|Year : 2015 | Volume
| Issue : 3 | Page : 66-68
Sweet's syndrome exhibiting the Koebner phenomenon at the site of dermatofibroma
Belkiz Uyar1, Oya Nermin Sivrikoz2, Ayse Gökduman3, Handan Saçar1
1 Department of Dermatology, Sifa University, Faculty of Medicine, Izmir, Turkey
2 Department of Pathology, Sifa University, Faculty of Medicine, Izmir, Turkey
3 Department of Biochemistry, Sifa University, Faculty of Medicine, Izmir, Turkey
|Date of Web Publication||8-Oct-2015|
Dr. Belkiz Uyar
Department of Dermatology, Sifa University, 172/2 Fevzipaşa Bulvarı Basmane, Izmir-35240
Source of Support: None, Conflict of Interest: None
Sweet's syndrome (SS), also known as acute febrile neutrophilic dermatosis, is characterized by fever, neutrophilia, and inflammatory skin lesions. SS can be associated with several conditions, such as infections, malignancy, autoimmune disease, vaccination, pregnancy, and drug exposure. We present a woman with SS; skin lesions developed at the site of dermatofibroma and scratched area. To the best of our knowledge, this is the first case of SS developed at the site of dermatofibroma.
Keywords: Acute febrile neutrophilic dermatosis, dermatofibroma, Koebner phenomenon, Sweet′s syndrome
|How to cite this article:|
Uyar B, Sivrikoz ON, Gökduman A, Saçar H. Sweet's syndrome exhibiting the Koebner phenomenon at the site of dermatofibroma. Sifa Med J 2015;2:66-8
|How to cite this URL:|
Uyar B, Sivrikoz ON, Gökduman A, Saçar H. Sweet's syndrome exhibiting the Koebner phenomenon at the site of dermatofibroma. Sifa Med J [serial online] 2015 [cited 2020 Apr 4];2:66-8. Available from: http://www.imjsu.org/text.asp?2015/2/3/66/166864
| Introduction|| |
Sweet's syndrome (SS) was first described as an acute febrile neutrophilic dermatosis by Sweet in 1964.  Patients with SS have presented many associated findings. In a few patients, the Koebner phenomenon has been reported associated with SS, related to sun exposure, thermal injury, chemical irritation, radiotherapy, and previous skin trauma. 
The Koebner phenomenon was first described in psoriatic skin by Koebner in 1876.  This phenomenon has since been reported in various diseases, including vitiligo, lichen planus, sarcoidosis, autoimmune blistering dermatoses, discoid lupus erythematosus, systemic lupus erythematosus, systemic sclerosis, dermatomyositis, and pyoderma gangrenosum.
We report a woman with SS exhibiting the Koebner phenomenon at the site of dermatofibroma. In this paper, we also review the literature on the Koebner phenomenon in SS.
| Case Report|| |
A 67-year-old woman was admitted to our hospital due to fever, diarrhea, and skin rash lasting for 4 days. On examination, she had fever of 38.5°C. Dermatologic examination revealed purplish-red, infiltrated, 2-cm diameter plaque that had developed over old skin lesions on the left upper arm [Figure 1]; a linear red plaque scattered over the right arm [Figure 2]; and a purplish-red, tender papulonodular rashes on the fingertips.
|Figure 1: Purplish-red, infiltrated, 2-cm diameter plaque developed over old dermatofibroma on the left upper arm|
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Laboratory evaluation revealed leukocytosis (26,500/μl) with 47% neutrophils and an elevated erythrocyte sedimentation rate (82 mm/1 st hour) and C-reactive protein (7.20 mg/l). A urine sample analysis revealed 20-22 leukocytes with urine microalbumin spot test 37 mg/dl.
The rest of the laboratory evaluations - including the biochemical parameters; stool analysis; immunoglobulin A, immunoglobulin G, and immunoglobulin M levels; carcinoembryonic antigen (CEA); alpha-fetoprotein (AFP); Ca 19-9; protein electrophoresis, gastroscopy, and colonoscopic examinations - returned results that were either negative or with inthenormal range. Cranial magnetic resonance imaging, transthoracic echocardiography, and abdominal computerized tomography were normal.
A skin biopsy of the left shoulder was performed and revealed mild hyperkeratosis; intraepidermal spongiosis; increased melanin hyperpigmentation; vacuolar degeneration of the basal cell layer; a mixed inflammatory infiltration of the dermis, especially around the proliferated capillary; swollen endothelial cells; and an intense edema on the upper dermis [Figure 3], in addition to an area of dermatofibroma [Figure 4].
|Figure 3: The biopsy specimen shows mild hyperkeratosis, intraepidermal spongiosis, increased melanin hyperpigmentation and vacuolar degeneration of the basal cell layer, mixed inflammatory infiltration of the dermis, swollen capillary endothelial cells, and an intense edema on the upper dermis (hematoxylin and eosin, ×100)|
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|Figure 4: The bottom left side of the slide shows an area of dermatofibroma (shown by arrows; hematoxylin and eosin, ×40)|
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We administered clobetasol propionate ointment to her 2 × 1/day. The patient's lesions improved dramatically within 3days of steroid treatment, and she became afebrile. The patient was followed-up for 8months. No more skin lesions appeared during this time. Based on the clinical and laboratory evidence and the immediate response to treatment with corticosteroids, the diagnosis of SS was established.
| Discussion|| |
SS, also known as acute febrile neutrophilic dermatosis, is characterized by fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltration consisting mostly of mature neutrophils. SS presents in three clinical settings: Classical (or idiopathic), malignancy associated, and drug induced. Classical SS, usually presents in women between the ages 30 and 50 years, is often preceded by an upper respiratory tract infection, and may be associated with inflammatory bowel disease and pregnancy.  In this case, although we did not find any etiological factors, we think that diarrhea might be the causal factor.
The pathogenesis of the SS is not understood precisely. The association with infections, autoimmune diseases, neoplasms, and drugs suggests a hypersensitivity reaction. Circulating autoantibodies, cytokines, dermal dendrocytes, human leukocyte antigen (HLA) serotypes, immune complexes, and leukotactic mechanisms have been suggested as factors that contribute to the pathogenesis of this syndrome. Cytokines appear to play an etiologic role in the development of lesions and symptoms of SS. Granulocyte colony-stimulating factor, macrophage colony-stimulating factor, interferon-gamma (IFN-γ), interleukin (IL)-1, IL-3, IL-6, and IL-8 are potential candidates. Elevated serum levels of IL-1, IL-2, and IFN-γ, but not IL-4, suggest that the expression of Th1 cytokines may be involved in the pathogenesis of this syndrome. Although an increase in the frequency of HLA-BW54 has been shown in Japanese patients with SS, analysis of HLA antigens in the Caucasian population showed no association between this syndrome and certain HLA-ABC antigens. 
In histopathological appearance, edema is characteristically present, as well as a dense polymorphonuclear cell infiltration in the upper dermis. Fragmented neutrophil nuclei (called karyorrhexis or leukocytoclasia), swollen endothelial cells, and dilated small blood vessels may also be present. However, eosinophils have been observed within the dermal infiltration in the SS skin lesions of patients with both classical and drug-induced dermatosis. Occasionally, lymphocytes or histiocytes may be present in the inflammatory infiltration.  In our patient, histopathological examination revealed mixed inflammatory cell infiltration.
Despite the fact that vacuolar degeneration of the basal cell layer could be seen in erythema multiforme, our diagnoses was SS because of the presence of polymorphonuclear leukocytes rich mixed inflammatory infiltration of the perivascular region, the presence of proliferating capillaries, the presence of endothelial swelling, the absence of epidermal necrosis, and the absence of apoptotic cell in the epidermis. Furthermore, clinically the patient looked like SS rather than erythema multiforme. The Koebner phenomenon refers to the development of similar pathological lesions in the traumatized, uninvolved skin of a patient with preexisting cutaneous disease. This phenomenon has been reported in association with various diseases, including psoriasis, vitiligo, lichen planus, sarcoidosis, autoimmune blistering dermatoses, discoid lupus erythematosus, systemic lupus erythematosus, systemic sclerosis, dermatomyositis, and pyoderma gangrenosum. The exhibition of the Koebner phenomenon requires that the injury affect both the epidermis and the dermis.  Factors provoking the Koebner phenomenon include needling, scratches, trauma, tattooing, scars, heat, cold, pressure, sun exposure, X-rays, drug administration, silica granulomas, and inflammatory dermatoses. 
In 1975, a review by Gunawardena et al., reported that SS patients may exhibit the Koebner phenomenon.  Tavadia et al., reported that a patient operated on for oral squamous cell carcinoma exhibited the SS at sites corresponding to the position of the sandbag and hemostatic cuff used during the operation. 
Newly reported cases have localized SS lesions in areas exposed to the sun, chemical irritation, previous skin trauma, radiation therapy, and sensitizing antigens. Occasionally, SS lesions have appeared on an arm affected by post-mastectomy lymphedema.  To the best of our knowledge, our patient is the first SS exhibiting the Koebner phenomenon at the site of dermatofibroma.
These findings support that the Koebner phenomenon can exhibit with SS.
| References|| |
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56.
Tavadia SM, Smith G, Herd RM, Zuk RJ. Sweet's syndrome associated with oral squamous cell carcinoma and exhibiting the Koebner phenomenon. Br J Dermatol 1999;141:169-70.
Köbner H. The etiology of psoriasis, Vierteljahrsschr. Dermatology 1876;8:539-61.
Cohen PR. Sweet's syndrome-a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;2:34.
Bonamigo RR, Razera F, Olm GS. Neutrophilic dermatoses: Part I. An Bras Dermatol 2011;86:11-25.
Taniguchi T, Asano Y, Okada A, Sugaya M, Sato S. Ultraviolet light-induced Köbner phenomenon contributes to the development of skin eruptions in multicentric reticulohistiocytosis. Acta Derm Venereol 2011:160-3.
Gunawardena DA, Gunawardena KA, Ratnayaka RM, Vasanthanathan NS. The clinical spectrum of Sweet's syndrome (acute febrileneutrophilic dermatosis)-a report of eighteen cases. Br J Dermatol 1975;92:363-73.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]