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Results for “Anetoderma”

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PubMed2006

Secondary anetoderma associated with mastocytosis.

BACKGROUND: Mastocytosis represents a wide spectrum of proliferative disorders of mast cells in the bone marrow, skin and/or internal organs. The most common manifestation is urticaria pigmentosa (UP), which is characterized by small or large brown-red maculopapules on the skin. Occasionally, elastic and collagen fibers in the lesions degenerate and result in a lax area of skin termed anetoderma. METHODS: We report a 21-year-old male patient with multiple cutaneous anetodermic lesions, present since infancy, at UP sites confirmed with histochemistry. RESULTS: Urinary N-methyl 24-hour histamine levels were elevated, but serum tryptase levels were within normal limits. Radiologic examination of long bones was unremarkable, as well as all other blood results. UP biopsy showed absence of epidermal involvement and increased number of mast cells located perivascularly. There was fragmentation of elastic fibers in the papillary dermis. CONCLUSIONS: A patient with multiple cutaneous anetodermic lesions, presented since infancy, led to the diagnosis of UP. Such an association is rare and raises intriguing questions concerning the pathogenesis of anetoderma.

Adult↗

PubMed2002

Primary anetoderma associated with primary Sjögren's syndrome.

We report the case of a woman with a primary Sjögren's syndrome who developed asymptomatic anetoderma lesions with no other pathology responsible. This dermatosis has been associated with many autoimmune disorders, in particular lupus erythematosus and lupus-like syndromes. Our literature review found only one previous description of primary anetoderma associated with primary Sjögren's syndrome.

Adult↗

PubMed2003

Anetoderma associated with antiphospholipid syndrome and systemic lupus erythematosus.

Anetoderma is an uncommon disorder characterized by the loss of elastic fibres in the dermis histologically and herniation of subcutaneous tissue clinically. Recent studies indicate that immunologic mechanisms may play a role in this process. Here we report a 33-year-old woman with numerous well-circumscribed, asymptomatic skin lesions in whom clinical and histopathologic features were consistent with anetoderma. Additionally, history and investigations revealed antiphospholipid syndrome and systemic lupus erythematosus. It has been speculated that immune deposits in the dermis or within the capillary walls may lead to ischaemia and subsequent degeneration of the elastic fibres.

Adult↗

PubMed2000

Primary anetoderma.

The authors report a case of primary anetoderma in a 27-year-old black woman. Primary anetoderma is characterized by circumscribed oval plaques with overlying loose, wrinkled skin appearing as a depression or pouch-like protrusion of the skin.

Adult↗

PubMed1992

Anetoderma and human immunodeficiency virus infection.

BACKGROUND: Autoimmune disorders such as vitiligo and alopecia areata have been reported in persons infected with the human immunodeficiency virus (HIV). OBSERVATIONS: Three HIV-infected men had anetoderma develop early in the course of their HIV infection. In two patients this preceded other features of HIV disease, and in the third it occurred while the helper T-cell count exceeded 200/mm3. CONCLUSIONS: These findings suggest that anetoderma may be an early manifestation of HIV disease, perhaps autoimmune in its pathogenesis.

Adult↗

PubMed1988

[Pseudotumoral lupus anetoderma. Child chorea. Development over 28 years].

The finding in a 40-year old and apparently healthy woman of a pseudotumoral lesion of the left arm which had gradually developed over a 9-year period led to a tentative clinical diagnosis of lupus anetoderma. This diagnosis was confirmed on the one hand by histological examination of the skin--which showed almost total disappearance of dermal elastic fibres, while the lympho-histiocytic infiltrate was so discreet that it excluded a deep lupus--and on the other hand by direct immunofluorescence, with lupus band on the lesion and a few abnormalities of the exposed and covered healthy skin. A history of chorea at the age of 13 years raised the problem of a possible relationship between this neurological disease and a systemic lupus erythematosus. This hypothesis was supported by resolutive episodes of arthralgia and abnormal laboratory findings, such as accelerated ESR, leucopenia, decrease of complement and presence of homogeneous antinuclear antibodies. Anticardiolipin antibodies, circulating anticoagulants and VDRL test were negative. Treatment with anti-malarials brought about some degree of cutaneous shrinkage and a significant decrease in ESR. This case is reported because anetoderma as sole manifestation of systemic lupus erythematosus and the occurrence, 17 years previously, of chorea are exceptional events.

Adult↗

PubMed1984

Ultrastructural findings in the skin lesions of patients with anetoderma.

Eight biopsy specimens from the skin lesions of five patients with anetoderma were studied for their ultrastructural findings. In all of them, normal elastic fibers were absent and a few very thin, irregular elastic fibers with a more or less complete loss of the amorphous substance and a relative conservation of the microfibrils were observed. The collagen fibers were normal. Inflammation composed of macrophages and lymphocytes, with some plasma cells, was a prominent finding. It is suggested that anetoderma and acquired cutis laxa are part of the same spectrum of elastolytic disease.

Adolescent↗

PubMed1995

Anetoderma of Jadassohn-Pellizzari.

Anetoderma (derived from the Greek anetos, meaning slack) is a term used to describe localized increased laxity of the skin with herniation or outpouching, resulting from abnormal dermal elastic tissue. Primary anetoderma is distinctly rare. We describe a case where we suspect an auto-immune aetiology.

Adult↗

PubMed1996

[Pilomatrixoma associated with anetoderma].

The authors describe a pilomatricoma with perilesional anetoderma in a young woman. The papillary dermis showed a complete lack of elastic fibres and epidermal atrophy. Clinical diagnosis of pilomatricoma is possible but histologic examination is required to differentiate it from cystic lesions or other benign appendage tumors. At different rates shadow and basophilic cells must be found. The association of a pilomatricoma with anetoderma appears more frequent than usually reported and is important for clinical diagnosis. The histogenesis of this cutaneous lesion is controversial: probably there is a relation between inflammatory processes of pilomatricomas and lack of elastic fibres.

Adult↗

PubMed1998

Primary anetoderma.

Primary anetoderma is a rare disorder characterized by sac-like herniations and depressions in previously normal skin. On histologic examination, elastic fibers are destroyed by an inflammatory infiltrate. We describe a striking case of primary anetoderma and review the classification, differential diagnosis, causes, and treatment.

Adult↗

PubMed1987

Postgranulomatous anetoderma associated with Takayasu's arteritis in a child.

Takayasu's arteritis (TA) is a rare chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum and subcutaneous inflammatory lesions of the leg are the most frequent. We studied a boy with TA in whom a papular rash of the trunk preceded the onset of vascular symptoms by many years. Histologically, the lesions were superficial and consisted of middermal noncaseating tuberculoid granulomas, which progressed to atrophy and anetoderma because of elastic network disruption. Granulomas were also found in synovial tissue but not in a temporal artery biopsy specimen, which showed only intimal hyperplasia. Our observations suggest that vascular and skin lesions with elastic tissue may both result from a common granulomatous hypersensitivity process.

Aortic Arch Syndromes↗

PubMedDate not supplied

Connective tissue diseases: pseudoxanthoma elasticum, anetoderma, and Ehlers-Danlos syndrome in pregnancy.

The relationship between pregnancy and diseases of the elastic fibers, such as pseudoxanthoma elasticum, cutis laxa, and anetoderma, is discussed in this article. Dermatologists and other physicians must be aware that these problems may be present in pregnant women and must also know how to counsel those who suffer from these diseases because they can have severe manifestations and consequences during or after this period for both the pregnant mother and her offspring.

Connective Tissue Diseases↗

PubMed1998

Hamartomatous congenital melanocytic nevi showing secondary anetoderma-like changes.

A 47-year-old man had multiple large congenital melanocytic nevi associated with striking atrophic changes. Biopsy specimens showed a prominent reduction and fragmentation of elastic fibers in the papillary dermis. The anetoderma-like changes may have been secondary to a past inflammatory process but more likely represent a hamartoma.

Connective Tissue Diseases↗

PubMed2003

Familial anetoderma.

A 31-year-old Caucasian male presented with a history of erythematous, saccular outpouchings of the skin on his back, shoulders, and upper extremities (Fig. 1). The patient reported that his mother and aunt had a similar skin disorder, which initially began with inflammation, but healed leaving lax skin in its wake. He did not recall the name of the skin condition. Physical examination revealed large confluent zones of sac-like protrusions of erythematous skin on the back and shoulders. Histopathologic examination of the excisional biopsy revealed a relatively unremarkable epidermis. Perivascular lymphocytes were present in small numbers in the papillary dermis. Adnexal structures and deep dermis remained intact. The acid-orcein-Giemsa stain highlighted the absence of elastic fibers within the mid and lower reticular dermis (Fig. 2a,b), consistent with anetoderma.

Adult↗

PubMed2001

Anetoderma associated with antiphospholipid antibodies: case report and review of the literature.

The association of primary anetoderma (PA) with antiphospholipid antibodies (APAs; with or without criteria of primary antiphospholipid syndrome) has been observed repeatedly and a possible pathogenic significance of this asssociation has been hypothesized. We report the case of a 21-year-old-woman who developed anetodermic lesions on her upper trunk and arms. The presence of APAs was demonstrated, but no diagnostic criteria of systemic lupus erythematosus were present. A review of the literature disclosed 20 previously reported patients presenting this peculiar association. Thrombotic phenomena, represented by spontaneous abortions or venous thrombosis, are a frequent complication in this setting. Taking these observations into account a systematic search for APAs in patients with PA seems advisable.

Adult↗

PubMed2002

Anetoderma: an altered balance between metalloproteinases and tissue inhibitors of metalloproteinases.

The amount of elastic fibers from lesional and healthy skin areas of five patients with anetoderma was determined by automated image analysis. Dermal elastic fibers were almost completely absent in anetodermic skin and preelastic fibers were undetectable or extremely rare. Organ cultures were performed using explants from affected and unaffected skin areas of the same patient. We identified and quantified proteases in the culture media of explants: MMP-1 (collagenase 1), MMP-2 and MMP-9 (gelatinases A and B), MMP-3 (stromelysin 1), MMP-7 (matrilysin 1), and tissue inhibitors of metalloproteinases, TIMP-1 and TIMP-2. The data were compared with those of two healthy donors. For the five samples of anetodermic skin, MMP-1 levels were significantly higher compared with the uninvolved cultures and the two healthy samples. A significant increase of TIMP-1 expression was also observed in the affected cultures. We demonstrated a significant increase in the production of gelatinase A in lesional skin when compared with nonlesional skin and healthy donor samples. We found no significant production of TIMP-2 in the five samples of anetodermic skin compared with the samples from the two healthy donors. There was a significant decrease in TIMP-2 expression in the five nonlesional samples compared with the control samples. These data are in favor of an altered balance in anetodermic patients between MMP-2 and TIMP-2. Levels of MMP-9, MMP-3, and MMP-7 were significantly higher in the culture-conditioned media of the anetodermic skin samples than the nonlesional skin cultures. Because MMP-3, MMP-7, MMP-9 are known to degrade elastin, and MMP-3 can activate the latent forms of MMP-7 and MMP-9, we propose that these metalloproteinases also participate in the degradation of elastic fibers in anetodermic skin.

Adolescent↗

PubMed1997

Increased expression of gelatinases A and B by skin explants from patients with anetoderma.

The extent of alterations to the elastic fibre network in lesional skin areas of three patients with anetoderma was assessed by quantitative image analysis of tissue sections and compared with morphometric parameters from unaffected sites of the same individuals. In the anetodermic skins pre-elastic fibres were undetectable or extremely rare: the volume fraction (Vv%) occupied by these pre-elastic fibres was 0-0.3%, while in unaffected skins the Vv% occupied by pre-elastic fibres was 0.5-0.8%. A nearly complete absence of dermal elastic fibres in lesional skins from the three patients was evidenced (Vv% = 0.2-0.3%). Organ cultures were performed using explants from skin with or without anetodermic lesions to quantify the expressions of elastase-type proteinases. All tissues from anetodermic lesions expressed proforms of gelatinases A and B and the activated form of gelatinase A; their levels increased with the culture time. In comparison, enzymatic activities on oligopeptide substrates specific for leucocyte elastase and fibroblast plasma membrane-associated metalloelastase were not detected in the conditioned media of any explants at any time of culture from 1 to 5 days. Increased production of progelatinases A and B and activation of progelatinase A could be mainly responsible for the degradation of skin elastic fibres demonstrated in anetodermic skins.

Adult↗

PubMedDate not supplied

Anetoderma.

Anetoderma is a rare cutaneous condition with characteristic clinical features and histopathology. The condition is classified into idiopathic (Jadassohn or Pellizzari type) and atrophic (Schweinger-Buzzi type) concomitant with other diseases, and secondary to infection or drugs.

Atrophy↗