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Acrodermatitis chronica atrophicans, the characteristic cutaneous manifestation of late Lyme borreliosis, typically occurs in elderly women.

Visit: Lyme Disease (Lyme Borreliosis) ; Erythema chronicum migrans

It is a chronic manifestation of infection by Borrelia burgdorferi . B. afzelli is the predominant etiological agent.

Clinical presentation: Image Link   Early inflammatory stage is characterized by diffuse or localized erythema. It gradually spreads on the extensor surfaces of the extremities and areas around joints. Later there is gradual atrophy of the skin with loss of appendages. Sclerodermatous patches and linear fibrotic bands may be present over ulna and tibia.

Histological features: Early stages of the disease show superficial and deep inflammatory cellular infiltrate in the dermis. The moderately dense infiltrate is composed of lymphocytes and some histiocytes and plasma cells. Prominent vascular channels together with telangiectasia may be present.

In some cases there is a superficial band-like infiltrate of inflammatory cells with subepithelial thin zone of collagen.

Vacuoles are sometimes noted in the upper dermis.

As the disease progresses there is atrophy of the dermis together with loss of elastic fibers and pilosebaceous follicles and atrophy of subcutis.

Other changes include epidermal atrophy and loss of rete ridges, features resembling lichen sclerosus et atrophicus.

In the sclerodermatous patches are characterized by dense dermal sclerosis.

This process can be told to be active by the density of the lymphoplasmacytic infiltrate and can be considered to be long standing by the extent of fibroplasia, thick collagen bundles being aligned mostly parallel to the skin surface, sclerosis being evident in the deep reticular dermis, and telangiectases being prominent in the upper part of the dermis.

Juxtra-articular fibrous nodules are characterized by broad bundles of homogeneous collagen in the upper subcutis. Perivascular and interstitial inflammatory infiltrate is present.

Differential diagnosis: Morphea:  ACA shows atrophy of collagen and elastic tissue as well as hypertrophic basophilic elastic tissue; whereas in morphea, sclerosis and polarizing elastic tissue are prominent. Graft-versus-host-like reactions may be present in both dermatoses.

                     

Abstracts:

Acrodermatitis chronica atrophicans in a 15-year-old girl misdiagnosed as venous insufficiency for 6 years.J Am Acad Dermatol. 2005 Jun;52(6):1091-4.

Isolation of Borrelia burgdorferi sensu lato from a fibrous nodule in a patient with acrodermatitis chronica atrophicans.Wien Klin Wochenschr. 2002 Jul 31;114(13-14):533-4

Acrodermatitis chronica atrophicans in the course of old Lyme disease.Pol Merkur Lekarski. 2001 Sep;11(63):263-5

Juxta-articular fibroid nodules and acrodermatitis chronica atrophicans in late stage Lyme borreliosis.Hautarzt. 2000 May;51(5):345-8

Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases.Acta Derm Venereol. 1998 May;78(3):207-13

Acrodermatitis chronic atrophicans: a chronic T-cell-mediated immune reaction against Borrelia burgdorferi? Clinical, histologic, and immunohistochemical study of five cases.J Am Acad Dermatol. 1993 Mar;28(3):399-405

Lyme disease: the evolution of erythema chronicum migrans into acrodermatitis chronica atrophicans.Cutis. 1993 Sep;52(3):169-70

A clinical, histological, and immunohistochemical comparison of acrodermatitis chronica atrophicans and morphea.Am J Dermatopathol. 1991 Aug;13(4):334-41

               

 
September 2009

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