ISSN 2394-7438 Volume 2 | Issue 3 | Sep-Dec 2016
Journal of MAMC Journal of Medical Sciences • Volume 2 • Issue 3 • September-December 2016 • Pages ???
MAMC
Journal of
Medical Sciences
Official Publication Maulana Azad Medical College
MAMC Journal of Medical Sciences Case Report
Sweet’s Syndrome with Nodular Scleritis: A Rare Case Report Anubhav Chauhan, Neeraj Sharma1, Shashi Datt Sharma Department of Ophthalmology, Dr. Yashwant Singh Parmar Government Medical College, Nahan, 1Department of Dermatology, Regional Hospital Hamirpur, Himachal Pradesh, India
Abstract Sweet’s syndrome is a disease of unknown etiology characterized by eruption of painful, erythematous, cutaneous plaques, and nodules of rapid onset accompanied by fever, leukocytosis, and neutrophilia. We report a case of a 52‑year‑old female having Sweet’s syndrome with nodular scleritis. Key words: Dermatological, scleritis, Sweet’s syndrome
Introduction Acute febrile neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, has been termed as Sweet’s syndrome. Involvement of the eyes, joints, oral mucosa, lungs, liver, kidneys, and central nervous system has been described.[1] It is a dermatological disorder with accompanying features of systemic inflammation. Ocular involvement most commonly is in the form of conjunctivitis, others being periorbital and orbital inflammation, dacryoadenitis, episcleritis, scleritis, limbal nodules, peripheral ulcerative keratitis, iritis, glaucoma, and choroiditis. The ocular inflammation appears concurrently with skin lesions.[2] Herein, we report a case of Sweet’s syndrome with ocular involvement.
Case Report A 52‑year‑old female presented to the skin department of a secondary care institute of the hilly state with high‑grade fever for 4 days accompanied by skin lesions and painful red left eye [Figures 1 and 2]. There was a history of sore throat 1 week before the onset of skin lesions. The lesions were present over dorsal aspect of the feet, anterior aspect of the legs, forearms bilaterally and were erythematous, noduloplaques varying from 1 cm × 1 cm to 2 cm × 3 cm in size and tender on palpation with pseudovesiculation. On hematological investigations, positive Access this article online Quick Response Code:
Website: www.mamcjms.in
DOI: 10.4103/2394-7438.191682
findings were leukocytosis (10,000 cmm) with predominant neutrophilia (80%), elevated erythrocyte sedimentation rate (30 mm in the first hour), and positive C‑reactive proteins. A provisional diagnosis of Sweet’s syndrome was made, and a punch skin biopsy of the skin lesion was sent for histopathological examination. The patient was started on tablet prednisolone 1 mg/kg once a day along with tablet azithromycin 500 mg once a day (for 3 days) plus tablet hydroxyzine 25 mg at night (for 10 days). The patient was referred to the eye department for the evaluation of painful red left eye, and a diagnosis of nodular scleritis was made by an ophthalmologist on the basis of a characteristic eye lesion (scleral nodule in the interpalpebral area with a reddish‑blue color) and a further confirmation by phenylephrine test, where instillation of 2.5% and 5% phenylephrine drops did not blanch/reduce the redness of the eye. The patient was further advised blood investigations in the form of rheumatoid factor, antinuclear antibodies, serum Address for correspondence: Dr. Anubhav Chauhan, Senior Resident, Department of Ophthalmology, Dr Yashwant Singh Parmar Government Medical College, Nahan, District Sirmaur, Himachal Pradesh, India. E‑Mail:
[email protected] This is an open access article distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as the author is credited and the new creations are licensed under the identical terms. For reprints contact:
[email protected]
How to cite this article: Chauhan A, Sharma N, Sharma SD. Sweet's syndrome with nodular scleritis: A rare case report. MAMC J Med Sci 2016;2:149-51.
© 2016 MAMC Journal of Medical Sciences | Published by Wolters Kluwer - Medknow
149
Chauhan, et al.: Sweet’s Syndrome with Nodular Scleritis
Figure 2: Scleritis Figure 1: Skin lesion
angiotensin converting enzymes, anti‑nuclear cytoplasmic antibodies, antiphospholipid antibodies, Lymes titer, QuantiFERON‑TB gold test, herpes markers, blood culture, and serum calcium as a number of systemic diseases are associated with scleritis. All the above investigations were within normal limits. The patient was advised to continue prednisolone (1 mg/kg) for 2 weeks and then taper the dose over the next 2 weeks. The patient reported to us after 2 weeks with her histopathological report which showed findings consistent with Sweet’s syndrome, i.e. dermal infiltrate composed predominately of neutrophils. There was marked improvement in her skin and ocular signs and symptoms, and prednisolone dose was subsequently tapered. This is a rare case report in which Sweet’s syndrome was associated with nodular scleritis.
Discussion The exact pathogenesis of Sweet’s syndrome remains unknown. Altered immunological reactivity in the form of hypersensitivity to bacterial, viral, tumor antigens, or circulating immunocomplex reaction and cytokine deregulation are the proposed etiopathological factors.[3] It may be associated with a viral upper respiratory tract infection, hematologic and visceral malignancies, medications, autoimmune diseases, inflammatory bowel diseases, and pregnancy. It can be classified into three subtypes: (1) Classical or idiopathic, (2) associated with hematological malignancies, (3) associated with solid malignant visceral neoplasms. The first category accounts for most (80–90%) of the reported cases.[4] Ours was a case of classical Sweet’s syndrome [Table 1]. Histopathological findings include a dense dermal infiltrate composed of neutrophils with leukocytoclasis and prominent papillary dermal edema occasionally producing subepidermal vesiculation or bullae.[5] Incidence is usually in the fourth or fifth decade with a peak in January and February. One study found that Sweet’s syndrome accounted for 4/1000 new cases in a dermatological unit.[6] It is five times more common in females than in males.[6] Our case was seen in the 52‑year‑old female. 150
Table 1: Diagnostic criteria for classical Sweet’s syndrome versus drug‑induced Sweet’s syndrome Classicala 1. Abrupt onset of painful erythematous plaques or nodules 2. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis 3. Pyrexia >38°C 4. Association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, or preceded by an upper respiratory or gastrointestinal infection or vaccination 5. Excellent response to treatment with systemic corticosteroids or potassium iodide 6. Abnormal laboratory values at presentation (three of four): Erythrocyte sedimentation rate >20 mm/h; positive C‑reactive protein; >8000 leukocytes; >70% neutrophils Drug‑inducedb A. Abrupt onset of painful erythematous plaques or nodules B. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis C. Pyrexia >38°C D. Temporal relationship between drug ingestion and clinical presentation, or temporally‑related recurrence after oral challenge E. Temporally‑related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids a The presence of both major criteria (1 and 2) and two of the four minor criteria (3, 4, 5, and 6) is required to establish the diagnosis of classical Sweet’s syndrome, the patients with malignancy‑associated Sweet’s syndrome are included with the patients with classical Sweet’s syndrome in this list of diagnostic criteria, bAll five criteria (A, B, C, D, and E) are required for the diagnosis of drug‑induced Sweet’s syndrome
Fever is the most frequent symptom. Indeed, the skin eruption of Sweet’s syndrome is usually accompanied by fever and leukocytosis. However, the cutaneous manifestations of the disease may be preceded by several days to weeks of fever. The incidence of ocular involvement is variable in classical Sweet’s syndrome. However, ocular lesions of Sweet’s syndrome are uncommon in the malignancy associated and drug‑induced forms of the dermatosis.[7] First‑line agents in the treatment include oral prednisolone, potassium iodide, colchicine, and intravenous methylprednisolone. Usually, there is a dramatic response to systemic steroids. However, rarely, there may be a need to institute the second‑line drugs in resistant or steroid‑dependent cases. These include indomethacin, clofazimine, dapsone, cyclosporine, and cyclophosphamide.[3] Sweet’s syndrome
MAMC Journal of Medical Sciences ¦ Sep-Dec 2016 ¦ Volume 2 ¦ Issue 3
Chauhan, et al.: Sweet’s Syndrome with Nodular Scleritis
in association with scleritis has been described only by some authors,[8,9] and the use of steroids, colchicine, and dapsone as treatment modalities. We want to emphasize the need for a high index of suspicion, early diagnosis, timely intervention, and regular follow‑up in such cases.
Acknowledgments
We would like to thank our patient.
Financial support and sponsorship Nil.
Conflicts of interest
There are no conflicts of interest.
References 1. Von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535‑56.
2. Gottlieb CC, Mishra A, Belliveau D, Green P, Heathcote JG. Ocular involvement in acute febrile neutrophilic dermatosis (Sweet syndrome): New cases and review of the literature. Surv Ophthalmol 2008;53:219‑26. 3. Kapoor A, Beniwal S, Narayan S, Kalwar A. Sweet’s syndrome in accelerated chronic myelogenous leukemia: A case report and review of literature. Clin Cancer Investig J 2014;3:112‑5. 4. Requena L, Kutzner H, Palmedo G, Pascual M, Fernández‑Herrera J, Fraga J, et al. Histiocytoid Sweet syndrome: A dermal infiltration of immature neutrophilic granulocytes. Arch Dermatol 2005;141:834‑42. 5. Malone JC, Slone SP, Wills‑Frank LA, Fearneyhough PK, Lear SC, Goldsmith LJ, et al. Vascular inflammation (vasculitis) in Sweet syndrome: A clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 2002;138:345‑9. 6. Lear JT, Atherton MT, Byrne JP. Neutrophilic dermatoses: Pyoderma gangrenosum and Sweet’s syndrome. Postgrad Med J 1997;73:65‑8. 7. Cohen PR. Sweet’s syndrome – A comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;2:34. 8. Wong MH, Su DH, Loh RS. Nodular scleritis and Sweet’s syndrome. Clin Experiment Ophthalmol 2007;35:858‑60. 9. Mazokopakis E, Kalikaki A, Stathopoulos E, Vrentzos G, Papadakis JA. Acute febrile neutrophilic dermatosis (Sweet’s syndrome) with erythema nodosum and anterior scleritis. A case report. Int J Dermatol 2005;44:1051‑3.
MAMC Journal of Medical Sciences ¦ Sep-Dec 2016 ¦ Volume 2 ¦ Issue 3
151
