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PubMed1993

[Cutaneous mucinosis].

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.

Adolescent↗
PubMed2003

Focal mucinosis: clinical and histological features of an unusual condition.

Mucinoses constitute a diverse group of disorders that have in common the deposition of basophilic, finely granular and stringy material in the connective tissues of the dermis (known as dermal mucinoses); this also can occur in the hair follicles (follicular mucinoses) and finally in the epidermis (epithelial mucinoses). We present the clinical and histological findings of a case of focal mucinosis in a 76-year-old man who was otherwise fit and healthy. A literature review revealed a sparse number of such cases none of which was reported in the ENT literature. Focal cutaneous mucinosis is a benign condition but the authors believe it is of local and systemic interest to the Otolaryngologist as an addition to his or her armentarium of diagnoses.

Aged↗
PubMed1998

Type I and type III collagens in cutaneous 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.

Adolescent↗
PubMed1980

Cutaneous mucinosis of infancy.

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.

Diagnosis, Differential↗
PubMed2006

[Acral persistent papular mucinosis].

Cutaneous mucinoses, also called lichen myxoedematosus, are a group of diseases characterized by mucin deposit on the skin. They may be associated to systemic diseases (scleromyxedema) or be primary ones (papular mucinosis, localized lichen myxoedematous), although they share common findings (intermediate or atypical forms) in some cases. Acral persistent papular mucinosis (APPM) is a type of papular mucinosis that is located exclusively on the back of the hands and in the distal zone of the forearms and is not associated to any systemic disease. We present the case of a 52-year-old healthy woman who had skin lesions on the back of her hands and whose histological study confirmed an APPM.

Biopsy↗
PubMedDate not supplied

Cutaneous focal mucinosis: a case report.

Cutaneous or dermal mucinoses are a heterogeneous group of connective tissue disorders in which there is an accumulation of mucin in the skin or within the hair follicle. They have a number of different morphologic presentations and can often be confused with other diseases. We report a 12-year-old Chinese girl with a 1-year history of a well-defined, hypopigmented plaque on her chin, consistent with cutaneous focal mucinosis, a rare condition in children.

Child↗
PubMedDate not supplied

Self-healing infantile familial cutaneous mucinosis.

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.

Adolescent↗
PubMed1994

Self-healing localized cutaneous mucinosis.

A 54-year-old woman presented a lesion on the dorsum of the right hand. Routine laboratory tests were normal or negative. Histological examination revealed mucin deposits in the reticular dermis. The lesion cleared within 1 year without any treatment but biopsy. This case does not fit the diagnostic criteria of any known cutaneous mucinoses.

Female↗
PubMedDate not supplied

Reticular erythematous mucinosis (REM) with telangiectasias associated with essential thrombocytosis and lung carcinoma.

The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin. Reticular erythematous mucinosis (REM) is an infrequent variant. We present a 48-year-old man with essential thrombocytosis and REM lesions with atypical telangiectasias on his chest, who developed a non-small cell lung carcinoma. We discuss the unusual clinical finding of telangiectasias over REM lesions and the association with essential thrombocytosis and lung carcinoma.

Biopsy, Needle↗
PubMed1995

Self-healing juvenile cutaneous mucinosis.

BACKGROUND: Mucinoses represent a puzzling and heterogeneous group of rare diseases, and self-healing juvenile cutaneous mucinosis is an extremely rare disease among them. OBSERVATIONS: A scleroedematous condition of the face, associated with papular lesions and arthropathies, had occurred in a 5-year-old boy 10 days after onset of fever, arthralgia, muscle tenderness, and weakness. RESULTS: Histologic examination revealed an edematous dermis, occupied by mucin. Skin lesions and the joint swellings disappeared spontaneously after 2 months. At the follow-up 5 years later, the patient remains in excellent health. CONCLUSION: Although exceptional, this entity has a well-defined clinical picture, marked by manifestations that are initially worrisome but which, surprisingly, prove to be temporary and benign.

Child, Preschool↗
PubMed1994

Papulonodular dermal mucinosis in systemic lupus erythematosus.

A 38-year-old woman with systemic lupus erythematosus presented to the dermatology department with diffuse nodules and plaques on her face, trunk and extremities. A lesional biopsy revealed large dermal mucin deposits. Papulonodular dermal mucinosis is characterized histologically by diffuse dermal mucin without the classic epidermal or inflammatory changes seen in lupus erythematosus (LE). This rare variant of LE should be distinguished from other cutaneous mucinoses.

Adult↗
PubMed1995

[Quadri-chromic staining in routine cutaneous histopathology (hematoxylin, eosin, saffron and astra blue)].

INTRODUCTION: The routine histologic stains used in dermatopathology are usually hematoxylin-eosin-safran. In order to stain the mucins, specific dyes are necessary. The purpose of this study was to perform a routine quadrichromic stain which included Astra blue as specific dye of mucins. The applications of this hematoxylin-eosin-safran-Astra blue stain were evaluated after 5 years of systematic use. METHODS: The quadrichromic stain was realized by Varistain (Shandon). The time of coloration was 15 minutes for Astra blue pH 2.5, 10 minutes for hematoxylin, 45 seconds for eosin and 4 minutes for safran. A total of 56,000 samples were analyzed using the quadrichromic stain. RESULTS: When mucin was present, it was colored in blue and the contrast with the other dyes was enhanced. Astra blue stained specifically the mucin deposits and it did not modify the other colours. The mucin deposits present in primary and secondary cutaneous mucinoses were most often visible after quadrichromic stain. Because of the variability of the mucin composition, the Astra blue did not react with all mucins. This stain was particularly useful in extramammary Paget's disease and in atrophic malignant papulosis. CONCLUSION: This new stain was easy to perform and allowed a specific coloration of mucin deposits in many cases. The use of four colours therefore improves the results of a routine stain, with a high quality of coloration.

Coloring Agents↗
PubMed1996

[Persistent acral papulous mucinosis].

INTRODUCTION: Acral persistent papular mucinosis is an uncommon skin disease with characteristic mucin deposits in the medial layer of the derma. In the Rongioletti and Rebora classification, this disease belongs to the group of specific cutaneous mucinoses. CASE REPORT: A 14-year-old girl consulted for symmetrical papular lesions on the back of the hand and wrists. The first manifestations had occurred at the age of 3 years. Laboratory tests were positive for antinuclear antibodies at 1/320. The histology and the clinical presentation led to the diagnosis of acral persistent papular mucinosis. DISCUSSION: This case shows that manifestations of acral persistent papular mucinosis can occur early in childhood. This disease may be a single clinical entity or an attenuated clinical expression of papular mucinosis. Clinical and laboratory observation were required for our patient.

Adolescent↗
PubMed2002

[Scleredema adultorum in secondary hyperparathyroidism].

Scleredema adultorum, or Buschke's scleredema, belongs to the group of mucinoses. It is characterised by thickened and indurated skin. Histopathology shows thickened dermis with an infiltration of mucin between swollen collagen bundles. There are reports about many associations with scleredema adultorum, e.g., with diabetes mellitus and multiple myeloma. One case is known with associated primary hyperparathyroidism. For the first time we report a case of scleredema adultorum and secondary hyperparathyroidism, in a 46-year-old patient. Both forms of hyperparathyroidism have increased levels of parathormone. Therefore, these increased levels could have an influence on collagen metabolism.

Biopsy↗
PubMed1991

Papular mucinosis in L-tryptophan-induced eosinophilia-myalgia syndrome.

Five patients with the L-tryptophan-related eosinophilia-myalgia syndrome had a generalized eruption of flesh-colored papules. In all patients, histologic examination revealed a focal accumulation of mucin in the upper mid dermis, associated with increased dermal cellularity. The mucin was composed predominantly of hyaluronic acid, with small amounts of sulfated acid mucopolysaccharides. The cells within the lesion were fibroblasts. The lesions slowly regressed after L-tryptophan was discontinued. Proposed explanations for the L-tryptophan-related eosinophilia-myalgia syndrome have centered on contaminants, chemically related to L-tryptophan, introduced in the manufacturing process. Tryptophan metabolites have been linked with sclerotic cutaneous diseases but have not been previously implicated in cutaneous mucinoses.

Adult↗
PubMed2000

Lipedematous alopecia: a clinicopathologic, histologic and ultrastructural study.

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.

Adipose Tissue↗
PubMed1998

Reticulate postinflammatory hyperpigmentation with band-like mucin deposition.

BACKGROUND: Mucinoses of the skin are a group of disorders sharing accumulation of mucin in the skin or hair follicles. Postinflammatory hyperpigmentation, with pigmentary incontinence, is due to loss of melanin from epidermal basal cells and its accumulation in dermal macrophages. METHODS: We describe clinicopathologic features of two patients presenting with the association of pigmentary incontinence with an unusual diffuse, band-like dermal deposition of mucin, clinically presenting with reticular pigmented macular lesions. RESULTS: Two patients were observed with asymptomatic, persistent, reticular, pigmented patches located in the flexures, thighs, neck and back. Histology showed melanophages with a diffuse, band-like dermal deposition of mucin, an increased number of fibroblasts, a slight T-cell infiltrate and scattered mast cells. Blood markers of lupus erythematosus were negative. CONCLUSIONS: These findings may draw attention to pigmentary disorders such as lichen planus pigmentosus, erythema dyschromicum perstans, pigmentatio maculos eruptiva idiopathica, dermatopathia pigmentosa reticularis, prurigo pigmentosa and frictional melanosis. None of these entities, however, includes mucin deposition among its microscopic features. Macules were not preceded by erythema or any other lesions. We suggest that our cases could belong to group II of Rongioletti and Rebora's classification, i.e. they could be cases of secondary mucin deposition in postinflammatory hyperpigmentation, possibly in an unusual form of lichen planus pigmentosus or, less likely, frictional melanosis.

Adult↗
PubMed2001

Cutaneous lupus erythematosus.

Lupus erythematosus (LE) has many different clinical manifestations including a variety of cutaneous findings. Some of the cutaneous manifestations are not specific for LE, such as photosensitivity reactions, oral ulcers, alopecia, urticaria, vasculitis, vesiculo-bullous lesions, acral changes, cutaneous mucinoses, and cutaneous calcinosis. Other findings are specific for LE in that they are found only in patients who have lupus erythematosus. These LE-specific disorders include acute cutaneous LE, subacute cutaneous LE, and several forms of chronic cutaneous LE, including discoid LE. Skin biopsies are often helpful in differentiating LE-specific skin lesions from other disorders that can mimic them. Photoprotective measures and a number of drugs are useful in treating cutaneous LE.

Anti-Inflammatory Agents, Non-Steroidal↗