Persistent generalized erythema in adult‐onset Still's disease

Abstract

A 64-year-old Japanese woman had been in good health before she developed intermittent fever (up to 38.5 ° C), cervical lymphadenopathy, myalgia of the thighs, and polyarthralgia in association with evanescent macular erythema on the dorsa hands. Within a week the constitutional symptoms progressively aggravated, the intermittent fever rose to 40 ° C, and she became lethargic. The erythema became persistent and widespread, and coalesced into a diffuse edematous plaque lesion that involved the face, limbs, and trunk. The erythema was most extensive on the back, and was of linear distribution ( Fig. 1a). The mucous membrane was not affected. Skin biopsy specimens showed a moderate degree of mononuclear cell infiltration with a few neutrophils around dilated blood vessels in the edematous upper-mid-dermis (not shown). The epidermis was intact and there was no evidence of vasculitis. Laboratory examinations revealed neutrophilic leukocytosis (25,600/ µ L, 81% neutrophil with a left shit), an increase in the serum CRP and LDH levels (26.5 mg /mL and 1927 IU/mL, respectively; LDH normal range: 200 -450 IU/ mL), and hyperferritinaemia (42,870 ng /mL; normal range: 10-100 ng /mL). Radiological examinations denied lymphoproliferative disorders. Serological tests for collagen vascular diseases and those for various viral diseases were all negative. The results of repeated blood, urine, and throat cultures were not diagnostic, and systemic antibiotics or discontinuation of all medications did not improve the symptoms or the abnormal laboratory data. Adult-onset Still's disease (AOSD) was suspected, and all the constitutional symptoms and the abnormal laboratory data improved in response to the systemic administration of methylprednisolone (1000 mg / day for 3 days). The skin rash disappeared without any sequels. Figure 1 (a) Coalescent linear erythema on the back in Case 1. (b) Coalescent macular erythema on the forearm in Case 2

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

“…The atypical skin eruption was the presenting finding in 3 cases 18,20 while it was reported to appear concurrently with systemic symptoms in the majority of cases. 7,[10][11][12][13][14][15][16][17]20,21 In these latter cases, atypical skin rash appeared as soon as 1 week 18 and as late as 5 months 10 after the classic symptoms of Still disease.…”

“…17 Linear configurations were frequently discussed. 9,[11][12][13][16][17][18] Other descriptions included prurigo pigmentosaelike, 14,16 urticarial papules, 10 lichenoid papules, 11 dermatographismlike, dermatomyositis-like, and lichen amyloidosiselike rashes. 11,13 More than 1 morphology or distribution pattern was observed in several patients.…”

“…[7][8][9][10]12,[14][15][16]18,21 The most common diagnostic procedure was a computed tomography scan of the abdomen that revealed hepatosplenomegaly (5/19; 26%), 8,9,[14][15][16] or no abnormal findings (1/19; 5%). 10 Chest radiography, positron emission tomography, lymph node biopsy, and bone marrow biopsy were pursued in select cases, 7-10,12,14-16,18,21 and a workup for malignancy was not described in 5 articles.…”

“…2 Recently, small studies and case reports have described an atypical, persistent skin rash consisting of pruritic papules and plaques with various configurations with distinct histopathology in patients with active AOSD. [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] This atypical rash often presents in addition to the typical rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In addition to these reports of atypical cutaneous presentations, emerging literature highlights an increasing recognition of malignancy in association with Still disease, sometimes occurring years later.…”

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.…”