Neutrophilic dermatosis on postmastectomy lymphoedema: a localized and less severe variant of Sweet syndrome

ARTICLE

Auteur(s) : Cheng-Han Lee¹, Hsing-Chuan Lee¹, Chia-Fang Lu^2,3, Cheng-Hsiang Hsiao⁴, Shiou-Hwa Jee¹, Jeng-Wei Tjiu¹

¹Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 7, Chung-Shan South Road, Taipei 100, Taiwan
²Department of Periodontics, Chang-Gung Memorial Hospital, Taipei, Taiwan
³Graduate Institute of Dental and Craniofacial Science, Chang-Gung University, Taoyuan, Taiwan
⁴Departments of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan

Arm lymphoedema is a common complication of breast cancer patients treated with axillary dissection and radiation therapy. Neutrophilic dermatosis or Sweet syndrome (SS) localized on the area of postmastectomy lymphoedema is rare and only ten cases have been reported before [1-3]. Here, we report a case of neutrophilic dermatosis on postmastectomy lymphoedema (NDPL).

A 60-year-old woman was diagnosed with infiltrating ductal carcinoma of the right breast. She received a mastectomy with axillary dissection, adjuvant chemotherapy, hormone therapy, and radiation therapy. Lymphoedema of her right upper extremity developed two months after the mastectomy. After the lymphoedema had existed for five months, she presented with a one-day history of rapidly spreading, tingling red rashes on the right arm affected by postmastecomy lymphoedema (figure 1A). On examination, there were many variously sized erythematous patches and inflammatory papules with pseudovesicles located on her right arm, forearm and the dorsal aspect of her hand (figure 1B). There were no external triggering factors and the possibility of contact dermatitis was ruled out. She was prescribed oxacillin for a presumed cutaneous infection. A skin biopsy taken from her right arm revealed papillary dermal edema, dense neutrophilic infiltration in the dermis without evidence of vasculitis (figure 1C). All cultures gave negative results. The total leukocyte count was 4.07 × 10⁹/L (normal 4-10 × 10⁹/L) with 73.3% of neutrophils (normal < 70%). A diagnosis of NDPL was made. The administration of oral naproxen 750 mg daily and topical clobetasol propionate resolved the skin lesions within one week.

NDPL is a newly recognized disease entity [1-3]. The skin changes of the ten previously reported cases were painful erythematous papules that gradually became plaques on the arm affected by lymphoedema [1-3]. The skin eruption may become vesicles or blisters, but no hemorrhagic bullae or pustules have been reported before [1-3]. Because patients with NDPL had typical skin lesions of SS on the lymphoedematous area, NDPL was considered as a localized variant of SS [1, 2]. However, the differences between NDPL and SS were far beyond anatomical predilections. Comparing the features of NDPL with that of SS, we found patients with NDPL less frequently had leukocytosis, neutrophilia, and recurrences (table 1) [1-4]. Only one case of NDPL was reported as having had an erythrocyte sedimentation rate (ESR) at 29 mm/hr (others were not reported) [3]; however, 89% of patients with SS had ESR greater than 30 mm/hr [4]. Patients with SS responded rapidly to systemic corticosteroids and potassium iodide [4]. However, patients with NDPL treated with antibiotic monotherapy showed a shorter recovery time (7.5 days) comparing to those treated with systemic corticosteroids or potassium iodide (17.3 days) [1-3]. In sum, patients with NDPL had less systemic involvement, fewer recurrences and different responses to treatments. Therefore, we chose the term “NDPL” instead of “SS on the postmastectomy lymphoedema” to suggest that NDPL is not only a localized but a severity variant of SS.
Table 1 Differences between neutrophilic dermatosis on postmastectomy lymphoedema and Sweet syndrome

45.6% of NDPL cases were treated with antibiotics during their treatment course; this reflected the fact that half of the NDPL was mistaken for erysipelas or cellulitis [1-3]. The actual incidence of NDPL would have been higher if all patients with NDPL had been diagnosed correctly [1-3]. The ethiology of NDPL is unclear. However, the lymphoedema was a result of accumulation of protein-rich interstitial fluid containing high levels of cytokines and chemokines which might attract neutrophils to migrate and infiltrate the area of lymphoedema [1-3, 5]. Failure of lymphatic drainages disrupted the normal trafficking of neutrophils and trapped them in the lymphoedematous area [1-3, 5]. It is important to remind clinicians of the emerging disease entity, NDPL. Further investigations are required to elucidate the pathogenesis of NDPL.

Acknowledgements

References

2 Petit T, Frances C, Marinho E, Herson S, Chosidow O. Lymphoedema-area-restricted Sweet syndrome during G-CSF treatment. Lancet 1996; 347: 690.

3 Demitsu T, Tadaki T. Atypical neutrophilic dermatosis on the upper extremity affected by postmastectomy lymphedema: report of 2 cases. Dermatologica 1991; 183: 230-3.

4 von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31: 535-56.

5 Ruocco E, Puca RV, Brunetti G, Schwartz RA, Ruocco V. Lymphedematous areas: privileged sites for tumors, infections, and immune disorders. Int J Dermatol 2007; 46: 662.