Background/Purpose:

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder characterized by spiking fevers, polyarthritis and an evanescent salmon-pink maculopapular eruption. Several cases of atypical persistent skin eruption (APSE) have been reported in AOSD to date. APSE could be associated with AOSD poor prognosis and resistance to treatment. We report the second largest series of AOSD with accurate clinical and histological data.

Methods:

A multicenter retrospective study was conducted (6 french centers) including all patients with a diagnosis of AOSD (Yamaguchi and/or Fautrel criteria) with skin involvement and a skin biopsy between 2005 and 2017. Clinical, biological and histological data, delay of diagnosis and treatments used were recorded. A review of all APSE cases described in the literature was also performed.  

Results:

Thirty-six patients were included (median age: 46 years-old, female: 69%). Nineteen (53%) presented an APSE (Figure 1). Extracutaneous attempts included: arthralgia/arthritis (94%), lymphadenopathy (36%), hepato/splenomegaly (25%), pericarditis/pleurisy (11%). The following factors seemed to be increased in patients with APSE (n=19) in comparison with patients without APSE (n=17): time to diagnosis (p=0,1), arthritis (p=0.09), liver dysfunction (p=0,08), ferritin level higher than 1,500 ng/mL (p=0.018), number of line of treatments to achieve complete remission (p=0,08). Reactive hemophagocytic syndrome and associated malignancy were similar in both groups. Ninety-four cases of APSE in AOSD were previously reported, characterized by urticarial lesions (with a linear configuration in some cases), pigmented papules or plaques, lichenoid lesions or dermatomyositis-like lesions. Histology of APSE compared with evanescent salmon-pink maculopapular skin eruption displayed increased dermal inflammatory infiltrate and neutrophils, and increased epidermal alterations (dyskeratosis/necrotic keratinocytes, spongiosis).

Conclusion:

Cutaneous clinical and histological spectrum in AOSD is large and should not delay the diagnosis. These results have to be confirmed by large prospective cohorts.

Figure 1: A 58-year-old woman presenting a pruritic APSE (erythematous and pigmented papules and plaques of the cheeks and the leg) (A,B). Skin biopsy of the leg lesion revealed a moderate perivascular and interstitial dermal infiltrate composed of lymphocytes and neutrophils, a dermal oedema and vascular walls infiltrate (C)