Sometimes, Sweet’s syndrome can occur in an unusual form, and this is known as a disease variant.
What is subcutaneous Sweet’s syndrome?
Sweet’s syndrome affects the skin, but subcutaneous Sweet’s syndrome, otherwise known as Sweet’s panniculitis, is a rare form of Sweet’s syndrome where the subcutaneous tissue, the fatty tissue under the skin, becomes inflamed. In some people with subcutaneous Sweet’s syndrome, both the skin and fatty tissue are affected at the same time. This has led to debate over whether or not inflammation that only affects the fatty tissue and inflammation that affects both the skin and fatty tissue should be classified separately (Oakley, 2015).
Sometimes, subcutaneous Sweet’s syndrome can develop secondary to other conditions or be triggered by something. When this happens, it’s most commonly associated with a group of blood cancers known as myeloid disorders, e.g. myelodysplastic syndromes, but has also occurred in association with chronic lymphocytic leukaemia and other cancers, infection, and medication (Fernandez-Torres et al, 2014; Hashemi et al, 2016; Kim et al, 2010; Tan et al, 2018; Wang et al, 2014). In Oct 2018, a case of pregnancy-associated subcutaneous Sweet’s syndrome was reported in a woman who was 29 weeks pregnant (Byrne et al, 2018).
What does it look like?
The skin lesions caused by subcutaneous Sweet’s syndrome can look like coin-sized red areas or red circular bumps that appear under the skin, but may be deep and ulcerate and ‘discharge oily material’ (Oakley, 2015). The lesions can be tender or very painful, and commonly appear on the extremities, e.g. arms, legs, ankles, or soles of the feet, but can develop on any part of the body (Fernandez-Torres et al, 2014; Kim et al, 2010; Wang et al, 2014). When the inflammation has settled, a temporary or permanent depression may be left in the skin.
Can subcutaneous Sweet’s syndrome be confused with other conditions?
Yes, subcutaneous Sweet’s syndrome can be confused with infectious panniculitis, cellulitis, but more commonly, the skin condition erythema nodosum.
How is it diagnosed?
What kind of biopsy is needed?
An incisional and not a punch biopsy is often needed to accurately diagnose subcutaneous Sweet’s syndrome. This is because a punch biopsy doesn’t always provide an adequate sample as it doesn’t penetrate deeply enough.
Will I only need one biopsy?
You may need more than one biopsy, and if possible, biopsies should be taken from different lesions on different parts of the body. This is because Sweet’s syndrome or subcutaneous Sweet’s syndrome can appear at the same time as erythema nodosum or one condition can appear after the other (Kaur et al, 2015; Qiao et al, 2015). However, when this happens it hasn’t been determined whether or not this is always a true erythema nodosum or an erythema nodosum-like manifestation of Sweet’s syndrome (Kaur et al, 2015).
What will the biopsy show?
The biopsy will normally show neutrophil/neutrophil-rich lobular or septal panniculitis without vasculitis, i.e. lots of white blood cells called neutrophils and no inflammation of the vessels in the fatty tissue under the skin.
How is it treated?
Subcutaneous Sweet’s syndrome is treated in the same way as other forms of Sweet’s syndrome. In July 2016, the biological therapy, adalimumab, was used to successfully treat persistent subcutaneous Sweet’s syndrome (Agarwal et al. 2016). Read more about treatment here.
Another Sweet’s syndrome variant is subcutaneous histiocytoid Sweet’s syndrome, which has been associated with Crohn’s disease and acute myeloid leukaemia (Fernandez-Torres et al, 2014; Srisuttiyakorn et al, 2014). Read more here.
Byrne, B., Schaler, L., DeMaio, A., Ryan, K., Regan, C. and Lynch, C. (2018) 420. A case report of Maternal Sweet’s syndrome: A rare complication of pregnancy. Pregnancy Hypertension, Oct, 13(Suppl 1):S150 (ScienceDirect).
Fernandez-Torres, R., Castro, S., Moreno, A., Alvarez, R. and Fonseca, E. (2014) Subcutaneous histiocytoid sweet syndrome associated with crohn disease in an adolescent. Case Reports in Dermatological Medicine, March 26th (PMC).
Hashemi, S., Fazeli , S., Vahedi, A. and Golabchifard, R. (2016) Rituximab for refractory subcutaneous Sweet’s syndrome in chronic lymphocytic leukemia: A case report. Molecular and Clinical Oncology, Mar;4(3):436-440 (PMC).
Qiao, J., Wang, Y., Bai, J., Wu, Y. and Fang, H. (2015) Concurrence of Sweet’s Syndrome, Pathergy Phenomenon and Erythema Nodosum-Like Lesions. Anais Brasileiros de Dermatologia, Mar-Apr;90(2):237-9 (PMC).
Srisuttiyakorn, C., Reeve, J., Reddy, S., Imaeda, S. and Lazova, R. (2014) Subcutaneous histiocytoid Sweet’s syndrome in a patient with myelodysplastic syndrome and acute myeloblastic leukemia. Journal of Cutaneous Pathology, May;41(5):475-9 (PubMed).
Wang, H., Huang, Y., Chen, W. and Chiang Y. (2014) Subcutaneous Sweet syndrome associated with disseminated nontuberculous mycobacterial infection leading to the diagnosis of adult-onset immunodeficiency. Journal of the American Academy of Dermatology, Jul;71(1):e20-2 (online).
2012-present, Sweet’s Syndrome UK