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PubMed1992

Immunologic abnormalities associated with primary anetoderma.

BACKGROUND AND DESIGN: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. RESULTS: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. CONCLUSIONS: These findings indicate that there is an immunologic involvement in primary anetoderma.

Adult↗
PubMed1987

[Anetoderma and lupus erythematosus. Study of 2 cases].

Two cases of anetoderma and lupus erythematosus (LE) were studied. The first one showed secondary anetoderma and subacute LE. There was leucocytoplastic angitis in involved skin besides generalized lacking of elastic fibers (EF). The second one is a Schweninger Buzzi anetoderma with face and scalp lesions of LE tumidus. In new lesions angitis and immunoglobulin deposits of EF were seen. Another cases, pathogenic and characteristics of this unusual association were analyzed we pointed out: 1) leucocytoplastic angitis presence; 2) immunoglobulin deposits on EF finding; 3) angitis seems to play a principal rol in elastic fibers disappearance.

Adult↗
PubMed1984

Histopathologic findings in anetoderma.

We reviewed the histopathologic findings in 34 biopsy specimens from 15 patients with anetoderma. Focal loss of normal elastic fibers, necessary for the diagnosis of anetoderma, was found in all 15 cases. The persistence of fine, irregular, or twisted elastic fibers is common. A perivascular infiltrate composed of lymphocytes was found in all specimens. Plasma cells were observed in specimens from six patients, and histiocytes with some granuloma formation were found in specimens from six patients. Variations in the intensity of inflammation or in the loss of elastic tissue were not related to the clinical findings, course, or associated diseases. All anetoderma lesions appeared to have an inflammatory pathogenesis.

Atrophy↗
PubMed1984

Anetoderma: biochemical and ultrastructural demonstration of an elastin defect in the skin of three patients.

Three patients with localized cutaneous lesions characteristic of anetoderma were studied. Clinically, the onset of the disease was between the ages of 17 and 25, and numerous flaccid, saclike skin lesions developed over several subsequent years. Histologically, the lesions were characterized by paucity and fragmentation of the elastic fibers. Electron microscopy demonstrated that the elastic fibers, both in papillary and deep reticular dermis in the lesional skin, were fragmented and irregular in appearance. The concentration of elastin, determined by a radioimmunoassay of desmosine, an elastin-specific cross-link compound, was markedly reduced in the lesions, as compared with unaffected skin from the same patients or with normal skin from unrelated control subjects. In contrast, the concentrations of hydroxyproline, an index of collagen, or deoxyribonucleic acid (DNA), a measure of cellularity, were not changed in the lesions. Thus, the results indicate that in the three patients studied, the elastic fibers are defective and reduced in quantity. These observations suggest that the deficiency of elastin in the dermis may lead to development of the cutaneous lesions of anetoderma.

Adult↗
PubMed2000

Multiple cutaneous immunocytoma with secondary anetoderma: a report of two cases.

We describe two men with multiple erythematous dermal nodules which were clinically and histologically consistent with a diagnosis of primary cutaneous immunocytoma. Both patients exhibited the very unusual feature of secondary anetoderma occurring in spontaneously resolving lesions. There is one previous report of anetoderma in association with a plasmacytoma. The pathogenesis remains unknown but release of cytokines such as interleukin-6 may be implicated.

Adult↗
PubMed1994

Secondary anetoderma involving a pilomatricoma.

We describe an 11-year-old girl with secondary anetoderma involving a pilomatricoma. She presented with a soft, wrinkled pedunculated lesion overlying a firm subcutaneous mass on her right anterior shoulder. Pathologic examination revealed a pilomatricoma in the subcutaneous tissue, with focal loss of elastic fibers in the overlying dermis. Secondary anetoderma has been reported to involve various infections, inflammatory disorders, and tumors, but the association with pilomatricoma is very rare.

Atrophy↗
PubMed1990

An immunofluorescence study of primary anetoderma.

Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early inflammatory and atrophic anetoderma lesions and from the exposed and unexposed uninvolved skin of each patient. The pattern of immune deposits observed in one patient was indistinguishable from that which is often seen in systemic LE, and in the other patient from that which may be observed in chronic cutaneous LE. The direct IF study also showed fibrillar immune deposits in the dermis that resembled elastic fibres morphologically. The indirect IF study, however, failed to demonstrate anti-elastic fibre antibodies in the patients' sera. The results of this study and a review of the literature suggest that some cases of PA have direct IF findings similar to those of either chronic cutaneous or systemic LE. However, these findings, along with the serological findings, are insufficient to establish a diagnosis of LE in most of these PA cases.

Adult↗
PubMed1994

Primary anetoderma: phagocytosis of elastic fibres by macrophages.

We report a 22-year-old female with a 2-year history of increasingly numerous well-circumscribed, asymptomatic skin lesions. The clinical and histological features were consistent with anetoderma and investigations failed to reveal any associated underlying disorders. Electron microscopy of abnormal skin showed phagocytosis of elastic fibres by macrophages. Although the aetiology of primary anetoderma remains unknown, this finding suggests that phagocytic destruction of elastic fibres plays a major role in its pathogenesis.

Adult↗
PubMedDate not supplied

Primary Anetoderma in children: report of two cases and literature review.

Two boys, age 7 and 9 years, with the diagnosis of primary anetoderma are presented. In one patient a growing number of indolent lesions developed for one year on the neck. The other boy complained of single lesions appearing over four months on the arms, feet, and chest wall. Individual lesions measured up to 1 cm in diameter and showed a palpable herniation phenomenon and wrinkled surface. The lesions did not have an inflammatory onset. Histologically, in both patients the diagnosis of anetoderma was verified by loss of elastic fibers and a lympho-histiocytic infiltration in the middermis. Administration of oral penicillin for three weeks did not result in marked improvement, and atrophic macules continued to appear in the younger boy. Although no autoimmunologic abnormalities or other associated diseases have arisen in our patients, long-term follow-up is mandatory to detect autoimmune disorders that are reported to occur in the course of the disease.

Child↗
PubMed2001

Anetoderma in a systemic lupus erythematosus patient with anti-PCNA and antiphospholipid antibodies.

Anetoderma is a rare elastolytic disorder included within the group of cutaneous atrophies. Its pathogenesis is not yet clearly established, but immunological mechanisms could play an important role in dermal elastolysis. It has been associated with different autoantibodies and autoimmune disorders. We present a case of anetoderma in a systemic lupus erythematosus patient with anti-proliferating-cell-nuclear-antigen and antiphospholipid antibodies, highlighting the peculiarities of such an association.

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↗
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↗