Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment

Elizabeth Alvarez Connelly, Cheryl Aber, Gary Kleiner, Carlos Nousari, Carlos Charles, Lawrence A Schachner

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Pemphigus foliaceus is an autoimmune disease that clinically manifests with cutaneous blisters of the superficial skin. The nonendemic or sporadic form of this entity is rare in children and typically presents with a milder, more localized rash that usually follows a benign course of short duration. We describe an affected patient atypical in both her young age and the severity of skin findings. Our patient presented with a full body exfoliative erythroderma at 21 months of age. After an extensive work-up to determine the etiology of her exfoliative erythroderma, direct and indirect immunofluorescence studies confirmed the diagnosis of pemphigus foliaceus. Rituximab therapy was initiated based on the patient's refractory disease course to multiple immunosuppressive agents. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. The patient's skin exhibited marked clinical improvement after the start of rituximab infusions over 12 weeks. Her initial desmoglein 1 antibody level was greater than 1:1280, which decreased to 1:16 after seven rituximab treatments. She has had no skin flares since initiating treatment with rituximab therapy. Based on this clinical and serologic response, the use of rituximab may be helpful in the treatment of pediatric pemphigus foliaceus refractory to mainstays of therapy.

Original languageEnglish
Pages (from-to)172-176
Number of pages5
JournalPediatric Dermatology
Volume24
Issue number2
DOIs
StatePublished - Mar 1 2007

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Pemphigus
Skin
Exfoliative Dermatitis
Therapeutics
Desmoglein 1
Direct Fluorescent Antibody Technique
B-Lymphoid Precursor Cells
Blister
Immunosuppressive Agents
Indirect Fluorescent Antibody Technique
Rituximab
Exanthema
Autoimmune Diseases
Membrane Proteins
B-Lymphocytes
Monoclonal Antibodies
Pediatrics
Antibodies

ASJC Scopus subject areas

  • Dermatology
  • Pediatrics, Perinatology, and Child Health

Cite this

Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. / Connelly, Elizabeth Alvarez; Aber, Cheryl; Kleiner, Gary; Nousari, Carlos; Charles, Carlos; Schachner, Lawrence A.

In: Pediatric Dermatology, Vol. 24, No. 2, 01.03.2007, p. 172-176.

Research output: Contribution to journalArticle

Connelly, Elizabeth Alvarez ; Aber, Cheryl ; Kleiner, Gary ; Nousari, Carlos ; Charles, Carlos ; Schachner, Lawrence A. / Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. In: Pediatric Dermatology. 2007 ; Vol. 24, No. 2. pp. 172-176.
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