Mucinoses
Cutaneous mucinosis and mastocytosis in a shar-pei. (1/11)
A 7-year-old shar-pei was presented because of a recurrent dermatologic condition. Skin biopsies revealed an idiopathic (primary) cutaneous mucinosis that initially responded to corticosteroids. The condition reappeared 2 years later and subsequent biopsies revealed a mast cell tumor in some of the skin sites previously diagnosed with mucinosis. (+info)Severe lung involvement in systemic scleromyxoedema: a highly unusual finding. (2/11)
Scleromyxoedema is a rare systemic disorder characterized by a lichenoid papular rash. Although scleromyxoedema can involve any organ, very few cases of pulmonary involvement have been reported. Moreover, there are no reports in the literature on treatment of this condition, especially with lung transplantation. The authors report a case of scleromyxoedema in a young man with neurological, skin and respiratory involvement, the latter being mainly characterized by pulmonary emphysema. Due to the serious respiratory compromise, and to the stability of the systemic lesions, a bilateral lung transplantation was performed with successful results at 4.5 yrs. In conclusion, lung transplantation may be required in patients with severe respiratory failure caused by scleromyxoedema. (+info)Papulonodular mucinosis in systemic lupus erythematosus. (3/11)
A 41-year-old man with systemic and serological manifestations of systemic lupus erythematosus presented with a diffuse eruption comprising annular plaques. Histopathology revealed diffuse deposition of mucin throughout the dermis, consistent with papulonodular mucinosis. This uncommon entity of unclear pathogenesis has been described in systemic lupus erythematosus, discoid lupus erythematosus, and subacute cutaneous lupus erythematosus. (+info)Pimecrolimus 1 percent cream and pulsed dye laser in treatment of a patient with reticular erythematous mucinosis syndrome. (4/11)
We report on the efficacy of twice daily application of pimecrolimus 1 percent cream in a 48-year-old woman with reticular erythematous mucinosis (REM) syndrome and compare its results with pulsed dye laser (PDL) on the other side of her chest and back. The patient was previously treated by hydroxychloroquine but only a fair response was observed. After application of 5 months of pimecrolimus, the lesions completely resolved and the result was comparable with the other side of her body treated by pulsed dye laser PDL. Topical pimecrolimus and pulsed dye laser appear to be effective and safe treatments for REM. (+info)Linear morphea with secondary mucinosis. (5/11)
(+info)Hereditary progressive mucinous histiocytosis: first report in a male patient. (6/11)
(+info)Brown plaques on the lower back. (7/11)
A 12-year-old girl presented with a 2-year history of a 10 cm area of asymptomatic brown coalescent plaques of coalescent, linearly arranged papules on the lower back. No other physical or systemic abnormality was detected. Routine laboratory investigations, including thyroid function tests, were normal. The histopathological examination revealed hyperkeratosis, papillomatosis, and elongated rete ridges in the epidermis. On special staining (Alcian blue), mucin deposition was seen in the papillary dermis. The remaining dermis was normal. A diagnosis of Mucinous Nevus was made. (+info)Oral focal mucinosis: a rare case report of two cases. (8/11)
Oral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology. Clinically, it is most commonly found on the gingiva and presents as a painless, sessile or pedunculated mass of the same colour as the surrounding mucosa. Histologically, it is characterized by focal myxoid degeneration of connective tissue. OFM occurs predominantly in adults during the fourth and fifth decade of life, although it has been reported infrequently in children and adolescents. Its diagnosis mainly relies on histological analysis and the treatment involves complete surgical excision. Its recurrence is unreported. The aim of this report of two cases is to describe the clinical and histological presentation and subsequent management of OFM. The cause of OFM remains unknown. The cases presented in this report bring OFM to the attention of anatomical pathologists while considering the differential diagnosis of myxoid lesions of the oral cavity. (+info)Mucinoses are a group of cutaneous disorders characterized by the abnormal deposit of mucin in the dermis. Mucin is a complex sugar-protein substance that provides cushioning and lubrication to various tissues in the body. In mucinoses, an excess of mucin accumulates in the skin, leading to various clinical manifestations such as papules, nodules, plaques, or generalized swelling.
Mucinoses can be classified into two main categories: primary and secondary. Primary mucinoses are caused by genetic mutations that affect the production or degradation of mucin, while secondary mucinoses occur as a result of other underlying medical conditions, such as autoimmune disorders, infections, or neoplasms.
Examples of primary mucinoses include:
* Lichen myxedematosus (also known as papular mucinosis): characterized by multiple, firm, flesh-colored to yellowish papules and nodules, usually on the trunk and proximal extremities.
* Follicular mucinosis: a condition that affects hair follicles and is characterized by the accumulation of mucin in the follicular epithelium, leading to hair loss, itching, and inflammation.
* Scleromyxedema: a rare systemic disorder characterized by generalized thickening and hardening of the skin due to excessive deposition of mucin and collagen fibers.
Examples of secondary mucinoses include:
* Lupus erythematosus: an autoimmune disorder that can affect various organs, including the skin, and is characterized by the accumulation of mucin in the dermis.
* Dermatomyositis: another autoimmune disorder that affects the skin and muscles, and can also cause mucin deposition in the dermis.
* Rosai-Dorfman disease: a rare histiocytic disorder characterized by the accumulation of large, foamy histiocytes that contain mucin in the lymph nodes and other organs, including the skin.
The diagnosis of mucinoses is usually based on clinical examination, skin biopsy, and laboratory tests. Treatment depends on the underlying cause and may include topical or systemic medications, phototherapy, or surgical intervention.
Pretibial myxedema
Lichen myxedematosus
Perifollicular mucinosis
Nodular lichen myxedematosus
Myxoid lipoblastoma
Myxoid cyst
Cutaneous lupus mucinosis
Eccrine mucinosis
Self-healing juvenile cutaneous mucinosis
Reticular erythematous mucinosis
Self-healing papular mucinosis
Localized lichen myxedematosus
Atypical tuberous myxedema
Atypical lichen myxedematosus
Acral persistent papular mucinosis
Cutaneous focal mucinosis
Discrete papular lichen myxedematosus
Papular mucinosis of infancy
Mucinosis
Alopecia mucinosa
Oral mucocele
List of skin conditions
Scleroderma
Stiff skin syndrome
Systemic scleroderma
Neuropathia mucinosa cutanea
Papular mucinosis
Cases reported • Mucinoses
Pretibial myxedema - Wikipedia
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Kerry R Carder - Research output - University of Texas Southwestern Medical Center
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Follicular3
- BACKGROUND: Primary cutaneous mucinoses (PCM) are rare diseases characterized by dermal or follicular mucin deposits. (bvsalud.org)
- MUC1 expression in tissue histiocytes, fibroblasts, CD4+ and CD8+ T cells, and follicular epithelial cells of FM was significantly higher than the same cell types in the dermal mucinoses (p (bvsalud.org)
- Using MFS, we showed that CD8+ T cells seem to be more involved in the production of mucin in FM than in dermal mucinoses, which could indicate that mucin in dermal and follicular epithelial mucinoses have different origins. (bvsalud.org)
Connective Tissue1
- 4. Secondary dermal mucinoses are associated with autoimmune connective tissue disorders. (eyepath.org.uk)
Cutaneous1
- Cutaneous mucinoses. (medlineplus.gov)