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Pathology of Acrodermatitis Chronica Atrophicans

Dr Sampurna Roy MD               

 

Dermatopathology Quiz Case 102

Diagnosis: Acrodermatitis Chronica Atrophicans

 

                                                                                                                      

 

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

It is a chronic manifestation of infection by Borrelia burgdorferi.

Borrelia afzelli is the predominant etiological agent.

Clinical presentation: 

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:  Acrodermatitis chronica atrophicans 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.

Visit: Lyme Disease (Lyme Borreliosis) Erythema chronicum migrans

 

Further reading:

Lyme disease acrodermitis chronica atrophicans: Misleading vascular signs.

Acrodermatitis Chronica Atrophicans With Pseudo lymphomatous Infiltrates

Borrelia burgdorferi sensu lato from blood of adult patients with borrelial lymphocytoma, Lyme neuroborreliosis, Lyme arthritis and acrodermatitis chronica atrophicans

Acrodermatitis chronica atrophicans. 

Acrodermatitis Chronica Atrophicans: A Case Report and Review of the Literature

Acrodermatitis chronica atrophicans in a 15-year-old girl misdiagnosed as venous insufficiency for 6 years.

 

 

 

 


 

 


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