230 Sweet syndrome Karolyn A. Wanat, Joel M. Gelfand and William D. James Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports (Courtsey of Misha Rosenbach MD.) Sweet syndrome is a neutrophilic dermatosis, characterized clinically by multiple painful, well-demarcated, non-scarring erythematous plaques or pustules on the face, neck, upper trunk, and extremities. There can be a pseudovesicular appearance. Fever, leukocytosis, arthralgias, myalgias, headaches, and general malaise may occur. Oral, ocular, and internal organ involvement is rare. On histopathology, there is a diffuse neutrophilic infiltrate in the upper dermis without evidence of primary leukocytoclastic vasculitis. Neutrophilic dermatosis of the dorsal hands (NDDH) is classified as an anatomically limited subset of Sweet syndrome. Management strategy Sweet syndrome is often idiopathic, but may be associated with malignancy (most commonly acute myelogenous leukemia, but also lymphomas, dysproteinemias, and carcinomas), inflammatory bowel disease, infection (commonly Streptococcus or Yersinia), medication, radiation, and pregnancy. Work-up includes skin biopsy, complete physical examination, and laboratory studies. Treatment of the underlying associated condition or discontinuation of the causative medication may lead to resolution of skin lesions. The standard treatment is corticosteroids. For patients with contraindications to corticosteroids, or for therapeutic failures, second-line therapies may be used. Specific investigations History and physical examination Complete blood count with differential Erythrocyte sedimentation rate or C-reactive protein Cultures as indicated by history and physical examination Pregnancy test (in women of childbearing potential) Medication history Sweet’s syndrome revisited: a review of disease concepts. Cohen PR, Kurzrock R. Int J Dermatol 2003; 42: 761–78. An excellent review of the literature. The relationship between neutrophilic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Walling HW, Snipes CJ, Gerami P, Piette WW. Arch Dermatol 2006; 142: 57–63. A report of nine cases of NDDH and review of all 43 cases reported. Histiocytoid Sweet syndrome. Heymann WR. J Am Acad Dermatol 2009; 61: 693–4. The term ‘histiocytoid’ refers to the histiologic presence of immature neutrophils; the clinical implications are identical to those of the classical Sweet syndrome. Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia. Vignon-Pennamen MD, Julliard C, Rybojad M, Wallach D, Danie MT, et al. Arch Dermatol 2006; 142: 1170–6. Nine cases of chronic Sweet syndrome in which the initial biopsies demonstrated a lymphocytic infiltrate with atypical mononuclear cells positive for CD68 and myeloperoxidase, suggesting a myeloid origin; this presentation appeared to be a predictive marker of underlying myelodysplasia. Necrotizing Sweet syndrome. Kroshinsky D, Alloo A, Rothschild B, Cummins J, Tan J, Montecino R, et al. J Am Acad Dermatol 2012; 67: 945–54. Three cases of acute necrotizing neutrophilic dermatosis characterized by deep tissue neutrophilic infiltration and soft tissue necrosis. The authors state that ‘the diagnosis of NSS should be entertained in all cases of suspected necrotizing fasciitis failing to respond to therapy and lacking a causative organism.’ Neonatal Sweet syndrome. Gray PE, Bock V, Ziegler DS, Wargon O. Pediatrics 2012; 129: e1353–9. Sweet syndrome in the neonatal period requires thorough investigation as it can be associated with a serious underlying disorder. First-line therapies Oral corticosteroids C Topical and intralesional corticosteroids C Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: Cat scratch disease Hemangiomas Drug eruptions Erythropoietic protoporphyria Ichthyoses Jellyfish stings Stay updated, free articles. Join our Telegram channel Join Tags: Treatment of Skin Disease Comprehensive Therapeutic Strategies Aug 7, 2016 | Posted by admin in Dermatology | Comments Off on Sweet syndrome Full access? Get Clinical Tree
230 Sweet syndrome Karolyn A. Wanat, Joel M. Gelfand and William D. James Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports (Courtsey of Misha Rosenbach MD.) Sweet syndrome is a neutrophilic dermatosis, characterized clinically by multiple painful, well-demarcated, non-scarring erythematous plaques or pustules on the face, neck, upper trunk, and extremities. There can be a pseudovesicular appearance. Fever, leukocytosis, arthralgias, myalgias, headaches, and general malaise may occur. Oral, ocular, and internal organ involvement is rare. On histopathology, there is a diffuse neutrophilic infiltrate in the upper dermis without evidence of primary leukocytoclastic vasculitis. Neutrophilic dermatosis of the dorsal hands (NDDH) is classified as an anatomically limited subset of Sweet syndrome. Management strategy Sweet syndrome is often idiopathic, but may be associated with malignancy (most commonly acute myelogenous leukemia, but also lymphomas, dysproteinemias, and carcinomas), inflammatory bowel disease, infection (commonly Streptococcus or Yersinia), medication, radiation, and pregnancy. Work-up includes skin biopsy, complete physical examination, and laboratory studies. Treatment of the underlying associated condition or discontinuation of the causative medication may lead to resolution of skin lesions. The standard treatment is corticosteroids. For patients with contraindications to corticosteroids, or for therapeutic failures, second-line therapies may be used. Specific investigations History and physical examination Complete blood count with differential Erythrocyte sedimentation rate or C-reactive protein Cultures as indicated by history and physical examination Pregnancy test (in women of childbearing potential) Medication history Sweet’s syndrome revisited: a review of disease concepts. Cohen PR, Kurzrock R. Int J Dermatol 2003; 42: 761–78. An excellent review of the literature. The relationship between neutrophilic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Walling HW, Snipes CJ, Gerami P, Piette WW. Arch Dermatol 2006; 142: 57–63. A report of nine cases of NDDH and review of all 43 cases reported. Histiocytoid Sweet syndrome. Heymann WR. J Am Acad Dermatol 2009; 61: 693–4. The term ‘histiocytoid’ refers to the histiologic presence of immature neutrophils; the clinical implications are identical to those of the classical Sweet syndrome. Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia. Vignon-Pennamen MD, Julliard C, Rybojad M, Wallach D, Danie MT, et al. Arch Dermatol 2006; 142: 1170–6. Nine cases of chronic Sweet syndrome in which the initial biopsies demonstrated a lymphocytic infiltrate with atypical mononuclear cells positive for CD68 and myeloperoxidase, suggesting a myeloid origin; this presentation appeared to be a predictive marker of underlying myelodysplasia. Necrotizing Sweet syndrome. Kroshinsky D, Alloo A, Rothschild B, Cummins J, Tan J, Montecino R, et al. J Am Acad Dermatol 2012; 67: 945–54. Three cases of acute necrotizing neutrophilic dermatosis characterized by deep tissue neutrophilic infiltration and soft tissue necrosis. The authors state that ‘the diagnosis of NSS should be entertained in all cases of suspected necrotizing fasciitis failing to respond to therapy and lacking a causative organism.’ Neonatal Sweet syndrome. Gray PE, Bock V, Ziegler DS, Wargon O. Pediatrics 2012; 129: e1353–9. Sweet syndrome in the neonatal period requires thorough investigation as it can be associated with a serious underlying disorder. First-line therapies Oral corticosteroids C Topical and intralesional corticosteroids C Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: Cat scratch disease Hemangiomas Drug eruptions Erythropoietic protoporphyria Ichthyoses Jellyfish stings Stay updated, free articles. Join our Telegram channel Join Tags: Treatment of Skin Disease Comprehensive Therapeutic Strategies Aug 7, 2016 | Posted by admin in Dermatology | Comments Off on Sweet syndrome Full access? Get Clinical Tree
Oral corticosteroids
Topical and intralesional corticosteroids
