Exfoliating erythroderma

Exfoliative erythroderma is a rare seborrhoeic condition characterized by profuse exfoliation and inflammation of the skin in infants. It is very rare and mainly affects infants between 1 and 3 months of age.

The causes of exfoliative erythroderma

It is believed that exfoliative erythroderma is an inherited autosomal dominant defect of the C5 complement component leading to disorders of opsonization. Currently, we are talking about the Leiner phenotype characterized by a wide spectrum of immune deficiencies in infants, which include:

  1. hipogammaglobulinemia,
  2. hypergammaglobulinemia E syndrome,
  3. severely complex immune deficiencies,
  4. many types of complement deficiencies (C3 and C5) leading to opsonization disorders.

Exfoliating erythroderma – symptoms

It appears in the course of the disease generalized skin inflammation and profuse exfoliation, all skin of the baby. Within the hairy skin and on the face, there is a significant build-up of seborrheic scabs. The exudative symptoms are slight and concern only the articular flexions, and the nails show dystrophic changes. In addition, the lymph nodes are reactively enlarged.

Diagnostics of exfoliative erythroderma

Patients have skin inflammation with profuse hyperplasia of the stratum corneum. Diagnostic methods are used to determine the level of proteins in the blood and the concentration of immunoglobulin E, the complement components. In addition, exfoliating erythroderma should be differentiated from:

  1. seborrheic dermatitis in infants,
  2. atopic eczema,
  3. inborn ichthyosis erythroderma.

What are the treatments for exfoliative erythroderma?

Treatment of ailments consists of the use of antibiotics, general tonic and sometimes steroids. In turn, in local treatment, it is recommended to use neutral or weakly keratolytic ointments.

What’s the prognosis?

Generally good.

Lit .: [1] Braun-Falco O., Plewig G., Wolff HH, Burgdorf WHC: Dermatology, eds. half. Gliński W., Wolska H., Wydawnictwo Czelej, Lublin 2002.

Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House

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