Erythematous edematous plaques in a 47-year-old woman with postmastectomy lymphedema

Question

What is your diagnosis?

Answer

Neutrophilic dermatosis on postmastectomy lymphedema

Discussion

Suspecting neutrophilic dermatosis, prednisone treatment with a dosage of 30 mg per day was initiated which achieved complete clearance of cutaneous lesions and resolution of systemic symptoms after 48 h [Figure 3]. After the patient was discharged, she continued to take oral prednisone in tapering doses for six days, starting with 30 mg daily with a reduction of 10 mg each 48 h. During the two-year follow-up, we have not recorded recurrent episodes.

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Figure 3:

The skin lesions resolved completely after the treatment of systemic corticosteroids for 2 days

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Lymphedema-associated neutrophilic dermatosis is considered as an atypical or localized presentation, different from the classical sweet syndrome.^1,2 Compared with the classic sweet syndrome, this atypical variant differs from a milder clinical course, less systemic involvement and fewer recurrences.¹

It is often found in women with a history of breast cancer that underwent lymphadenectomy.^1,3 Nevertheless, there have been reported recently two clinical cases of patients with lower limb lymphedema.² The time elapsed since surgery until the appearance of skin lesions is highly variable, from a few months to 14 years.¹ There is no reported evidence of recurrent local relapse or distant metastasis from breast cancer after the resolution of the skin lesions.^1,3,4

Clinically, it consists of multiple painful erythematous papules or plaques which may coalesce which predominantly develops on the lymphedematous area and can spread to the ipsilateral trunk.^1,3,4 Systemic symptoms are usually mild or they even may be absent. Both extracutaneous manifestations and laboratory findings are often less remarkable comparing to the classical sweet syndrome.^2,3

Histopathological findings are similar to those of classical sweet syndrome; an infiltrate primarily composed of neutrophils and localized mainly in the dermis. In some cases, the inflammatory infiltrate includes lymphocytes, histiocytes and eosinophils and there is marked edema in the papillary dermis.^1,3

Regarding treatment, some publications are showing a good response to oral antibiotics and potassium iodide. However, systemic steroids remain as the first-line treatment for this entity, as well as in the classical sweet syndrome with the improvement of the symptoms and resolution of the cutaneous lesions shortly after initiation of therapy.^1,2

Differential diagnosis is broad and it includes many different entities such as cellulitis, recall phenomenon, inflammatory breast carcinoma and herpes zoster.^1,2,4 The patchy distribution of skin lesions, their extension beyond the irradiated area, the acute onset, as well as the absence of clustered vesicles, respectively, can aid in this differential diagnosis. Furthermore, a substantial improvement after systemic steroid therapy is a useful clue. Complementary tests such as Tzanck smear and laboratory and histopathological findings may also help along with the clinical presentation. The history of lymphedema should raise suspicion of this entity.

We consider that lymphedema-associated neutrophilic dermatosis is a poor-known complication and, therefore, it is most probably underdiagnosed, since this condition may mimic other infectious or inflammatory entities.