Adult onset Still's disease with persistent skin lesions complicated by secondary hemophagocytic lymphohistiocytosis

Sarkar, Rathindranath; Bhattacharya, Raja; Bhattacharyya, Kallol; Paul, Rudrajit; Mullick, Omar Sharif

doi:10.1111/1756-185x.12170

Cited by 13 publications

(16 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, in recent years, other atypical cutaneous manifestations of AOSD have been reported; these rare presentations are not routinely recognized [6–38] Atypical cutaneous features often present in addition to the typical evanescent rash, but in 43% of the cases they are the only skin manifestation. [6,8,11,14,15,17,25,28–33,37,38] . A delayed diagnosis in these cases is still common, as the cutaneous lesions are often misdiagnosed as an allergic reaction to drugs, usually NSAIDs prescribed for joint symptoms or fever.…”

Section: Discussionmentioning

confidence: 99%

“…Rarely, cutaneous lesions were the presenting feature of AOSD preceding the appearance of the classic symptoms of the disease [9,13,23,29,30] or appeared during a flare several months after the diagnosis. [17,26,37] …”

Section: Discussionmentioning

confidence: 99%

“…Other nonclassical skin lesions less frequently observed include urticaria and urticaria-like eruptions, [28–34] generalized or widespread non-pruritic persistent erythema, [18,35–37] vesiculopustular eruptions, [38,39] a widespread peau d’orange appearance of the skin (cutaneous mucinosa), [40] and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. [18] In cases of urticaria/urticaria-like eruptions and generalized (widespread) nonpruritic persistent erythema, biopsy specimens reveal a nonspecific slight to moderate inflammatory infiltrate in the upper dermis, similar to that observed in biopsies of the typical evanescent skin rash.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Adult-onset Still's disease with atypical cutaneous manifestations

García¹,

Pascual²,

Recalde³

et al. 2017

Medicine

View full text Add to dashboard Cite

The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition.In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis.The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome.The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion.The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy with immunosuppressant drugs and/or biologic agents (mainly anakinra or tocilizumab) to control or manage symptoms because of a polycyclic or chronic course. The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis, with a mortality rate reaching 8% primarily because of infectious complications related to immunosuppressive therapy.In conclusion, the appearance of atypical cutaneous manifestations is not uncommon in AOSD. Recognition of this clinical variant is crucial for the early diagnosis of AOSD, as it might imply persistent disease activity and the need for more aggressive treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Adult-onset Still's disease with atypical cutaneous manifestations

García¹,

Pascual²,

Recalde³

et al. 2017

Medicine

View full text Add to dashboard Cite

show abstract

“…The ferritin level of our patient decreased to 5.654 ng/mL with corticosteroid therapy. Elevated serum ferritin levels (>10.000 mg/L) was reported as more suggestive of HS complicating AOSD, and associated with hemophagocytosis syndrome (8). MAS, AOSD, septic shock and catastrophic antiphospholipid syndrome are associated with very high ferritin levels.…”

Section: Discussionmentioning

confidence: 99%

“…It was proposed these four disorders under a single nomenclature as 'The Hyperferritinemic Syndrome' due to similar pathogenic mechanisms, clinical, laboratory presentations and treatment modalities (5). HS secondary to AOSD was reported rarely (8)(9)(10)(11). IL-18 may play an important role for both AOSD and HS.…”

Section: Discussionmentioning

confidence: 99%

Extremely high serum ferritin level in a patient with Hemophagocytic Syndrome and Adult-Onset Still's disease

et al. 2017

View full text Add to dashboard Cite

Hemophagocytic syndrome (HS) and Adult-onset Still's disease (AOSD) are both rarely systemic inflammatory disorders. It may be difficult to differentiate these inflammatory disorders from each other. There are some signs that may help clinicians in the diagnosis such as high ferritin levels for AOSD, and leukopenia, thrombocytopenia, hypertriglyceridemia for HS. Hemophagocytic syndrome secondary to Adult-onset Still's disease was reported rarely in literature. In this article, we aimed to report a patient of 65-year old female diagnosed with HS and AOSD with extremely high serum ferritin levels.

show abstract

Cutaneous manifestations of adult-onset Still’s disease: a case report and review of literature

et al. 2014

View full text Add to dashboard Cite

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, serositis, and transient cutaneous manifestations. Although more common in children, cases are seen also in adults. Cutaneous involvement is common and may be suggestive for the diagnosis. A case of AOSD in a 35-year-old man is reported here, presenting with urticarial maculopapular rash of trunk, high spiking fever, acute respiratory distress syndrome, and myopericarditis. Skin biopsy showed interstitial and perivascular mature CD15(+) neutrophils. A comprehensive review of literature showed that cutaneous involvement occurs in about 80 % of patients, with various clinical presentations. The most common skin manifestation is an evanescent salmon pink or erythematous maculopapular exanthema, predominantly on the trunk and proximal limbs, with rare involvement of face and distal limbs. Less common manifestations include persistent erythematous plaques and pustular lesions. A constant histopathologic finding is the presence of interstitial dermal neutrophils aligned between the collagen bundles. This pattern may provide an easy accessible clue for the definitive diagnosis of AOSD and exclude other diagnosis such as drug eruptions or infectious diseases.

show abstract

Adult onset Still's disease with persistent skin lesions complicated by secondary hemophagocytic lymphohistiocytosis

Cited by 13 publications

References 6 publications

Adult-onset Still's disease with atypical cutaneous manifestations

Adult-onset Still's disease with atypical cutaneous manifestations

Extremely high serum ferritin level in a patient with Hemophagocytic Syndrome and Adult-Onset Still's disease

Cutaneous manifestations of adult-onset Still’s disease: a case report and review of literature

Contact Info

Product

Resources

About