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In the past years seven new cutaneous mucinoses have been described. These are briefly reviewed and included in a revised classification of cutaneous mucinoses.
Cutaneous mucinoses are a heterogeneous group of disorders in which mucin accumulates in the skin or in the follicles. Mucin is a gelatinous substance composed of glycosaminoglycanes, especially hyaluronic acid and dermatan sulfate bound to small quantities of chondoitin sulfate and heparin sulfate. Though the causes of mucinoses remain unknown, they can be divided into distinctive cutaneous (primary) mucinoses, in which mucin deposition is the distinctive histological sign resulting in clinically distinctive lesions, and cutaneous disorders, in which mucin deposition is an epiphenomenon (secondary mucinoses). Histologically, mucin is recognized after special staining techniques using alcian blue and colloidal iron. The microscopic localization of the mucin deposit is used to distinguish between dermal and follicular forms of primary mucinoses and between epidermal, dermal and follicular forms of secondary mucinoses. We present here the clinical and histological features of primary cutaneous mucinoses and an updated classification. The main therapeutic schemas are outlined.
The cutaneous mucinoses are a group of connective tissue disorders characterized by the deposition of mucin, either focally or diffusely, in the interstices of the dermis. The diseases may be a primary (metabolic) or secondary (catabolic) process. Systemic abnormalities are seen with most of these disorders. This review discusses the primary mucinoses in which the predominant dermal mucin is hyaluronic acid. Current therapy and proposed mechanisms for the mucinoses are considered.
Glycosaminoglycan (GAG) infiltration of the skin is a feature of hyperthyroidism, hypothyroidism, pretibial myxedema, scleromyxedema, and scleredema. We investigated the pathogenesis of the GAG deposits using light microscopy, histochemical digestion with a series of GAG-specific enzymes, and electron microscopy. Hyaluronic acid was the main GAG in all the conditions and in normal skin. Furthermore, there was minimal histologic variability of GAG dermal distribution. A striking distinguishing feature involved dermal fibroblast activity, which appeared normal or inactive in thyroid disorders and hypertrophic and/or hyperplastic in scleromyxedema and scleredema. Thus, the acquired cutaneous mucinoses exhibit similar skin GAG distribution and biochemical composition. The morphologic differences in fibroblastic activity suggest that the mucinoses of scleredema and scleromyxedema represent a local process, whereas the GAG infiltration of thyroid diseases may have a systemic origin.
In the last few years cutaneous mucinoses have been reported with increased frequency in HIV patients. We report the occurrence of scleredema, reticular erythematous mucinosis and lichen myxoedematosus in three different HIV-infected patients, review the literature and discuss the possible relationship between mucin deposits and HIV infection. This is the first report of scleredema and the second of reticular erythematous mucinosis in an HIV-infected patient. Only the association of HIV infection with lichen myxoedematosus seems to be more than coincidental.
Seven dogs had one or more asymptomatic nodules, papules, or plaques on the skin or oral mucosa. The primary histologic feature was the accumulation of excess mucin within the dermis or submucosa. Based upon the clinical presentation and the histopathologic changes, it was proposed that these lesions represent the canine analogue of focal mucinosis in man, and that the same name be applied to the lesion in dogs. The criteria for the diagnosis of focal mucinosis were: (1) the presence of a single (rarely multiple) papule, nodule, or plaque which may be firm, rubbery, or soft, (2) the accumulation of mucin which disrupts and separates collagen fibers, (3) mild to extensive fibroblast proliferation, and (4) a mild mononuclear cell infiltration. The mucinoses of man and animals were reviewed.
A 54-year-old woman with myxedema had hundreds of discrete cutaneous mucinous papules, which responded dramatically to appropriate replacement therapy with L-thyroxine. Each lesion both clinically and histologically resembled a cutaneous focal mucinosis (cutaneous myxoma). Multiple cutaneous focal mucinoses are a previously undescribed manifestation of hypothyroidism that can be differentiated from other syndromes that have cutaneous mucinous deposits.
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The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin or within the hair follicle. We divide the cutaneous mucinoses into two groups: the distinctive cutaneous mucinoses in which the mucin deposit is a distinctive histopathologic feature that manifests as a clinically specific lesion, and the diseases associated with histopathologic mucin deposition as an additional finding. This article deals with the clinical and histopathologic features and the treatment of the distinctive cutaneous mucinoses and updates their classification. They may be divided, according to the microscopic location of mucin, into dermal and follicular mucinoses. The former group includes; lichen myxedematosus, acral persistent papular mucinosis, reticular erythematous mucinosis, scleredema, dysthyroidotic mucinoses (i.e. localized myxedema, generalized myxedema, papular mucinoses associated with thyroid diseases), papular and nodular mucinosis associated with lupus erythematosus, self-healing juvenile cutaneous mucinosis, cutaneous mucinosis of the infancy, cutaneous toxic mucinoses (papular mucinosis of the toxic oil syndrome and of eosinophiliamyalgia syndrome), neuropathia mucinosa cutanea, cutaneous focal mucinosis, mucous cyst (digital and of the oral mucosa), while the latter group includes Pinkus' follicular mucinosis and urticaria-like follicular mucinosis.
Cutaneous mucinoses are a heterogeneous group of diseases characterized by the focal or diffuse dermal deposition of glycosaminoglycans. The histopathologic examination of many cutaneous mucinoses reveals that the collagen fibers are fragmented. We wanted to characterize the type I (COL1) and type III (COL3) collagen distribution in skin biopsy specimens of patients with cutaneous mucinosis. The diagnosis of mucinosis was based on a modification of the classification by Rongioletti and Rebora: four patients had familial papulonodular mucinosis: four had papular mucinosis, one of which was associated with myxedema and one had scleromyxedema; and one had focal mucinosis. We performed anti-type I and type III collagens immunolabeling on frozen sections. Immunofluorescence for COL1 was increased in the superficial dermis of 2/4 familial papulonodular mucinosis, in 5/5 of papular mucinosis, and in scleromyxedema and focal mucinosis cases. The mid-dermis showed intense staining for COL1 at the periphery of collagen bundles and, in three cases of familial papulonodular mucinosis and two cases of papular mucinosis, a lacy appearance. The superficial dermis of familial papulonodular mucinosis specimens and of papular mucinosis + myxedema, scleromyxedema, and focal mucinosis specimens had decreased COL3 staining. The mid-dermis showed a more prominent fibrillar staining at the periphery of the collagen bundles, and two cases of papular mucinosis showed intense labeling for COL3. Both COL1 and COL3 distributions are altered in cutaneous mucinosis. An intense labeling with COL1 is predominantly found in the superficial layer of cutaneous mucinosis. Cases of FTP revealed decreased COL3 reactivity at the superficial layer.
Cutaneous mucinosis is a term that has been used to describe a group of diseases or conditions in which accumulation of mucin in the skin is a prominent feature. The cutaneous mucinoses includes myxedema (both diffuse and localized), lichen myxedematosus (papular mucinosis), lipoid proteinosis, follicular mucinosis, cutaneous focal mucinosis, cutaneous myxoid cyst, and others. All of these diseases share distinct histologic features. I examined a 16-month-old infant with a case of cutaneous mucinosis that had unique clinical and histologic features, unlike any of the known mucinoses.
Childhood cutaneous mucinoses have been rarely reported and are difficult to classify. We describe two brothers who developed multiple, extensive cutaneous lesions during the first few months of life. Histologically the lesions were composed of mucin deposits in the dermis. In the first patient, the lesions spontaneously disappeared over the years. We believe that familial self-healing cutaneous mucinosis represents a unique entity not previously reported.