Neutrophilic dermatosis of the hand ndh is a rare localized variant of the syndrome, originally described two decades ago by strutton et al. Sweets syndrome ss, or acute febrile neutrophilic dermatosis is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, welldemarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination the syndrome was first described in 1964 by robert douglas sweet. Rare cases of a nonbullous neutrophilic dermatosis occurring in patients with lupus erythematosus le have been reported, often as the presenting manifestation of the disease. Photoexposed area involvement in acute febrile neutrophilic dermatosis is uncommon and has rarely been described in literature. Case report periodic fever and neutrophilic dermatosis. Neutrophilic and eosinophilic dermatosisstateautocollapse shows the template collapsed to the title bar if there is a.
When on the lower extremities, this form can resemble erythema nodosum. Omim entry % 608068 neutrophilic dermatosis, acute. Neutrophilic dermatosis may have various clinical presentations, but these all share common histopathological manifestations with an aseptic infiltrate of polymorphonuclear neutrophils. Sweets syndrome acute febrile neutrophilic dermatosis is a rare skin disorder characrerized by fever, neutrophilic leucocytosis, and erythematous skin nodules. Acute febrile neutrophilic dermatosis, also termed sweet syndrome, is a reactive process characterized by the abrupt onset of tender, redtopurple papules, and nodules that coalesce to form plaques. Sweet syndrome is a rare disorder characterized by fever and the sudden onset of a.
Acute febrile neutrophilic dermatosis sweets syndrome sweets syndrome ss is a rare skin disorder characterised by the abrupt development of tender, red papules and nodules that coalesce to form plaques. Sweets syndrome revealed by oral pustulosis citeseerx. Firstline treatment of acute neutrophilic conditions is steroid therapy. Rheumatoid neutrophilic dermatosis thursday, may 23. The following is a list of the betterknown types of neutrophilic dermatoses, it is by no means comprehensive. The prototype of the neutrophilic dermatoses is sweet syndrome acute febrile neutrophilic dermatosis. The neutrophilic process itself may cause systemic illness. Neutrophilic dermatoses are significantly associated with systemic disease. It commonly affects the dorsum of the hand and often mimics infection, with violaceous inflammatory papules and plaques that may ulcerate. Neutrophilic and eosinophilic dermatosis wikipedia. Neutrophilic dermatoses often arise at the site of injury such as a needle prick, biopsy or insect bite. Neutrophilic dermatosis on postmastectomy lymphedema ndpl is a newly suggested disease by demitsu and tadaki3 it is also referred to as localized sweets syndrome ss because of the histological similarities between the two conditions4.
Sweets syndrome ss, or acute febrile neutrophilic dermatosis is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, welldemarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination. Apr 09, 2020 beside the extracutaneous neutrophilic manifestations described previously, some autonomous diseases, which may occur in the absence of any context of neutrophilic dermatoses, may be associated. The authors conclusion and the editorial by cohen2 tend to merge this condition with sweet syndrome ss. Neutrophilic dermatoses part i dermatoses neutrofilicas parte i renan rangel bonamigo1 fernanda razera2 gislaine silveira olm3 abstract.
They arise in reaction to some underlying systemic illness. In the absence of infection of the skin or neoplasia, a diagnosis of neutrophilic dermatosis and panniculitis, resembling the subcutaneous form of acute febrile neutrophilic dermatosis, was made. Clinical mediterranean ancestry, especially nonashkenazi sephardic jews, armenians, and arabs begin between ages 515. Neutrophilic dermatoses nd are inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes. Neutrophilic dermatoses nd are a polymorphous group of noncontagious. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Neutrophilic dermatosis of the hands ndh is a recently described disorder of which we present two cases and discuss the aetiopathogenic link to sweets. Pdf neutrophilic dermatoses and their implication in. Acute febrile neutrophilic dermatosis sweet syndrome at a glance. The main clinical forms of nd include sweet syndrome, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and their atypical or transitional forms. Neutrophilic dermatoses and their implication in pathophysiology of asthma and other respiratory comorbidities. The etiopathogenesis of neutrophilic dermatoses has continuously evolved. Pathology outlines neutrophilic dermatosis associated.
Pemphigus in 1897 duhring stated that dermatitis herpetiformis. Neutrophilic dermatosis of the hands is an inflammatory skin condition related to sweet syndrome that responds to corticosteroids. Neutrophilic dermatoses primary care dermatology society. The plaques usually occur on the upper extremities, face, or neck and are typically accompanied by fever and peripheral neutrophilia.
Neutrophilic dermatoses nd are a polymorphous group of noncontagious dermatological disorders that share the common histological feature of a sterile cutaneous infiltration of mature neutrophils. Clinical features of neutrophilic dermatosis variants resembling. Both diagnoses are characterized by an acute onset of fever, leukocytosis, and tender, erythematous infiltrated plaques. After intensive care, three surgical wound debridements and wound adaptations, the wound healed by secondary intention within ten weeks. Acute febrile neutrophilic dermatosis resulting from a hematologic cancer can cause a subcutaneous form, typically with 2 to 3cm erythematous nodules, commonly involving the extremities. Nov 18, 2015 chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature, also known as candle syndrome, is a rare autoinflammatory condition. Neutrophilic dermatosis of the dorsal hand wounds research. Ask12 signaling promotes inflammation in a mouse model of neutrophilic dermatosis sarang tartey, 1 prajwal gurung,1,2 tejasvi krishna dasari, amanda burton, 1 and thirumaladevi kanneganti 1department of immunology, st. These include hematological disorders, inflammatory bowel disease, joint immunological disorders, and other malignant or inflammatory conditions. They may arise at the site of injury such as a needle prick, biopsy or insect bite. Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. They tend to grow slowly, eventually joining together coalescing to form. Acute febrile neutrophilic dermatosis is a skin condition characterized by fever, inflammation of the joints arthritis, and painful skin lesions that appear mainly on the face, neck, back and arms. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext.
We believe, however, that the reported features of nddh. Sweets syndrome the eponym for acute febrile neutrophilic dermatosis is. Often there is the need to combine two drugs for better control of the disease. Aug 26, 2014 neutrophilic dermatoses constitute a heterogeneous group of dermatologic diseases, which are unified by the predominance of neutrophils within the inflammatory infiltrate on histopathology. The lesions mostly occur in the upper extremity, neck and face region.
Reactive neutrophilic dermatoses are a spectrum of conditions mediated by neutrophils, and. Sweets syndrome the eponym for acute febrile neutrophilic dermatosis. Sweet syndrome acute febrile neutrophilic dermatosis is an uncommon inflammatory disorder characterized by the abrupt appearance of. Nov 04, 2016 acute febrile neutrophilic dermatosis is a skin condition characterized by fever, inflammation of the joints arthritis, and painful skin lesions that appear mainly on the face, neck, back and arms. Dermatosis is a condition that affects the skin, nails, or hair. A heterogeneous but linked spectrum diseases, with significant overlapping histopathologic findings and similar pathogenic mechanisms and therapeutic approaches. Sweets syndrome presents in three clinical settings. We read with interest the article by dicaudo and connolly1 reporting 7 cases of neutrophilic dermatosis of the dorsal hands nddh. Acute febrile neutrophilic dermatosis dermatologic. Acute febrile neutrophilic dermatosis genetic and rare. Neutrophilic dermatoses are conditions characterized by a dermal.
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature, also known as candle syndrome, is a rare autoinflammatory condition. Neutrophilic dermatoses primary care dermatology society uk. The skin lesions are rapidly evolving juicy often pseudovesicular plaques or nodules accompanied by fever, leukocytosis, conjunctivitis and arthralgia. Above are all associated with vascular injury, typically with prominent extravascular neutrophilic or histiocytic dermal infiltrate. A case of neutrophilic dermatosis of the dorsal hands with. Pdf sweets syndrome the eponym for acute febrile neutrophilic dermatosis is characterized by a constellation of clinical symptoms, physical. Acute febrile neutrophilic dermatosis dermatologic disorders. It frequently occurs with underlying cancer, especially hematologic cancers. Nodular scleritis and sweet syndromenoduler sklerit ve sweet sendromu. In 7 of the patients the condition was preceded by an illness, usually infective. Sweet syndrome acute febrile neutrophilic dermatosis uptodate. We describe 7 patients that developed urticarial lesions in the context of a known or concurrently diagnosed autoimmune connective tissue disease. Neutrophilic dermatoses constitute a heterogeneous group of dermatologic diseases, which are unified by the predominance of neutrophils within the inflammatory infiltrate on histopathology.
Neutrophilic dermatoses and their implication in pathophysiology. Aug 21, 2012 sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. Although middleaged women are most likely to develop this condition, it may also affect men, older adults and even infants. Sweet 1964 described 8 patients, all women, with an acute dermatosis characterized by fever, neutrophilia, painful erythematous plaques, a dense dermal infiltrate of mature neutrophils, and rapid response to corticosteroids. This reaction to injury is known as koebner phenomenon, or isomorphic response. Acute febrile neutrophilic dermatosis or sweet disease presents acutely as a rash accompanied by systemic symptoms. Pyoderma gangrenosum and sweets syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. Sweets syndrome is a neutrophilic dermatosis, characterised by fever, peripheral neutrophil leucocytosis and the acute onset of painful, erythematous papules, nodules or plaques. With febrile neutrophilic dermatosis red tender plaques on the skin suddenly appear. Sweet syndrome, pyoderma gangrenosum, behcets disease and neutrophilic urticaria. We propose the term neutrophilic urticaria with systemic inflammation nusi for the disease in these patients. Omim entry % 608068 neutrophilic dermatosis, acute febrile.
Chronic atypical neutrophilic dermatosis with lipodystrophy. Neutrophilic dermatoses are autoinflammatory skin conditions characterised by dense infiltration of inflammatory cells neutrophils in the affected tissue. Clinical manifestations can vary from nodules, pustules, and bulla to erosions and ulcerations. It is more common amongst women and patients who have severely sero positive rheumatoid arthritis ra.
The cause of sweet syndrome can vary, from certain types of cancer to an infection or medication. Overlapping cutaneous disorders related to dermatitis. Recently, these disorders have been known as neutrophilic diseases. Another term for a dermatosis is a cutaneous condition. A case of neutrophilic dermatosis of the dorsal hands with concomitant involvement of the lips vol. A neutrophilic dermatosis may be seen in isolation or more than one type may occur in the same individual. Also called acute febrile neutrophilic dermatosis abrupt onset of tender or painful erythematous plaques and nodules on the face and extremities and less commonly on the trunk, in association with fever usually, malaise and a neutrophil leukocytosis. Sweets syndrome is a neutrophilic dermatosis, characterised by fever, peripheral neutrophil leucocytosis and the acute onset of painful, erythematous papules, nodules or. Jun 06, 20 neutrophilic dermatosis of the dorsal hand nddh is a rare condition characterized by painful pustule ulcerations usually localized to the dorsal and lateral aspect of the hand, however additional lesions may also present elsewhere. Pyoderma gangrenosum and sweet syndrome with prominent systemic inflammation define a subset of neutrophilic dermatoses, termed. Inflammation in the lining of the abdominal cavity, chest cavity, skin, or joints occurs high fevers usually peak in 12 to 24 hours attacks vary in severity of symptoms.
A tender, erythematous, welldemarcated plaques on the dorsum of the right hand. The term neutrophilic dermatoses includes a rare inflammatory pattern characterized by neutrophilrich cutaneous infiltrate. Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of sweets syndrome. Sweet syndrome, pyoderma gangrenosum, behcets disease and. In the periodic fever and neutrophilic dermatosis, the rheumatologist should search for sterile abscesses in other organs. The lesions of ndh and sweet syndrome are similar, as indicated in the first report of ndh. A tender, erythematous, welldemarcated plaques on the dorsum of. Acute febrile neutrophilic dermatosis sweet syndrome is characterized by a constellation of symptoms and findings. Neutrophilic dermatoses as systemic diseases sciencedirect. Rheumatoid neutrophilic dermatosis rnd is a rare and distinct manifestation of rheumatoid arthritis. Neutrophilic dermatosis article about neutrophilic. Occurs in 14 44% of patients with crohns disease includes pyoderma gangrenosum, erythema nodosum and socalled metastatic crohns disease nonnecrotizing sarcoidlike granulomatous dermatitis hum pathol 2003. This new term is intended to describe a spectrum of diseases that includes neutrophilic urticaria and highlights the role of il1 in driving this particular inflammatory process.
Acute febrile neutrophilic dermatosis is characterized by tender, indurated, darkred papules and plaques with prominent edema in the upper dermis and dense infiltrate of neutrophils. Introduction neutrophilic dermatosis nd is a group of disorders with intense neutrophilic in ltration in the skin and extracutaneous involvement. Neutrophilic dermatosis confined to the lymphedematous area. They share several characteristics and respond to immunosuppressives. Also called acute febrile neutrophilic dermatosis, sweets syndrome ss is. Signs and symptoms generally develop during the first year of life and may include recurrent fevers, purpura, swollen eyelids, joint pain, contractures, developmental delay and progressive. Acute febrile neutrophilic dermatosis sweet syndrome. Large, tender, raised dark red plaques occurred most commonly on the face, neck, arms.
Mgus, the pustular eruption which merged to form annular pat. The lesions are most often located asymmetrically on the upper extremities, neck and face and may be painful. The neutrophils are usually located within the dermis in sweet syndrome and pyoderma gangrenosum. Pdf a case of rheumatoid neutrophilic dermatosis omer. Sweet syndrome nord national organization for rare disorders. We report a case of photodistributed sweets syndrome in a middle aged lady who responded to symptomatic treatment with nsaid and systemic antibiotic. Jan 22, 2010 febrile neutrophilic dermatosis is known as sweets syndrome. Reactive neutrophilic dermatoses are a spectrum of conditions mediated by neutrophils, and typically associated with underlying diseases, such as inflammatory bowel disease and hematologic malignancy. Tagami et al found a pmn chemotactic factor in a patients skin. Acral manifestations of sweet syndrome neutrophilic. Acute febrile neutrophilic dermatosis sweet syndrome this skin condition is marked by a fever and skin lesions that are usually reddish to blue or violet in color.
302 1176 1414 1244 831 254 232 1059 484 1415 1519 1423 1153 1211 1591 892 487 407 1325 356 962 1032 418 290 1568 173 1018 1691 1548 24 402 1005 721 305 541 463 220 362 838 1182 684 75 19 184 374 735 291 302 672 161 1450