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Pathology of Erythema Elevatum Diutinum

Dr Sampurna Roy MD                    

Dermatopathology Quiz Case 190

Diagnosis: Erythema elevatum diutinum

 

                                                                                                                      

 

 

Erythema elevatum diutinum was first described by Hutchinson in 1878.

It was later classified as chronic leukocytoclastic vasculitis in 1929.

Usually these lesions are asymptomatic. In some cases patients may have pain or pruritus.

Gross appearance: This rare dermatosis is characterized by persistent red, brownish-purple, and yellow papules, nodules or plaques.

The lesions are initially soft but later becomes firm.

In some cases vesicles and bullae have been noted on the nodules and plaques.

Site: The lesions are located in symmetric distribution on the extensor surfaces of the extremities, on the elbows and knees, dorsal aspect of hands and feet and also on the buttocks.

Age and sex: The disease occurs equally in both men and women.

It is primarily noted in the middle aged patients (30 - 60 years).

Other associated systemic diseases: Erythema elevatum diutinum may be associated with celiac disease, ulcerative colitis, Crohn's disease, arthralgia, rheumatoid arthritis, myelodysplastic syndrome, HIV and HHV-6 infections.

Microscopic features:

Early stage:

There is a dense predominantly perivascular infiltrate composed largely of neutrophils. 

A few lymphocytes, histiocytes and eosinophils may be present.

There is widespread vasculitis and fibrin deposits (toxic hyalin) are noted within and around the walls of the small dermal vessel together with endothelial swelling.

Some neutrophils show fragmentation of nuclei (nuclear dust).

Extravasation of red blood cells is rare.

More advanced stage:

The neutrophilic infiltrate involves the entire dermis.

This infiltrate is not present within the epidermis.

Epidermal spongiosis and subepidermal bullous lesions may be present.

Late stage:

There is granulation tissue formation and prominent fibrosis.

There are fascicles of spindle cells resembling dermatofibroma.

The cellular infiltrate (mainly neutrophils) is less prominent. 

There is capillary proliferation and small foci of neutrophilic vasculitis is present in a fibrous backround.

Differential diagnosis:

This lesions should be clinically differentiated from other neutrophilic dermatoses which include inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes.

The main clinical forms of neutrophilic dermatoses include Sweet syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, rheumatoid neutrophilic dermatosis, and Behcet's disease. 

Clinically, old lesions may be mistaken by tuberous xanthoma, annular granuloma, rheumatoid nodules and multicentric reticulohistiocytosis.

Clinicopathological correlation plays an important role in establishing the diagnosis.

So you think you know all about Granuloma Faciale? Answer these 8 questions.

                                                                                               

Further reading:

Erythema elevatum diutinum: skin and systemic manifestations, immunologic studies, and successful treatment with dapsone.

Erythema elevatum diutinum: a clinicopathological study

Erythema elevatum diutinum

Erythema elevatum diutinum

Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus

Erythema elevatum diutinum in a patient with Crohn's disease

The evolution of lesions in erythema elevatum diutinum.

Erythema elevatum diutinum in a patient with relapsing polychondritis

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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