Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
Atypical Persistent Plaquelike Skin Rash of Adult Onset Still's Disease. Case Report and Review of Literature.
Muthalaly1, Asha, Ang2, Dennis C., Hugenberg3, Steven T., Sampson1, Roy, Muthalaly4, Agith
25 y/o Malaysian woman with no past medical history presented to the hospital with a 2 month history of fevers, chills, night sweats, diffuse myalgia, lethargy and sore throat. She also had a diffuse pruritic persistent hyper pigmented rash on back, chest, distal extremities. The patient was diagnosed with adult-onset Still's disease (AOSD) based on Yamaguchi criteria and skin biopsy revealed spongiotic psoriasiform dermatitis with scattered necrotic keratinocytes and confluent hyperkeratosis. Our patient met criteria for diagnosis of prurigo pigmentosa based on clinical and histopathologic findings. There have only been two other reported cases. However, when a review of literature was performed of all known cases of persistent plaques associated with AOSD, it was found that prurigo pigmentosa may be associated more commonly with AOSD than previously thought.
Meta analysis of 10 articles reviewed (total of 21 patients with AOSD) with persistent papules and plaques
The histopathologic findings reported are commonly found in different stages of prurigo pigmentosa. See attached table for details.
The typical salmon colored evanescent rash is observed in 87% of patients in AOSD with histopathology showing relatively sparse perivascular mixed inflammatory infiltrate containing some neutrophils. Prurigo pigmentosa is a distinctive inflammatory disease first described in Japan. Clues to diagnosis include impression of trunk centered "scratched urticaria" Usually involves back, chest and neck and may also involve proximal extremities and upper extremities. It is rarely diagnosed outside of Japan because of its unfamiliarity. Various mechanisms proposed have been friction, contact allergy, sensitivity to sunlight, endocrine disorders such as diabetes, and metabolic disorders such as ketosis. Early lesions have superficial infiltrate of neutrophils and spongiosis with a few necrotic keratinocytes. Fully developed lesions have lymphocytic predominate infiltrate. In late lesions the epidermis is slightly hyperplastic and parakaratotic. Pruritis of early lesions is severe, however resolving lesions are devoid of symptoms. The pathologic skin diagnosis of the cases reviewed was unclear in the majority of cases. Prurigo pigmentosa may be an unusual yet more common, but under recognized skin manifestation of AOSD of which rheumatologists need to be aware in clinical practice.
To cite this abstract, please use the following information:
Muthalaly, Asha, Ang, Dennis C., Hugenberg, Steven T., Sampson, Roy, Muthalaly, Agith; Atypical Persistent Plaquelike Skin Rash of Adult Onset Still's Disease. Case Report and Review of Literature. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1962