Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult‐onset and juvenile Still's disease

Fortna, Ryan R.; Guðjónsson, Jóhann E.; Seidel, Gregory D.; DiCostanzo, Damian; Jacobson, Mark; Kopelman, Margaret B.; Patel, Rajiv M.

doi:10.1111/j.1600-0560.2010.01570.x

Cited by 48 publications

(66 citation statements)

References 9 publications

(25 reference statements)

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“…9 This nonclassic variant also has unique histopathologic features including dyskeratosis in the upper layers of the epidermis extending into the stratum corneum without involvement of the basal layer and a superficial dermal infiltrate with neutrophils and lymphocytes. [4][5][6][7][8] While this nontypical variant was first described by Kaur et al 4 in 1994, it may be an underrecognized cutaneous manifestation of AOSD. In the largest case series of persistent pruritic papules and plaques published to date, Lee et al 6 found that 65% (11 of 17) of their patients with AOSD had this variant.…”

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confidence: 99%

“…This variant manifests as nonevanescent pruritic erythematous papules and plaques with slight scale that often presents with a flagellate erythemalike presentation with linear configurations on the trunk and extremities. [4][5][6][7][8] The linearity may represent a Koebner phenomenon or scratching because the eruption characteristically spares the mid-back. Differential diagnosis of flagellate erythema includes bleomycin, dermatomyositis, and shiitake mushroom dermatitis.…”

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confidence: 99%

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Flagellate Erythema in a Patient With Fever

2013

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A woman in her 40s presented with a 6-month history of intermittent fevers, malaise, polyarthralgias, sore throat, and a pruritic exanthem on her trunk, scalp, and extremities. A previous biopsy was nonspecific. She had no significant medical history.Physical examination revealed erythematous, mildly scaly plaques in a shawl distribution ( Figure, A) and hyperpigmented, erythematous, excoriated papules in a linear arrangement on her back (Figure, B) and lower extremities. Laboratory studies showed leukocytosis (27 900/uL, 95% neutrophils) and increased levels of C-reactive protein (344 mg/L) and ferritin (63 000 ng/mL). Antinuclear antibody titer was 1:80. Findings for rheumatoid factor and antineutrophil cytoplasmic antibodies were negative, as were those for Lyme disease, rickettsial disease, cytomegalovirus, Bartonella, rapid plasma reagin, human immunodeficiency virus, and hepatitis. Serum and urine protein electrophoresis findings were also negative, and chest, abdomen, and pelvic computed tomography showed nonspecific hilar lymphadenopathy. A punch biopsy specimen from the back was obtained (Figure, C).

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Flagellate Erythema in a Patient With Fever

2013

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“…3, 4, 5). Recently, several papers show the unique histological features in the lesional skin of persistent pruritic papules and plaques [17][18][19][20][21][22][23][24][25][26]. Histopathology presents a characteristic pattern of dyskeratosis with a peculiar, distinctive distribution in (Fig.…”

Section: Persistent Pruritic Papules and Plaquesmentioning

confidence: 99%

“…Histopathology presents a characteristic pattern of dyskeratosis with a peculiar, distinctive distribution in (Fig. 6) [22][23][24][25]. A sparse cellular inWltrates containing neutrophils are seen in the upper dermis, but without vasculitis.…”

Section: Persistent Pruritic Papules and Plaquesmentioning

confidence: 99%

Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values

Yamamoto

2011

Rheumatol Int

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Adult-onset Still's disease (AOSD) is a systemic inflammatory condition, characterized by a high spiking fever, leukocytosis with neutrophilia, arthralgia, and skin rash. Typical skin rash is an evanescent, salmon-pink erythema predominantly involving extremities, which is included as one of the diagnostic criteria; however, recent findings show that not only typical evanescent rash but also various skin lesions are associated with AOSD. The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques, which presents erythematous, slightly scaly papules with linear configuration on the trunk. Interestingly, persistent pruritic papules and plaques show unique histological features such as peculiar, distinctive distribution of dyskeratotic keratinocytes in the cornified layers as well as in the epidermis. Other non-classical skin lesions include urticaria. Current insights suggest that several inflammatory cytokines such as interleukin-1 (IL-1), IL-6, IL-18, interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) play a pathogenic role in AOSD. In particular, IL-18 is suggested to play a crucial role in activating macrophages, favoring Th1 type cytokine production. IL-18 induces IFN-γ, IL-17, and TNF-α, which may play an important pathogenic role in AOSD. It is important to recognize the common and/or uncommon skin conditions of AOSD for early correct diagnosis. In this review, various skin lesions are introduced, and the complication with histiocytic necrotizing lymphadenitis (Kikuchi disease) is further discussed.

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“…Further non-specific laboratory findings are inflammation-induced anaemia, leukocytosis and thrombocytosis [19]. Histopathology from lesional skin of urticarial autoinflammatory diseases like CAPS and sJIA reveals neutrophil-rich dermal infiltrates [37][38][39].…”

Section: Laboratory Findingsmentioning

confidence: 99%