Prurigo pigmentosa as an atypical persistent plaque-like skin rash in adult-onset Still's disease

Case report and literature review

Asha S. Muthalaly, Joshua Bradish, Roy Sampson, Steven Hugenberg, Dennis C. Ang, Simon Warren, Agith Muthalaly

Research output: Contribution to journalArticle

Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disease of unclear etiology. It is classically characterized by spiking fevers, arthralgias, leukocytosis and a typical rash. Usually most of these clinical findings are not specific to this disease and it is often a diagnosis of exclusion. The exception to this is usually the typical maculopapular nonpruritic salmon-colored eruption, which has been shown to have high sensitivity and specificity for the diagnosis of patients with AOSD. It is important to be aware, however, that some patients also present with an atypical rash. Prurigo pigmentosa is a distinctive inflammatory disease first described in Japan. The patient in our case presentation was diagnosed with AOSD based on Yamaguchi criteria and also had a diffuse pruritic persistent hyperpigmented rash on the back, chest and distal extremities with a biopsy revealing histopathology and clinical findings consistent with the diagnosis of prurigo pigmentosa. This review of literature was carried out to examine all the known cases of persistent plaques associated with AOSD. The findings indicated that the histopathologic and clinical findings were similar to those found in prurigo pigmentosa. AOSD can be a chronic debilitating disease if left untreated and can ultimately lead to death. Prurigo pigmentosa may be an under-recognized atypical skin manifestation of AOSD that rheumatologists need to consider in clinical practice.

Original languageEnglish
Pages (from-to)521-529
Number of pages9
JournalInternational Journal of Clinical Rheumatology
Volume8
Issue number5
DOIs
StatePublished - Oct 2013

Fingerprint

Adult-Onset Still's Disease
Prurigo
Exanthema
Skin Manifestations
Salmon
Leukocytosis
Arthralgia
Japan
Chronic Disease
Fever
Thorax
Extremities
Biopsy
Sensitivity and Specificity

Keywords

  • adult-onset Still's disease
  • persistent plaques
  • prurigo pigmentosa
  • pruritic
  • salmon-colored

ASJC Scopus subject areas

  • Rheumatology

Cite this

Prurigo pigmentosa as an atypical persistent plaque-like skin rash in adult-onset Still's disease : Case report and literature review. / Muthalaly, Asha S.; Bradish, Joshua; Sampson, Roy; Hugenberg, Steven; Ang, Dennis C.; Warren, Simon; Muthalaly, Agith.

In: International Journal of Clinical Rheumatology, Vol. 8, No. 5, 10.2013, p. 521-529.

Research output: Contribution to journalArticle

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