Anetoderma before development of antiphospholipid antibodies: delayed development and monitoring of antiphospholipid antibodies in an SLE patient presenting with anetoderma.

INTRODUCTION: Anetoderma is an elastolytic skindisorder that has been associated with the presenceof antiphospholipid antibodies (aPL). Patients withantiphospholipid antibody-positive anetoderma havebeen reported to develop symptoms of Graves disease,antiphospholipid syndrome, and other autoimmuneconditions. The temporal relationship, however,between anetoderma onset and the emergence ofaPL remains unclear, a clarification of which may haveimplications for the screening and monitoring ofpatients with anetoderma. CASE: Herein we report acase of a patient with systemic lupus erythematosuspresenting with anetoderma that preceded thedevelopment of aPL. The patient was found to havesubsequently developed IgM cardiolipin antibodiesat a serology follow-up approximately two years later.Conclusion and Relevance: This finding suggests thatanetoderma can precede aPL seroconversion andthat patients with anetoderma may require continuedserology monitoring. Such long-term monitoring willbe important for identifying laboratory indicationsthat may portend the development of furtherautoimmune symptoms associated with anetoderma.

A Clinicoimmunohistopathologic Study of Anetoderma: Is Protruding Type More Advanced in Stage Than Indented Type?

Background. The clinical and histopathologic classification of anetoderma are not well characterized. Objective. We aimed to investigate the clinical and histopathologic characteristics of anetoderma and to correlate clinical phenotypes with immunohistopathologic findings. Methods. We retrospectively reviewed the medical records of 30 patients with anetoderma and performed immunohistochemistry for elastin, fibrillin-1, metalloproteinase- (MMP-) 2, MMP-7, MMP-9, and MMP-12, and tissue inhibitor of metalloproteinase- (TIMP-) 1 and TIMP-2. Results. Protruding type (n = 17) had a longer disease duration and more severe loss of elastin, without changes in fibrillin, than indented type (n = 13). MMP-2 and MMP-9 showed significantly higher expressions in the dermis compared with controls (p < 0.05). MMP-7 and MMP-12 showed little expressions in both anetoderma and control tissue. TIMP-1 was highly expressed in anetoderma lesions and controls. TIMP-2 expression was variable. Conclusions. Our findings suggest that protruding type anetoderma may represent a more advanced stage and that MMP-2 and MMP-9 could be responsible for elastic fiber degradation in anetoderma.

[Dermatologic manifestations of the antiphospholipid syndrome]. / Manifestations dermatologiques du syndrome des antiphospholipides.

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies.

Anetoderma secondary to antiphospholipid antibodies.

Anetoderma is an elastolytic disorder that is associated with a number of infectious and autoimmune disorders. We present a case of a patient with generalized anetoderma, who was later found to have positive antinuclear antibodies and antiphospholipid antibodies (APAs). Numerous other cases have been reported in literature and some authors have suggested that anetoderma is a highly specific sign of APAs, with or without other manifestations of systemic lupus erythematosus or antiphospholipid syndrome [14]. Thus, work up for connective-tissue disorders should be considered in any patients who present with this skin finding.

[Anetoderma with positive Borrelia serology]. / Anetodermie bei positiver Borrelienserologie.

Anetoderma is an uncommon disease characterized by multiple circumscribed atrophic, herniated skin lesions on trunk, thighs and upper arms caused by loss of elastic fibers. Associations with autoimmune diseases or infections, especially spirochetal infections, have been described. We report a case of anetoderma with an increased serum Borrelia burgdorferi IgM-titers. After treatment with doxycycline 200 mg/day for three weeks, the progression of the disease stopped and no new lesions appeared.

Primary anetoderma: a cutaneous marker of antiphospholipid antibodies.

Primary anetoderma is a rare idiopathic disease of the skin characterized by circumscribed areas of slack skin and loss of elastic fibers found on histopathologic examination. It has been related to systemic lupus erythematosus and other immune diseases. In recent years, however, its association with antiphospholipid antibodies has been highlighted, and it should be considered a clinical manifestation of these antibodies.

Anetoderma: evidence of the relationship with autoimmune disease and a possible role of macrophages in the etiopathogenesis.

Anetoderma is a benign condition characterized by round or oval macular lesions with focal loss of dermal elastic tissue resulting in localized areas of flaccid or herniated saclike skin. Often, the anetoderma is associated with immuno-mediated pathogenetic mechanism. In this article, we describe the association between anetoderma and autoimmune diseases, by underlining the role and the action of macrophages as a possible etiopathogenesis.

Anetoderma in cutaneous marginal-zone B-cell lymphoma.

Anetoderma is a rare condition, consisting of well-circumscribed areas of slack skin, in which dermal elastic fibres are destroyed or deficient. We present the case of a 45-year-old man with a 25-year history of deep nodules and plaques gradually progressing to areas of anetoderma. Histological examination found an infiltrate composed of neoplastic cells with lymphoplasmocytoid morphology. The cells were positive for CD20, CD38 and CD138, and there was a monoclonal kappa light chain gene rearrangement of plasma cells. A diagnosis of cutaneous marginal-zone B-cell lymphoma was made. The pathogenesis of anetoderma remains unknown, but it is possible that cytokines or other soluble factors produced by the infiltrating lymphocytes have a role in this process.