|LETTER TO THE EDITOR
|Year : 2013 | Volume
| Issue : 6 | Page : 824-826
Recurrent lymphocytic Sweet's syndrome
Kunnummal Muhammed, Khader Anza, Betsy Ambooken, Puravoor Jayasree
Department of Dermatology and Venereology, Government Medical College, Calicut, Kerala, India
|Date of Web Publication||29-Oct-2013|
Kunnummal House, Koroth School Road, Vatakara, Calicut - 673 101, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Muhammed K, Anza K, Ambooken B, Jayasree P. Recurrent lymphocytic Sweet's syndrome. Indian J Dermatol Venereol Leprol 2013;79:824-6
|How to cite this URL:|
Muhammed K, Anza K, Ambooken B, Jayasree P. Recurrent lymphocytic Sweet's syndrome. Indian J Dermatol Venereol Leprol [serial online] 2013 [cited 2020 Oct 29];79:824-6. Available from: https://www.ijdvl.com/text.asp?2013/79/6/824/120743
Sweet's syndrome (SS), also known as acute febrile neutrophilic dermatosis or Gomm-Button disease is characterized by fever; acute onset of painful, erythematous or plum-colored papules, plaques or nodules; peripheral neutrophil leukocytosis; and a dense neutrophilic infiltrate on histology, according to the classical description.  But rare histological presentations with lymphocytic and histiocytic infiltrate in patients with myelodysplasia have been reported. 
Here, we report a case of recurrent skin eruption resembling SS associated with dermal lymphocytic infiltrate without underlying hematological abnormality.
A 40-year-old female presented with recurrent episodes of red raised tender lesions over face, neck, forearms, and upper back of trunk of 8 years duration. Each episode was associated with high grade and continuous fever. There was no history of malar rash or arthralgia or photosensitivity. Each time she was treated with systemic corticosteroids, lesions responded completely, but recurred after a symptom free interval of 6-9 months.
Cutaneous examination revealed multiple plum-colored and erythematous edematous tender plaques of size ranging from 1 × 1.5 to 3 × 4 cm size over face, neck, upper chest, upper back, and bilateral forearms with some lesions showing pseudovesiculation [Figure 1] and [Figure 2]. No mucosal lesions were present. Systemic examination was within normal limits. The pathergy test was positive. Hematological examination showed leukocytosis with neutrophilia and raised erythrocyte sedimentation rate (ESR). Peripheral smear examination showed neutrophilia with toxic granules. Liver and renal function tests were normal. Antinuclear antibody (ANA), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), and cytoplasmic-ANCA (c-ANCA) were negative; antistreptolysin O (ASO) titer was within normal range. Skin biopsies were taken during each episodes of exacerbation (a total of five biopsies during 8 years of disease course) and review of all slides consistently showed dense lymphocytic infiltrate in the dermis [Figure 3]a and b, with few areas of perivascular pattern and no basal cell degeneration. Immunohistochemistry showed CD3 +ve [Figure 3]c], CD20 −ve cells confirming T cell lineage with no other or atypical cells. Direct immunoflourescence (DIF) did not reveal any complement or immunoglobulin deposits in the vessels or dermoepidermal junction. Bone marrow aspiration studies done repeatedly were of normal cytology.
|Figure 3: (a) Low power views showing dense dermal lymphocytic infiltrate (H and E, ×40). (b) High power view showing dermal lymphocytic infiltrate (H and E, ×400). (c) Most of the cells in the infiltrate are positive for CD3 (immunohistochemistry, ×100)|
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Chest radiogram, ultrasonographic studies of abdomen and pelvis, and neurological evaluation were unremarkable. A detailed literature search was made, and based on the aforementioned findings a diagnosis of recurrent lymphocyte predominant SS was made and the present episode was managed with systemic steroids. The lesions completely subsided and she is presently under follow-up.
Dr. Robert Douglas Sweet first described this entity in eight women with a "distinctive and fairly severe illness" in 1964.  The name Gomm-Button disease referred to the initial two patients with this condition.  Histopathological description of SS is a diffuse dermal infiltrate of mature neutrophils. Several studies suggest that there may be an admixture of lymphocytes and/or eosinophils, either of which can be prominent in some cases. 
A unique clinicopathologic subset of nine SS patients has recently been recognized where clinically diagnosed SS skin lesions showed an initial lymphocytic infiltrate in the dermis (initial SS) and late neutrophilic infiltrate (late SS) after 24-96 months. All these patients developed myelodysplastic syndrome later on.  Literature search revealed similar reports of lymphocytic infiltrate occurring 2 months to 4 years prior to classical neutrophilic infiltrate. ,,
'Histiocytoid SS' is a recently described histological variant of SS with infiltration of histiocyte-like cells into the upper dermis. These cells are considered as immature myeloid cells from the bone marrow in early acute stage. 
In our case, the clinical presentation during each episode was typical of SS, but with dense dermal lymphocytic infiltrate. The possibilities of Jessner's lymphocytic infiltrate (JLI), polymorphic light eruption (PMLE), and lupus erythematosus (LE) were ruled out with the characteristic clinical appearance, presence of fever, absence of basal cell degeneration in biopsy, and negative DIF. The bone marrow cytology was normal repeatedly. Unlike the previous reports, in our case there was no evidence of myelodysplasia or hematological malignancy or other systemic illnesses even after detailed evaluation and the dermal infiltrate has remained lymphocytic for 8 years. The presence of neutrophils in the dermis might herald an underlying malignant transformation and persistence of lymphocytes for years point to a benign nature of disease.
We propose that there can be an atypical lymphocytic SS with typical clinical features which may recur, not associated with myelodysplasia or hematological malignancy and need to be considered as a distinct entity. No report of such a rare presentation is available from India.
| References|| |
|1.||Cox NH, Jorizzo JL, Bourke JF, Savage CO. Vasculitis, neutrophilic dermatoses and related disorders. In: Burns T, Breathnack S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8 th ed. Oxford: Blackwell Publishing; 2010. p. 50.74-50.80. |
|2.||Vignon-Pennamen MD, Juilard C, Rybojad M, Wallach M, Daniel MT, Morel P, et al. Chronic recurrent lymphocytic sweet's syndrome as a predictive marker of myelodysplasia: A report of 9 cases. Arch Dermatol 2006;142:1170-6. |
|3.||Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56. |
|4.||Wawrzycki B, Chodorowska S, Pietrzak A, Krasowska DW, Wasik S, Dyblec E, et al. Recurrent skin eruption in patient with chronic lymphocytic leukemia and lymphocytic infiltrates of the dermis resembling Sweet's syndrome. G Ital Dermatol Venereol 2011;146:487-92. |
|5.||Evans AV, Sabroe RA, Liddell K, Russell-Jones R. Lymphocytic infiltrates as a presenting feature of Sweet's syndrome with myelodysplasia and response to cyclophosphomide. Br J Dermatol 2002;146:1087-90. |
|6.||Boeckler P, Noacco G, Maradeix S, Heid E, Lipsker D, Cribier B. Lymphocytic infiltrate of the dermis preceding typical Sweet's syndrome. Ann Dermatol Venereol 2007;134:559-63. |
|7.||Haung CF, Wu BY, Liaw FY, Wang WM, Chiag CP. Histiocytoid Sweet syndrome: Report of two cases and review of the literature. Dermatol Sin 2012;30:71-4. |
[Figure 1], [Figure 2], [Figure 3]