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Flagellate rash in adult-onset still's disease


1 Department of Dermatology, Venereology and Leprology, R.N.T. Medical College, Udaipur, Rajasthan, India
2 Senior Consultant Pathologist, Neuberg Supratech Reference Laboratories, Ahmedabad, Gujarat, India

Date of Submission28-Mar-2020
Date of Decision11-Apr-2020
Date of Acceptance21-May-2020
Date of Web Publication19-Sep-2020

Correspondence Address:
Asit Mittal,
Department of Dermatology, Venereology and Leprosy, R.N.T. Medical College, Udaipur, Rajasthan - 313 001
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_178_20



How to cite this URL:
Patidar A, Parikh PM, Balai M, Mittal A. Flagellate rash in adult-onset still's disease. Indian Dermatol Online J [Epub ahead of print] [cited 2020 Nov 1]. Available from: https://www.idoj.in/preprintarticle.asp?id=295424

Sir,

A 34-year-old female was in care of an internist for high-grade fever with chills, generalized body ache, sore throat, and pain in multiple joints for 1 week. After 3 days she developed a rash over her skin for which she was referred to us. On examination, she was febrile (103 °F), wrist and ankle joints were extremely tender with minimal swelling. A few cervical lymph nodes were enlarged. Skin examination showed lichenoid to hyperpigmented, mildly pruritic plaques over the limbs and trunk in a flagellate pattern [Figure 1] and [Figure 2]. There was no mucosal involvement.
Figure 1: Symmetrical lichenoid to hyperpigmented flagellate rash over back

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Figure 2: Flagellate rash extending from chest to abdomen

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Her investigations showed a TLC count of 22,400/mm3 with 86.1% neutrophils and an increase in erythrocyte sedimentation rate (71 mm/h) and CRP (273.70 mg/L). S. ferritin level was very high (>10,000 ng/ml). She was tested negative for rheumatoid arthritis factor, antinuclear antibodies, as well as p-ANCA and c-ANCA. Liver function tests and renal function tests were normal. Chest X-ray and abdominal ultrasonography were normal. ELISA test for HIV was negative too. No microorganisms grew on throat swab culture. No evidence of any infection such as malaria/dengue/typhoid was found on blood test. She was empirically treated by the internist with doxycycline and anti-malarials (quinine and primaquine), ranitidine and ebastine but no improvement was seen. None of the drugs which she was taking, have been reported in the literature to cause flagellate rash [Table 1].[1] This prompted us to think of adult onset Still's disease (AOSD) as the diagnosis. Patient also fulfiled Yamagushi's criteria for the diagnosis.[2]
Table 1: Causes of Flagellate dermatoses

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Skin biopsy showed mild ortho-keratosis, acanthosis with focal basal call vacuolation [Figure 3] and numerous eosinophils in epidermis and papillary dermis [Figure 4]. She was then referred to rheumatologist who concurred with the diagnosis of AOSD after excluding infectious, malignant or autoimmune causes. She was treated with high-dose steroids after which her fever subsided, general conditions improved and the rash resolved. She was discharged after a week on oral steroids.
Figure 3: Skin biopsy showing mild ortho-keratosis and acanthosis with focal basal call vacuolation and exocytosis of scattered eosinophils. The dermis exhibits mild to moderate peri-vascular inflammation [H&E × 100]

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Figure 4: High power view showing exocytosis of eosinophils [H&E 400x]

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AOSD is a multisystem disorder of unknown etiology characterized by high spiking fever, typical evanescent maculo-papular skin rash, arthralgias, neutrophilic leukocytosis, negative rheumatoid factor, and antinuclear antibodies and marked hyperferritinemia.[3] The diagnosis requires exclusion of infectious, malignant and connective tissue diseases. In recent years, atypical cutaneous manifestations are increasingly being reported.[4],[5] In our case, atypical lesions were present in the form of linear persistent pruritic plaques in a flagellate pattern which has been described previously in other case reports also.[4],[5]

AOSD is categorised as a multigenic autoinflammatory disease.[3] It is defined a disorder at the “crossroads” of autoinflammatory and autoimmune diseases, because of the involvement of both arms of immune system, innate and adaptive ones.[3] The presence of eosinophils and absence of dyskeratotic cells on histopathology in our case differs somewhat from previously described cases.[4],[5] As more cases are being reported, the clinicopathological spectrum of Still's disease will continue to be expanded and redefined.[6] We are reporting this case as accumulation of such cases are needed to sensitize the scientific community about the atypical skin rashes in AOSD. Recognition of such dermatological signs can also aid in the diagnosis of this uncommon entity.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Bhushan P, Manjul P, Baliyan V. Flagellate dermatoses. Indian J Dermatol Venereol Leprol 2014;80:149-52.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H,et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992;19:424-30.  Back to cited text no. 2
    
3.
Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still's disease. Autoimmun Rev 2014;13:708-22.  Back to cited text no. 3
    
4.
Suzuki K, Kimura Y, Aoki M, Takezaki S, Tuchida T, Takano T,et al. Persistent plaques and linear pigmentation in adult-onset Still's disease. Dermatology 2001;202:333-5.  Back to cited text no. 4
    
5.
Patra S, Bhari N, Mohta P, Singh S, Ramam M, Agarwal S. Adult-onset Still's disease presenting as blotchy and flagellate pigmentation. Indian J Dermatol Venereol Leprol 2019;85:626-8.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Larson AR, Laga AC, Granter SR. The spectrum of histopathologic findings in cutaneous lesions in patients with still disease. Am J Clin Pathol 2015;144:945-51.  Back to cited text no. 6
    


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