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PubMed1996

Localization of dermal edema in lipodermatosclerosis, lymphedema, and cardiac insufficiency. High-frequency ultrasound examination of intradermal echogenicity.

BACKGROUND: Chronic edema in venous insufficiency is associated with leg ulceration, whereas in lymphedema skin ulceration is less frequent and edema from cardiac failure does not cause major skin changes. The reason for these differences is unclear. OBJECTIVE: Our purpose was to investigate, by means of ultrasound, the distribution of intradermal fluid in patients with edema associated with lipodermatosclerosis, lymphedema, or cardiac insufficiency. METHODS: Sixteen patients with lipodermatosclerosis, 10 with lymphedema, 16 with leg edema from heart insufficiency, and 16 healthy control subjects were studied. Ultrasound images were obtained with a 20 MHz scanner. The echogenicity of the dermis was quantified by image analysis and the echogenicity of the upper (subepidermal) and lower portions of the dermis were measured. RESULTS: In any type of edema the dermal echogenicity was lower than that of the control group, indicating intradermal edema. In normal skin the upper dermis was only slightly less echogenic than the lower dermis. In patients with lipodermatosclerosis a low-echogenic area was present in the upper dermis, which suggests the papillary dermis as a preferential site of edema formation. In contrast, in lymphedema dermal echogenicity was uniformly distributed, whereas in patients with heart failure the lower portion of the dermis was less echogenic than the upper dermis. CONCLUSION: This study demonstrates that intradermal echogenicity shows characteristic patterns in different types of edema, which indicates that localization of fluid varies in the dermis, from subepidermal (lipodermatosclerosis), uniform (lymphedema), to deep dermal (heart failure). These differences may help in understanding the origin of different skin sequelae of edema.

Adolescent↗
PubMed1983

Positron emission tomography in venous ulceration and liposclerosis: study of regional tissue function.

Oxygen-15 administered by continuous inhalation and emission computed tomography were used to study regional tissue oxygen utilisation and blood flow in the limbs of 11 patients with venous ulceration and five patients with liposclerosis due to venous insufficiency. The results showed increased blood flow and appreciably reduced fractional oxygen extraction in the diseased tissues. These findings indicated a local, functional shunting of blood through the abnormal microcirculation of the skin and subcutaneous tissues. This may be an important factor in the aetiology of the skin changes seen in patients with calf muscle pump failure.

Humans↗
PubMed1989

Hemorrhagic bullous lesions in morphea.

The case of a 20-year-old girl who had morphea with bullous lesions is reported. Two bullae appeared on an indurated patch of morphea on the right side of the trunk. A half-moon-shaped lesion was localized along the superior border of the patch and the other followed the anterior border and extended into the patch. Both contained a hemorrhagic fluid. The bullae spontaneously regressed in a few months without therapy. We emphasize the very rare occurrence of hemorrhagic bullous lesions as a complication of morphea.

Adult↗
PubMed1998

Lipodermatosclerosis is characterized by elevated expression and activation of matrix metalloproteinases: implications for venous ulcer formation.

Lipodermatosclerosis refers to skin induration of the lower extremities and is associated with patients preceding venous ulcerations. To better understand the pathogenesis of ulcer formation we investigated the expression of matrix metalloproteinases (MMP) and tissue inhibitors of metalloproteinases (TIMP) in lipodermatosclerosis. By preparing biopsies from healthy skin and liposclerotic lesions, MMP-1, MMP-2, MMP-9, TIMP-1, and TIMP-2 were analyzed by using reverse transcriptase-polymerase chain reaction, western blot, zymography, hydrolysis of [3H]labeled collagens, and immunohistochemistry. Our investigations provide evidence that mRNA and protein expression of MMP-1, MMP-2, and TIMP-1 were significantly increased in lipodermatosclerosis, whereas the total amount of MMP-9 and TIMP-2 mRNA and protein was not altered. Western blot of liposclerotic lesions revealed an inactive proMMP-1-TIMP-1 complex, whereas MMP-2 was prominent as an active 66 kDa band. Increased proteolytic activity of MMP-2 could be proven in lesional in comparison with healthy skin by zymography and [3H] collagen degradation. Increased diffuse staining was found for MMP-1 in the epidermis and dermis in comparison with controls. In lipodermatosclerosis, MMP-2 was predominantly localized in the basal and suprabasal layers of the epidermis, in perivascular regions, and in the reticular part of the dermis. Furthermore, MMP-2 was imbalanced by locally reduced expression of TIMP-2 in the basement membrane zone of lesional skin. Our findings indicate lipodermatosclerosis to be characterized by elevated matrix turnover.

Collagen↗
PubMed2006

[Skin manifestations of Lyme borreliosis].

Lyme borreliosis is the most common tick-borne disease which is caused by Borrelia burgdorferi and transmitted in the Poland, as well as Europe, primarily by Ixodes ticks. After inoculation spirochetes spreads in the skin, activate locally immune response mechanisms and cause characteristic skin lesions like erythema migrans, lymphadenosis benigna cutis, erythema migrans multiplex and acrodermatitis chronica atrophicans. The correct clinical diagnosis of skin lesions and antibiotic treatment is most important for regression of symptoms and prevention of late manifestations of Lyme disease.

Acrodermatitis↗
PubMed1995

Zosteriform atrophoderma of Pasini and Pierini.

Astophoderma of Pasini and Pierini is generally regarded as an atrophic variant of morphoea. It arises most commonly on the trunk and abdomen. We describe a 53-year-old man who had a 12-year history of unilateral atrophoderma of Pasini and Pierini affecting the left side of his trunk in a zosteriform distribution. Within these clinically atrophic lesions, there were localized areas of induration which were typical of morphoea. The patient also had a preceding history of balanitis xerotica obliterans. The occurrence of atrophoderma of Pasini and Pierini, morphoea and lichen sclerosus within the same individual supports the view that these conditions are closely related, and suggests that common factors may be involved in their aetiologies.

Humans↗
PubMed1990

[Preputial sclero-atrophic lichen in children].

Clinicopathological findings and outcomes in ten pediatric cases of lichen sclerosus and atrophicus of the foreskin are reported. This well-defined entity usually presents as acquired sclerous phimosis. Diagnosis rests on histologic studies of biopsy specimens. Pathologic features change during the course of disease and are useful for selecting the most appropriate therapy. Local corticosteroid therapy may be helpful in early disease. However, circumcision is usually required for advanced disease, failure of medical therapy or prevention of squamous cell carcinoma. Pathophysiology is discussed. The incidence of this condition seems to be underestimated. Possible connections with diabetes mellitus and other conditions are reviewed.

Adolescent↗
PubMed1992

Analysis of collagen gene expression by cultured fibroblasts in morphoea.

Collagen gene expression was studied in dermal fibroblasts derived from the inflammatory and sclerotic skin lesions of patients with localized or generalized morphoea. The levels of mRNA for type I collagen in early-passage fibroblasts derived from inflammatory lesions were higher than those obtained from the uninvolved skin, whereas those fibroblasts obtained from sclerotic lesions were unaltered. No alteration in type I collagen mRNA levels was observed in late-passage fibroblasts derived from the inflammatory lesions. The relative rate of collagen to total protein synthesis in early-passage fibroblasts derived from inflammatory lesions was higher than that of fibroblasts from uninvolved skin, while no alteration or a slight decrease was observed in fibroblasts from the sclerotic lesions. The data suggest that the inflammatory reactions induce increased collagen synthesis by fibroblasts in the skin in scleroderma.

Actins↗
PubMed1981

Finger systolic pressures and skin temperatures in severe Raynaud's syndrome: the relationship to healing of skin lesions and the use of oral phenoxybenzamine.

Systolic pressures and skin temperatures were studied in 23 patients with severe Raynaud's syndrome who were given oral phenoxybenzamine. There was a relationship between the measurements and the severity of the disease. Statistically significant correlation was found between the measurements and the time for healing of skin lesions. The chances of early healing are high when digital systolic pressure exceeds 55 mm Hg, the branchial-finger pressure difference is less than 50mm Hg, and the maximal difference among fingers less than 30 mm Hg. Early healing is also likely when finger temperature of 10 degree C or more above room temperature is achieved during "reflex" heating and the difference in temperature among fingers is less than 4 degree C. Therefore, the measurements are of value in the determination of prognosis. Rest pain, local tenderness, trophic skin changes, and vasospastic phenomena improved in the majority of patients soon after beginning treatment with phenoxybenzamine. Some side effects, usually minor, were common but with doses not exceeding 50 mg used in this study they were usually not trouble-some and the drug was tolerated well. Phenoxybenzamine is a valuable drug in the treatment of patients with severe vasospastic disease.

Administration, Oral↗
PubMed1986

A case of primary biliary cirrhosis associated with generalized morphea.

A 50-year-old female with primary biliary cirrhosis associated with generalized morphea is reported. She had suffered from multiple painful indurated plaques on the trunk which was diagnosed as generalized morphea; subsequently, primary biliary cirrhosis was diagnosed on the basis of her hepatic dysfunction. Although progressive systemic sclerosis has been reported to occur in patients with primary biliary cirrhosis, this localized form of cutaneous sclerosis has not previously been reported. Since generalized morphea is one of the most common manifestations of chronic graft-versus-host disease, and primary biliary cirrhosis is known to have a hepatic histology resembling chronic graft-versus-host disease, this case report may represent evidence in support of a common pathogenesis for the two entities.

Female↗
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↗
PubMed1978

Speckled (particulate) epidermal nuclear IgG deposition in normal skin. Correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen.

Clinical and laboratory findings were correlated from 46 patients with IgG localization in epidermal nuclei in a speckled (particulate) pattern on direct immunofluorescence of normal skin. Cutaneous manifestations included lupus erythematosus (LE), swollen hands or sclerodactyly, alopecia, vasculitis, and dyspigmentation. Systemic manifestations included arthritis or arthralgia, Raynaud's phenomenon, serositis, vascular headaches, mild renal disease, myositis, and sicca syndrome. High titer (mean = 1:142, 800) serum antibody to extractable nuclear antigen (ENA) was found in 81%. Eighty-six percent had antibody to an RNase-sensitive antigenic component of ENA (ribonucleoprotein or RNP); 14% had antibody to an RNase-resistant ENA termed Sm. Deposition of IgG in a speckled pattern in epidermal nuclei is an immunopathologic marker for a subset of connective tissue disease characterized by antibody to ENA. Those with Sm specificity had systemic LE (SLE); Those with RNP specificity had Raynaud's phenomenon usually associated with overlapping features of SLE, scleroderma, and/or dermatomyositis.

Adolescent↗
PubMed1997

[Chronic sclerodermiform syndrome disclosing subcutaneous T-cell lymphoma].

INTRODUCTION: Subcutaneous tissue is an uncommon primary localization for T-cell lymphomas. Panniculitis with recurrent papulonodules are reported in most cases. CASE REPORT: We report a case in which small-cell pleiomorphic non-Hodgkin lymphoma developed initially in subcutaneous tissue followed by secondary systemic extension. The first clinical manifestation was a sclerodermiform syndrome involving the four limbs. DISCUSSION: This type of lymphoma appears to have a polymorphic clinical presentation. Physicians should be aware of this type of lymphoma and use immunohistological techniques for early diagnosis.

Fatal Outcome↗
PubMed2003

Widespread morphoea following radiotherapy for carcinoma of the breast.

We report a case of a 60-year-old lady who was treated with radiotherapy for breast cancer of both breasts 8 years apart. Thirteen years after the first dose of radiotherapy she developed localized morphoea in all the irradiated skin of the chest wall and also the gaiter regions of both lower legs. Radiation-induced localized morphoea has been previously reported; however, there is no previous publication of an occurrence at a distant site as in this case. This case demonstrates that morphoea can occur distant to the original breast carcinoma and site of radiotherapy. We postulate that radiotherapy can induce neoantigen formation, which initiates a T cell response and subsequent tissue growth factor alpha release. Tissue growth factor alpha induces fibroblast activation and collagen production may persist due to a positive feedback mechanism within the fibroblast. The reason why the disease did not generalize remains unclear.

Breast↗
PubMedDate not supplied

Associated localization of morphea and lichen planus of the lip in a patient with vitiligo.

Morphea, lichen planus and vitiligo are common diseases, but seldom associated. Morphea or lichen planus in the lips have not frequently been reported, and an associated localization of both lesions in the lip mucosa is indeed a rare event. A case of morphea and lichen planus localized in the upper lip mucosa in a patient with vitiligo is reported. Immunohistochemistry revealed a clear prevalence of T-lymphocytes in the inflammatory infiltrates of morphea and in the band-like infiltrate of the lichen, a finding consistent with previous reports of morphea, lichen planus, and vitiligo. The inverse ratio found between CD8+ and CD4+ lymphocytes, compared to other reports, seems to confirm that the pathogenesis of morphea and lichen planus evolves through different stages. The present report, besides presenting a rare case, may prompt to consider a common pathogenesis among morphea, lichen planus and vitiligo.

Humans↗
PubMed2002

Lipodermatosclerosis: review of cases evaluated at Mayo Clinic.

BACKGROUND: Lipodermatosclerosis describes bound-down, sclerotic skin involving the lower extremities. OBJECTIVE: Our purpose was to describe the demographic and clinical features of patients with lipodermatosclerosis. METHODS: This was a retrospective study of patients presenting to Mayo Clinic between 1976 and 1998 with a diagnosis of lipodermatosclerosis. RESULTS: Of 97 patients, 84 (87%) were women. Mean age was 62 years (range, 25-88 years). Mean body mass index was 34.3 (range, 17.8-71.5). Clinical signs were bilateral involvement in 44 patients (45%), induration localized to a discrete plaque in 49 (51%), erythema in 69 (71%), hyperpigmentation in 57 (59%), ulceration in 13 (13%), concomitant edema in 69 (71%), and varicosities in 55 (57%). Vascular studies performed on 72 patients showed abnormalities in 49: deep venous incompetence in 33 (67%), calf muscle pump abnormality in 19 (39%), abnormal pulsatility in 10 (20%), and obstruction in 1 (2%). CONCLUSION: Lipodermatosclerosis was associated with female sex, middle age, high body mass index, and venous abnormalities.

Adult↗
PubMed1993

[Localization and degree of expression of dermatoliposclerosis can be well evaluated with 20 MHz ultrasound].

33 legs of patients with chronic venous insufficiency and 21 legs of healthy persons were examined by high resolution 20 MHz ultrasound. With increasing lipodermatosclerosis--which was most pronounced in the medium part of the lower leg--an increase of thickness and a decrease of echo density of the dermis could be seen. The first sign of decreased density was an echolucent band in the upper part of the dermis. While in healthy subjects dermis and subcutaneous fat were clearly separated in ultrasound, in mild sclerosis differentiation was more difficult; in extensive lipodermatosclerosis it often was not possible to distinguish these parts of the skin. Localisation and extent of lipodermatosclerosis can be well demonstrated and evaluated by 20 MHz ultrasound.

Adipose Tissue↗

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