Dermatopathology
Histopathology of persistent papules and plaques in adult-onset Still's disease

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Background

Persistent plaques and linear pigmentation have been reported as specific skin lesions in some patients with adult-onset Still's disease (AOSD).

Objective

We sought to characterize the histologic findings of AOSD-associated persistent rash in 11 cases and correlate the histologic findings with the clinical features.

Methods

From 1988 to 2004, 17 cases fulfilling Yamaguchi's criteria for AOSD in our hospital were reviewed and 11 (65%) manifested persistent papules and plaques. The pathology of 13 biopsy specimens of persistent eruption from 9 patients was reviewed.

Results

The 11 patients consisted of 3 men and 8 women with age of onset ranging from 19 to 67 years (average 34.7 years). Evanescent Still's rash was recorded in 9 patients. The persistent rash manifested as pruritic, red, violaceous, or brownish scaly or crusted lichenoid papules and plaques usually widely distributed over the trunk, neck, face, and extensor sides of the extremities. Lesions arranged in a bizarre linear pattern resulting from scratching were noted in some patients. Three patients died of severe disease, systemic complications, or both. The histology of persistent papules and plaques was characterized by: (1) multiple individual necrotic keratinocytes, singly or in aggregates, mainly located in the upper epidermis, including the normal or parakeratotic horny layer; and (2) infiltration of lymphocytes and neutrophils in the papillary and middermis. Other less common findings included basal vacuolar alteration, nuclear dust, and subcorneal or intracorneal pustules.

Conclusions

A clinically and pathologically distinct form of persistent lichenoid eruption was commonly observed in our patients with AOSD. The combination of multiple individual necrotic keratinocytes in the upper epidermis and a dermal infiltrate of neutrophils allow for histologic differentiation of this persistent eruption from most other lichenoid and interface dermatitides and may facilitate an earlier diagnosis of AOSD.

Section snippets

Methods

All cases of AOSD diagnosed in our hospital from July 1988 to June 2004 were retrieved for review. Yamaguchi's criteria for AOSD was used (Table I).2 Specifically, high fever, arthralgia, typical evanescent rash, and neutrophilic leukocytosis are major criteria; sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction, and absence of rheumatoid factor and antinuclear antibody are minor criteria. Five or more criteria (including two major criteria) are required for diagnosis.

Results

There were 17 patients with AOSD; 11 patients (10 Taiwanese men and women, and one Indonesian woman) manifested persistent rash. The 11 patients consisted of 3 men and 8 women with age of onset ranging from 19 to 67 years (average 34.7 years). The clinical features of the first 16 cases are the subject of a separate study. The important clinical and laboratory findings of these 11 patients are summarized in Table II. Of the 11 patients with persistent rash, 10 manifested erythematous,

Discussion

We found in this study that the pathologic features of the AOSD-associated persistent lichenoid papules and plaques were characterized by: (1) mounds of parakeratosis and presence of necrotic keratinocytes, singly or in aggregates, mostly in the upper half of the epidermis, and commonly in the horny layer; and (2) a superficial perivascular and/or interstitial neutrophilic and lymphocytic infiltrate. The epidermis was essentially devoid of inflammatory cells, except in the occasional foci of

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Funding sources: None.

Conflicts of interest: None identified.

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Copyright © 2005 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.