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Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.

Michailidou D, Shin J, Forde I, Gopalratnam K, Cohen P, DeGirolamo A - Auto Immun Highlights (2015)

Bottom Line: Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis.AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease.Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA. de.michailidou@gmail.com.

ABSTRACT
Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.

No MeSH data available.


Related in: MedlinePlus

Atypical persistent rash: persistent pigmented plaque V shaped on the anterior chest extending down the midline to the umbilicus
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Fig3: Atypical persistent rash: persistent pigmented plaque V shaped on the anterior chest extending down the midline to the umbilicus

Mentions: On admission, his temperature was 39 °C and a persistent pigmented plaque V shaped was evident on his anterior chest extending down the midline to the umbilicus (Fig. 3). Further skin examination revealed a confluent salmon-pink papular eruption on his lower back area and a persistent pigmented plaque on the upper area of his back. Besides mild splenomegaly and a tender right wrist, left second and third proximal interphalangeal joints with no signs of swelling or erythema, the rest of the physical exam was unremarkable. Laboratory profile revealed severe neutrophilic leukocytosis (30,000, normal values 4800–10,800/mcL), and a highly elevated serum ferritin levels (>10,000, normal values 17.90–464.00 ng/mL). Autoantibodies (including ANA, ANCA, RF and anti-CCP) were negative. Blood cultures excluded common viral and bacterial infections and RPR were negative. Serological tests for Hepatitis B and C, HIV, Epstein-Barr and Cytomegalovirus were negative. Borrelia burgdorferi, Bartonella henselae, Rickettsia typhi, RMSF, Typhus and Parvovirus B-19 serologies were negative as well. Parasites for malaria or Babesia microti were undetectable on peripheral blood smear. Transthoracic echocardiogram was negative for vegetations and computed tomography (CT) of the neck, chest and abdomen revealed only borderline mild splenomegaly.Fig. 3


Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.

Michailidou D, Shin J, Forde I, Gopalratnam K, Cohen P, DeGirolamo A - Auto Immun Highlights (2015)

Atypical persistent rash: persistent pigmented plaque V shaped on the anterior chest extending down the midline to the umbilicus
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4633415&req=5

Fig3: Atypical persistent rash: persistent pigmented plaque V shaped on the anterior chest extending down the midline to the umbilicus
Mentions: On admission, his temperature was 39 °C and a persistent pigmented plaque V shaped was evident on his anterior chest extending down the midline to the umbilicus (Fig. 3). Further skin examination revealed a confluent salmon-pink papular eruption on his lower back area and a persistent pigmented plaque on the upper area of his back. Besides mild splenomegaly and a tender right wrist, left second and third proximal interphalangeal joints with no signs of swelling or erythema, the rest of the physical exam was unremarkable. Laboratory profile revealed severe neutrophilic leukocytosis (30,000, normal values 4800–10,800/mcL), and a highly elevated serum ferritin levels (>10,000, normal values 17.90–464.00 ng/mL). Autoantibodies (including ANA, ANCA, RF and anti-CCP) were negative. Blood cultures excluded common viral and bacterial infections and RPR were negative. Serological tests for Hepatitis B and C, HIV, Epstein-Barr and Cytomegalovirus were negative. Borrelia burgdorferi, Bartonella henselae, Rickettsia typhi, RMSF, Typhus and Parvovirus B-19 serologies were negative as well. Parasites for malaria or Babesia microti were undetectable on peripheral blood smear. Transthoracic echocardiogram was negative for vegetations and computed tomography (CT) of the neck, chest and abdomen revealed only borderline mild splenomegaly.Fig. 3

Bottom Line: Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis.AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease.Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA. de.michailidou@gmail.com.

ABSTRACT
Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.

No MeSH data available.


Related in: MedlinePlus