Clinical Dermatology

Diagnostic Hallmarks

1. Distribution: 70% of the skin surface area is involved
2. History of a preceding, localized skin disease in about 50% of patients

Clinical Presentation

Exfoliative erythrodermatitis is not a single disease but rather is an extreme example of an eczematous reaction pattern. The term “exfoliative erythrodermatitis” is used when eczematous disease covers 70% or more of the total body surface. Itching is usually severe, and as result, excoriation is notable. The extensiveness of the process is reflected by the accompanying presence of many systemic complications .

Exfoliative erythrodermatitis can occur in either a primary or a secondary form. The primary form consists of the de novo development of lesions in a patient with no past history of skin disease. The secondary form arises as the result of widespread extension from an already-existent skin disease.

Exfoliative erythrodermatitis of the primary type may develop as an idiopathic condition, as a medication reaction, or as a manifestation of malignant lymphoma. The two medications most often responsible for the development of exfoliative erythrodermatitis are gold and pheuytoiu (Dilantin).

Among the lymphomas, exfoliative erthrodermatitis is most commonly seen in association wilh fungoides, Sezary syndrome, and Hodgkin’s disease. Whell il occurs with mycosis fungoides and Sezary syndromemalignant T cells are found in biopsies from the involved skill, wherr as when it occurs with other lymphomas, such Hodgkin’s disease, no alignant cells are fciund on cutaneous biopsy. In this latter setting the eruption is presumably induced by scratching in response to the pruritus that accompanies the primary lymphoma.

Exfoliative erythrodermatitis of the secondary type occurs is an extension of either an eczematous or a papulosquamous disease. Most commonly, it develops secondary to atopic dermatitis or neurodermatitis . It can also occur during the course of psoriasis (particularly in its pustular or crythrodermic form) and pityriasis rubra pilaris.

The transition of psoriasis or pityriasis rubra pilaris toward exfoliative erythrodermatitis is, not unexpectedly, accompanied by change in lesional morphology. Plaques lose their sharp margination, and evidence of epithelial disruption (weeping and crusting) evolves. Pruritus, not notable in classic psoriasis and pityriasis rubra pilaris, develops and often leads to notable excoriation.

Course and Prognosis

The course of exfoliative erythrodermatitis is chronic, often with a tendency for continued worsening until effective therapy can be instituted. The ultimate outcome is, of course, dependent on the nature of the underlying problem. Patients whose disease is secondly to atopic dermatitis and psoriasis can often be cleared completely, whereas patients whose exfoliative erythrodermatitis is secondary to lymphoma often have a course that progresses unrelentingly until the patient dies from his or her hematopoietic disease.

Exfoliative erythrodermatitis, regardless of cause, is often complicated by anemia, leukocytosis, hyperuricemia, hypoalbuminemia, and problems with temperature regulation. Moreover, the vasodilation that accompanies the cutaneous inflammation occasionally leads to cardiac failure of the high-output type.

Pathogenesis

The pathogenetic mechanisms responsible for the conversion of any localized skin disease to that of exfoliative erythrodermatitis are not well understood. It is convenient, however, to think of the process as an immunologic reaction involving widespread extension of autoeczematization .

Therapy

Therapy of exfoliative erythrodermatitis is quite difficult. Patients are terribly uncomfortable, and most experience shaking, chills, and considerable malaise and toxicity. Because of this, hospitalization is usually necessary. The clinical picture often suggests the possibility of bacterial sepsis, but special care should be taken in interpreting positive blood cultures if they have been obtained by needle puncture through eczematized skin.

Soaks and topical steroids are almost always desirable, and any associated cutaneous infection is treated with appropriate antibiotics. Most patients will also require the use of systemically administered steroids. Obviously, therapy for the underlying disease (if one can be identified) is paramount.

This entry was posted on Tuesday, January 1st, 2008 at 7:34 am and is filed under Eczematous Diseases. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.