Neutrophilic Dermatosis Limited to Post-Mastectomy Lymphoedematous or Lipo-Lymphoedematous Skin: a Localized Variant

Links checked 4/04/17.

Sometimes, Sweet’s syndrome can occur in an unusual form. This is called a disease variant.

What is neutrophilic dermatosis limited to post mastectomy lymphoedematous or lipo-lymphoedematous skin?

Neutrophilic dermatosis limited to post-mastectomy lymphoedematous or lipo-lymphoedematous skin (NDPL) is a rare localized (confined to one part of the body) variant of Sweet’s syndrome that is sometimes referred to as localized Sweet’s syndrome. It is a variant that only affects the lymphoedematous or lipo-lymphoedematous skin of a patient who has had a mastectomy (surgical removal of a breast) (Ainechi and Carlson, 2016). A patient who has had a mastectomy with a lymph node dissection (surgical removal of the lymph nodes) is at significantly increased risk of developing lymphoedema in the arm that is on the same side as the surgery.

What is lymphoedematous or lipo-lymphoedematous skin?

What is lymphoedema?

Lymphoedema is fluid retention, i.e. swelling under the skin that is caused by a build-up of fluid in the lymphatic system (the network of vessels that drains excess fluid and waste products from body tissue) (NHS Choices, 2016). It can affect any part of the body, but usually develops in the arms or legs. In patients with lymphoedema or lymphoedematous skin, when the swollen skin is pressed, a pit or indent is left.

Other symptoms in an affected body part can include:

  • An aching, heavy feeling.
  • Difficulty with movement.
  • Repeated skin infections.
  • The skin becoming hard and tight.
  • Folds developing in the skin.
  • Wart-like growths developing on the skin.
  • A leakage of fluid through the skin.

What causes lymphoedema?

There are two main types of lymphoedema (primary and secondary), and they are caused by different things.

  • Primary lymphoedema – this is caused by faulty genes affecting the development of the lymphatic system. It can develop at any age, but usually occurs in early adulthood.
  • Secondary lymphoedema – this is caused by damage to the lymphatic system or problems with the movement and drainage of fluid in the lymphatic system. This is often due to an infection, injury, cancer treatment, inflammation of the limb, e.g. arm or leg, or a lack of limb movement.

What is lipoedema?

Lipoedema is a long-term (chronic) condition that usually involves an abnormal build-up of fat cells in the legs, thighs and buttocks (NHS Choices, 2014).

What are the most common signs and symptoms of lipoedema?

Lipoedema most commonly causes the legs to become enlarged from the ankles up to the hips. Usually, both legs are enlarged at the same time and to the same extent. However, lipoedema does not affect the feet, and this creates a ‘bracelet’ effect or ‘band-like’ appearance just above the ankles. The hands are not usually affected either, but on occasion, the arms can be.

As well as becoming enlarged, affected areas of the body may also:

  • Feel soft, ‘doughy’ and cold.
  • Be tender to touch.
  • Bruise easily.
  • Ache or feel painful.
  • Have small broken veins under the skin.

Sometimes, lipoedema can lead to LYMPHOEDEMA.

What causes lipoedema?

The cause of lipoedema is unknown, but:

  • There is sometimes a family history of the condition. This means that inherited genes probably play a role.
  • Lipoedema often starts at puberty or at times of hormonal change, e.g. pregnancy or menopause. This suggests that hormones may have an influence.
  • The accumulation of fat tends to be worse in people who are obese, but the condition is not caused by obesity and can affect people who are of a normal body weight.

Please note that lipoedema should not be mistaken for obesity, and dieting often makes little difference to the condition.

What is lipo-lymphoedema?

When a patient develops both lipoedema and lymphoedema, this is known as lipo-lymphoedema, i.e. having lipo-lymphoedematous skin.

How many cases of  NDPL have been reported?

By June 2014, only 13 cases of NDPL had been reported in medical literature (Park et al, 2014). In 2016, an additional case was reported in a 44-year-old morbidly obese female patient that was confined to an area of post-mastectomy lipo-lymphoedema (Ainechi and Carlson, 2016). The patient was also receiving dasatinib therapy for chronic myelogenous leukaemia which was in remission.

What are the symptoms of NDPL?

The symptoms of NDPL are the same as for Sweet’s syndrome. However, the patient is less likely to develop a fever (Park et al, 2014). Read more about the symptoms of Sweet’s syndrome here.

How is it diagnosed?

NDPL is diagnosed in the same way as Sweet’s syndrome – via blood tests and biopsy (sample of a lesion). However, a high white cell count or neutrophil (an inflammatory cell and a type of white blood cell) count is less likely to be found (Park et al, 2014). Read more about diagnosing Sweet’s syndrome here.

Ideally, more than one biopsy should be taken (including one taken several weeks after the first), as results may change (Ainechi and Carlson, 2016). In the 2016 case of NDPL, initial biopsy results suggested neutrophilic urticarial dermatitis (also known as neutrophilic dermatitis with systemic inflammation), but a later biopsy indicated Sweet’s syndrome.

Vasculitis (inflammation of the vessels) is not normally present in Sweet’s syndrome, but may be present in NDPL (Ainechi and Carlson, 2016). However, when vasculitis has occurred in Sweet’s syndrome and some of its variants, there has been debate over whether or not this is a true vasculitis or is caused by neutrophils releasing noxious substances and severe inflammation (Kaur et al, 2015).

How is it treated?

NDPL is treated in the same way as Sweet’s syndrome, and the main form of treatment is corticosteroid (steroid) medication, e.g. prednisone or prednisolone (Ainechi and Carlson, 2016; Park et al, 2014). Occasionally, steroid creams may be used. Read more about treating Sweet’s syndrome here.

Sometimes, antibiotics will be prescribed, due to the increased risk of infection in patients with lymphoedema.

Unfortunately, patients with NDPL may not respond to treatment as well as those with Sweet’s syndrome. This is probably because of problems associated with poor lymphatic drainage, i.e. the lymphatic system has a reduced ability to get rid of fluid, waste products, bacteria or viruses (Ainechi and Carlson, 2016; Park et al, 2014). This means that the presence of a bacteria or virus, or certain cytokines in the lymphatic fluid attracting and increasing the number of neutrophils (the main type of inflammatory cell involved in Sweet’s syndrome) could trigger the patient’s condition (see Hypersensitivity Reaction and Cytokine Dysregulation).

Further information – Sweet’s syndrome affecting lymphoedematous lower limbs.

In 2014, one case of Sweet’s syndrome affecting the lymphoedematous leg of a female patient after a hysterectomy (surgical removal of the womb) and pelvic lymph node dissection for cervical cancer was documented in medical literature (Park et al, 2014). In 2016, another two cases of Sweet’s syndrome on lymphoedematous lower limbs were reported: after hysterectomy, pelvic lymph node dissection and radiotherapy for cervical cancer; after radiotherapy for malignant melanoma (skin cancer) on the right groin (Chu et al, 2016).

As a result of Sweet’s syndrome having the potential to affect any lymphoedematous limb, it has been proposed that the term NDPL should be replaced with neutrophilic dermatosis on the lymphoedematous area (NDL) or lymphoedema-associated neutrophilic dermatosis (Chu et al, 2016; Park et al, 2014).

References.

Ainechi, S. and Carlson, J. (2016) Neutrophilic Dermatosis Limited to Lipo-Lymphedematous Skin in a Morbidly Obese Woman on Dasatinib Therapy. The American Journal of Dermatopathology, Feb;38(2):e22-6 (PubMed).

Chu, C., Cheng, Y., Kao, H.,  Liang, C., Chan, J., and Yu, Y. (2016) Lymphedema-associated neutrophilic dermatosis: Two cases of localized Sweet syndrome on the lymphedematous lower limbs. The Journal of Dermatology, Apr 21 (PubMed).

Kaur, S., Gupta, D., Garg, B. and Sood, N. (2015) Neutrophilic dermatosis of dorsal hands. Indian Dermatology Online Journal, Jan-Feb;6(1):42-5.

NHS Choices (2014) Lipoedema (online). Updated 11/08/14, and accessed 4/04/17.

NHS Choices (2016) Lymphoedema (online). Updated 13/11/16, and accessed 4/014/17.

Park, J., Kang, H. and Kim, Y. (2014) Neutrophilic Dermatosis Confined to the Lymphedematous Area. Annals of Dermatology, Jun; 26(3): 411–413 (online).

© 2012-2017 Sweet’s Syndrome UK