Unique Histopathologic Findings in a Patient With Adult-Onset Still Disease

Abstract

Adult-onset Still disease (AOSD) is an uncommon disorder characterized by fever, polyarthralgia, elevated white blood cell count, and a maculopapular rash, the histologic features of which have not been well-known. A 55-year-old Asian woman presented initially with a ''burning'' and severely pruritic eruption on her face, hands, and arms, thought clinically to be urticaria. Within 1 month, she began spiking high fevers, developed diffuse joint pain, and had marked elevations of ferritin, C-reactive protein, and erythrocyte sedimentation rate, characteristic of AOSD. The cutaneous eruption became more widespread, involving the trunk, scalp, and remainder of the extremities, with diffuse thickening of the skin with papular and linear hyperpigmentation and accentuation. Biopsies from several locations showed focal hyperkeratosis associated with dyskeratotic keratinocytes with a peculiar, distinctive distribution in the upper epidermis and cornified layers. In addition, increased dermal mucin was present, with minimal fibroblast proliferation and inflammation. This unusual combination of diffuse dermal mucinosis and a unique pattern of dyskeratosis can present a challenge in generating an accurate differential diagnosis, and may represent an unusual response to chronic scratching or be a distinctive histologic manifestation of AOSD. (Am J Dermatopathol 2007;49:194-196)

Urticaria and dermographism in patients with adult-onset Still’s disease

“…The three reported cases had atypical cutaneous eruption. Patients 1 and 3 presented persistent urticarial lesions, a rare manifestation previously reported in a few cases [8,[14][15][16][17][18][19][20][21][22]. Patient 2 had urticarial papules and persistent erythema which was aggravated during fever spikes.…”

“…However, only about 25 cases of AOSD are described in the literature which had a clinical presentation with urticaria or angioedema [8,[14][15][16][17][18][19][20][21][22].…”

A Case Report of Still’s Disease in the Adult

“…Therefore, the frequent delay on the diagnosis brings no surprises and our case is no exception. The lack of specificity of the symptoms and signs, and their absence of synchronicity concerning the cutaneous manifestations, represent a strong contributor to the difficulties on this diagnosis [9][10][11].…”

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

“…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.…”

“…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.…”