Localized Sweet's Syndrome in an Irradiated Field

Neutrophilic dermatosis on the site of lymphedema is a rare condition and considered as a localized and less severe variant of Sweet syndrome. Only 13 cases of this variant have been reported after mastectomy for breast cancer in the English-language published work. However, this condition has never been described on the lower limbs or from other causes of lymphedema. Herein, we report two cases of localized Sweet syndrome on the lymphedematous lower limbs: one occurred after radical hysterectomy, bilateral pelvic lymph node dissection and radiotherapy for cervical cancer; the other developed after radiotherapy for malignant melanoma on the right groin. Based on clinical and histological features, we suggest the name "lymphedema-associated neutrophilic dermatosis" for this underrecognized disease entity.

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“…Few cases of Sweet syndrome localized to the irradiated field have been reported . The pathophysiology of this condition remains unknown.…”

Section: Discussionmentioning

confidence: 99%

“…Few cases of Sweet syndrome localized to the irradiated field have been reported. [10][11][12] The pathophysiology of this condition remains unknown. Some authors proposed that the degenerated products of neoplastic cells due to irradiation may induce the neutrophilic response.…”

Section: Discussionmentioning

confidence: 99%

Lymphedema‐associated neutrophilic dermatosis: Two cases of localized Sweet syndrome on the lymphedematous lower limbs

Chu

Cheng

Kao

et al. 2016

The Journal of Dermatology

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“…[1][2][3][4][5] As in our case, previous reports describe the initiation of the rash within the radiation field, followed by spread to nonirradiated fields. Given the diverse etiologic associations, the mechanism of action has not been determined.…”

mentioning

confidence: 78%

“…[1][2][3][4] Sorafenib, the multikinase inhibitor, has been reported to cause a variety of cutaneous manifestations ranging from erythema multiforme to lesions resembling chloracne. 5 Although most reports focus on the development of multiple squamous cell carcinomas and other squamous lesions, some discuss the development of follicular hyperplasia and ectatic follicular infundibula. 2 Until recently, such eruptions had not been described in the setting of an investigational BRAF kinase inhibitor used in the treatment of metastatic melanoma (PLX 4032).…”

Section: Keratosis Pilariselike Eruption Observed During the Experimementioning

confidence: 99%

Sweet syndrome in a field of radiation therapy

Wanat

Kovarik

Quon

et al. 2012

Journal of the American Academy of Dermatology

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“…To date there have only been six cases of Sweet' syndrome localized to a cancer radiotherapy field, in four cases, the acute neutrophilic dermatitis developed during radiotherapy. All cases responded to a course of oral glucocorticosteroids . Together these findings suggest that radiotherapy promotes antigen expression that is able to elicit autoimmunity that is localized to the radiotherapy field, in susceptible individuals with loss of self‐tolerance.…”

mentioning

confidence: 79%

Delayed and localized Sweets syndrome after breast cancer radiotherapy

et al. 2017

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Radiotherapy treatment for breast cancer causes characteristic radiation dermatitis in the majority of patients. The DNA damaging effects of radiation can rarely predispose to primary inflammatory dermatoses, such as Sweets syndrome. In such cases, the disease presents with all the hallmarks of the primary dermatosis, but the eruption is limited to the field of irradiation and is often amenable to treatment. Over 95% of individuals following external beam ionizing radiation for breast cancer develop characterized by acute (within weeks), sub-acute and chronic (months to years) radiation dermatitis. In contrast, occurrence of more generalized Sweets syndrome in this setting may mean cancer recurrence. The mechanism by which radiotherapy induces localized disease remains unknown, but there is likely a loss of self-tolerance which maybe coupled to antigen exposure. Rarely radiotherapy may exacerbate or predispose to primary localized inflammatory dermatoses, such as Sweets syndrome that may develop after months or years.A 76-year-old female 6-years earlier had had left breast cancer resection followed by radiotherapy, a total of 56 Gy fractionated over 6 weeks. After an unremarkable follow-up period, she presented with recurrent episodes of swelling, redness, warmth, and tenderness of the left breast ( Figure 1A). Each episode was associated with fever and malaise. Examination was otherwise unremarkable and normal between attacks. The episodes began suddenly without warning and lasted approximately 5 days. Initially, the attacks were a year apart but the frequency increased over the previous year so that they occurred approximately every 3 months. She was entirely well between attacks, with no history of diarrhea, weight loss, or change in medication. During these intervals full blood count, urea and electrolytes, liver function tests, serum immunoglobulins, cancer markers, thyroid function test, anti-nuclear antibody and extra-nuclear antibody titers were all normal or negative. Both breast ultrasound and mammogram were also normal. During attacks, there was a marked neutrophilia (Figure 1B) and histology from a skin biopsy demonstrated a grenz zone overlying a dense upper dermal neutrophil-rich inflammatory infiltrate, with leucocytoclastic nuclear dust (Figure 1C, D). In the absence of clinical and histologic features of a vasculitis, the finding supported a diagnosis of Sweets syndrome.Remarkably, the episode abated with 2 days of low dose enteric coated prednisolone (10 mg); hence, the patient was prescribed prophylactic prednisolone to manage future attacks.Sweet's syndrome is a rare acute febrile neutrophilic dermatosis characterized by the abrupt onset of tender plaques and nodules on the face and extremities, less commonly on the trunk, in association with malaise, fever and a neutrophilia. Up to 15% of cases are associated with underlying malignancy, most notably myelogenous types but also solid cancers including breast cancer. 1 All patients presenting with Sweet's syndrome should therefore be screened fo...

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Localized Sweet's Syndrome in an Irradiated Field

Cited by 14 publications

References 11 publications

Lymphedema‐associated neutrophilic dermatosis: Two cases of localized Sweet syndrome on the lymphedematous lower limbs

Lymphedema‐associated neutrophilic dermatosis: Two cases of localized Sweet syndrome on the lymphedematous lower limbs

Sweet syndrome in a field of radiation therapy

Delayed and localized Sweets syndrome after breast cancer radiotherapy

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