[Scleromyxedema]. / Skleromyxödem.

Scleromyxedema or generalized diffuse lichen myxoedematosus is a rare mucinosis that is associated with monoclonal gammopathy and which frequently affects multiple extracutaneous organ systems. The pathogenesis of scleromyxedema has not been fully elucidated, but includes stimulation of glycosaminoglycan synthesis. The clinical course of scleromyxedema is chronic and often progressive, leading to severe morbidity and even death. The characteristic skin findings encompass multiple waxy papules often on indurated plaques, while thickening of skin leads to conspicuous folds on glabella and dorsal aspects of finger joints. Microscopical manifestations are dermal deposits of glycosaminoglycans between collagen bundles in reticular dermis, increased numbers of fibroblasts and fibrosis as well as loss of elastic fibers. Progressive skin involvement results in decreased mobility of the mouth and joints and even contractures. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, in the kidneys or in the central and peripheral nervous system. There are no in-label or evidence-based treatments available for scleromyxedema, but by expert consensus high-dose immunoglobulins are considered as treatment of choice, followed in case of insufficient efficacy by systemic glucocorticosteroids and then lenalidomide or thalidomide. In severe and refractory cases, autologous hematopoietic stem cell transplantation has been performed. Long-term maintenance treatment is usually required to prevent recurrences. Close interdisciplinary follow-up is recommended.

Papular Mucinosis.

This case report describes erythematous patches on the face that involved the nasolabial folds, as well as numerous skin-colored homogenous waxy papules on the arms, neck, and trunk.

Dermato-neuro syndrome after COVID-19 infection in a patient with scleromyxoedema: Previously successful treatment with intravenous immunoglobulins.

Scleromyxedema (SMX) is a rare disease of unknown cause. It is a chronic, progressive, metabolic disorder characterized by a generalized papular and scleroderma-like rash, as well as a subtype of lichen myxedematosus. Dermato-neuro syndrome (DNS) is a rare neurological complication of SMX. It has flu-like prodromal symptoms; consists of a triad of fever, coma, and seizures; and can be life-threatening. We describe a patient with SMX complicated by DNS after infection with COVID-19. Her symptoms resolved after treatment with acyclovir and low-dose glucocorticoids, suggesting that DNS seizures may have a viral cause. Her skin lesions also improved after seven courses of intravenous immunoglobulin treatment, confirming that intravenous immunoglobulin is effective in these cases.

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

ABSTRACT: Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis.

Scleromyxoedema is a rare mucinosis that primarily affects the skin. It is associated with monoclonal gammopathy and has many extracutaneous manifestations, however, renal involvement is rare. We report the case of a woman with monoclonal gammopathy and scleromyxoedema presenting with progressive exertional dyspnoea and acute renal failure. Workup of her renal failure revealed monoclonal gammopathy associated C3 glomerulonephritis. She was treated with intravenous steroids and discharged with plans to pursue annual monoclonal gammopathy laboratory monitoring. Given the rarity of renal scleromyxoedema, careful investigation of extracutaneous manifestations and comorbidities is critical to discern the primary pathological process in patients with scleromyxoedema who develop renal insufficiency.

Small lymphocytic lymphoma presenting as chronic diffuse lip swelling.

Although rare, small lymphocytic lymphoma can present as chronic lip swelling and papules, thus mimicking the features of orofacial granulomatosis, a chronic inflammatory disorder characterized by subepithelial noncaseating granulomas, or papular mucinosis, characterized by localized dermal mucin deposition of mucin. When assessing lip swelling, one must carefully consider the clinical clues and have a low threshold to perform a diagnostic tissue biopsy, preventing delays in treatment or progression of the lymphoma.

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.

Acral persistent papular mucinosis.

Acral persistent papular mucinosis (APPM) is a subtype of localized lichen myxedematosus (LM) characterized by the chronic development of white to skin-colored papules and limited to the extensor surfaces of the hands and distal part of forearms, in the absence of systemic or laboratory manifestations. There is a strong predominance in women. Histopathology shows focal accumulation of mucin in the upper portion of the dermis sparing the grenz zone. The etiology is unknown. It is a benign condition, although dynamic changes occur with the progressive development of additional lesions. No specific treatment is required, and patients should be reassured about the prognosis.