584 research outputs found
Scleromyxedema with an interstitial granulomatous-like pattern: A rare histologic variant mimicking granuloma annulare
No full textScleromyxedema is the generalized and sclerodermoid form of lichen myxedematosus. Its typical histological features include a diffuse deposition of mucin in the papillary and mid reticular dermis, an increased of collagen deposition, and a proliferation of irregularly arranged fibroblasts. We describe a 76-year-old man presenting with scleromyxedema associated with IgGλ monoclonal gammopathy whose biopsy showed histological features of an interstitial granulomatous-like process consistent with interstitial granuloma annulare. The significance of these unusual granulomatous findings in the setting of scleromyxedema are unknown and have been described only once in the literature. This observation expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition. Rongioletti F, Cozzani E, Parodi A. Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare
Scleromyxedema: A potentially disabling and fatal disease
No full textScleromyxedema (SCL), considered to be the generalized and sclerodermoid subtype of lichen myxedematosus (papular mucinosis), is a disease with a chronic, progressive, and potentially disabling course. It is usually, although not invariably, associated with a monoclonal gammopathy which rarely leads to multiple myeloma and with many systemic manifestations. The therapy is difficult and the prognosis is guarded. The author, on the basis of literature and personal experience, review the anatomo-clinical manifestations of this uncommon disease
New and emerging conditions of acquired cutaneous mucinoses in adults
No full textCutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti–colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement
Frequency, risk factors and prognosis of systemic haematologic malignancies, cutaneous and other neoplasms in lymphomatoid papulosis: where are we now?
No full textActinic keratoses: what classification is useful to predict the risk of progression? PROs and cons
No full textLinked article: L. Schmitz et al. J Eur Acad Dermatol Venereol 2019; 33: 1092-1097.
Colloid Milium
No full textColloid milium is a rare, degenerative skin condition characterized by deposition of amorphous hyaline-like material in the dermis. Colloid milium and colloid degeneration include at least four distinct clinicopathological conditions: classic adult-type colloid milium, juvenile colloid milium, nodular-colloid degeneration (paracolloid), and pigmented colloid milium (hydroquinone related). There are usually no systemic implications, with the exception of a rare ligneous conjunctivitis or periodontitis in the juvenile type. Special stains, immunohistochemical studies, and electron microscopy have allowed the distinction from similar conditions such as amyloidosis. © 2010 Springer Science+Business Media, LLC
Exogenous Cutaneous Deposits with Special Consideration to Skin Reactions to Soft-Tissue Fillers
No full textAny nonliving material introduced into the skin and-resistant to degradation may trigger an inflammatory response. The origin and way of introduction goes from traumatic (accidental or self-induced) to cosmetic and surgi-cal-procedures to topical application of drugs and products. The increased use of exogenous injectable aesthetic microimplants (soft-tissue fillers) is paralleled by an increase in adverse cutaneous reactions. The most common clinical presentation of granulomatous reactions includes papules, nodules and stiff infiltration of the skin with or without ulceration. Histopathology is a good means to identify the type of exogenous agent, particularly of filler particles. © 2010 Springer Science+Business Media, LLC
Lipoid Proteinosis
No full textLipoid proteinosis is rare autosomal recessive disorder starting in early infancy and characterized by deposition of hyaline material in the skin, mucous membranes, and multiple organs. Typical clinical symptoms are hoarseness, vesicles-leaving pitted scars, beaded papules on the eyelid margins, diffuse thickening of the skin with verrucous change on frictional areas, and infiltration of the oral mucosa. Mutations within the extracellular matrix protein gene (ECM-1) are the underlying defect. © 2010 Springer Science+Business Media, LLC
Glucagonoma Syndrome and Necrolytic Migratory Erythema
No full textNecrolytic migratory erythema is a cutaneous paraneoplastic manifestation, which is usually associated with a glucagon-secreting pancreatic tumor. Clues to the diagnosis are the anatomic distribution (perioral, acral, and genital), the waxing and waning course, the figurate migratory lesions with advancing scaling-borders, and the distinctive histopathologic pattern with pale, vacuolated keratinocytes in the upper epidermis. Necrolytic migratory erythema heals once the glucagonoma has been surgically removed, but in 50% of these cases metastasis exists at the moment of the diagnosis. © 2010 Springer Science+Business Media, LLC
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