as the first-line treatment either alone or associated with systemic steroids and/or thalidomide. In very recalcitrant cases, adjunctive bortezomib and/or autologous stem cell transplant might be considered. Melphalan treatment was associated with very toxic side effects and actually is no longer recommended.

A Tale of H Syndrome with Typical Radiographic Findings.

H syndrome is a recently described autosomal recessive genodermatosis with cutaneous phenotypes of varying severity and multi-system involvement. Patients suffering from this disorder can be easily mistaken for sclerodermoid conditions. The radiological findings of H syndrome are typical but have been described only anecdotally. We present a case of a 29 year old male patient of H syndrome with typical radiological features.