Quiz | Dermatol Pract Concept 2011;1(1):8 29 From the Dermatologikum Hamburg: Quiz Almut Böer-Auer, M.D.1, Alexandra Gust, M.D.1 1Dermatologikum Hamburg Citation: Böer-Auer A, Gust A. From the Dermatologikum Hamburg: Quiz. Dermatol Pract Concept 2001;1(1):8. http://dx.doi. org/10.5826/dpc.0101a08. Copyright: ©2011 Böer-Auer et al. This is an an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Corresponding author: Almut Böer-Auer, M.D., Dermatologikum Hamburg, Drehbahn 1-3, 20354 Hamburg, Germany. Email: boer@dermatologikum.de. the patient A 61-year-old woman presented herself with a chronic and widespread eruption of erythematous papules, pustules, ulcers, and hyperpigmented scars (Figures 1A–E). She had a history of rheumatoid arthritis. A biopsy specimen was taken from a plaque on the leg (Figures 2A–P). What is your diagnosis? answer and explanation rheumatoid neutrophilic dermatitis The section pictured in Figures 2A–P shows irregular epi- dermal and infundibular acanthosis and dilation of infun- dibula filled with neutrophils. Diffuse infiltrates are pres- ent in the dermis and are dominated by far by neutrophils. Some nuclear dust of neutrophils is present but there is no deposition of fibrin in the vessel walls. Fibrosis is seen in the deeper dermis. In the absence of clinical information, the best interpretation of the changes in this section is pyoderma gangrenosum. The clinical pictures (Figures 1A–E), however, illustrate an extensive eruption that consists of erythematous pap- ules, pustules, ulcers, and hyperpigmented scars. The clinical appearance taken together with the history of severe rheu- matoid arthritis enables a diagnosis of rheumatoid neutro- philic dermatitis to be made based on clinicopathological correlation. rheumatoid neutrophilic dermatitis (rND) was first described by Ackerman in 1978 [1]. It appears in adults of both sexes with severe rheumatoid arthritis. Lesions are typi- cally distributed symmetrically over joints of the elbows and knees. Individual lesions are papules, plaques and nodules, pustules, ulcerations, and scars. Urticarial lesions also have been observed in some patients [1–3]. Lesions come and go slowly over months or years [1]. rND is associated invariably with severe rheumatoid arthritis. Even though first described only in patients with high titers of rheumatoid factor, it was then shown to appear also in patients who are negative for a circulating rheuma- toid factor [4]. Histopathologic changes have been described as a heavy dermal infiltrate of neutrophils with variable degrees of leukocytoclasis but no vasculitis [1, 4]. Fibrinoid degenera- tion of collagen, resembling the collagen changes present in rheumatoid nodules in a miniaturized form also has been reported [5]. The pattern of rheumatoid neutrophilic dermatitis bears many attributes in common with Sweet’s syndrome, on one hand, and pyoderma gangrenosum, on the other. Ackerman suggested that rheumatoid neutrophilic dermatitis could be a synonym for Sweet’s syndrome in a patient with severe rheu- matoid arthritis because one of the conditions known to be responsible for Sweet’s syndrome is rheumatoid arthritis [6]. In contrast, association of rheumatoid neutrophilic dermati- tis with pyoderma gangrenosum has been described [7]. In the patient presented here, some lesions were indistinguish- able clinically from pyoderma gangrenosum, while typical lesions of Sweet syndrome were lacking. In clinicopathologi- Dermatology PrACTICAL & CoNCEPTUAL www.derm101.com 30 Quiz | Dermatol Pract Concept 2011;1(1):8 Figures 1a–e. Clinical appearance of the widespread eruption of erythematous papules, pustules, ulcers, and hyperpigmented scars. A C D E B cal correlation, rND seems to be distinct and different from both Sweet’s syndrome and pyoderma gangrenosum. rheumatoid arthritis is a chronic inflammatory arthritis that relatively commonly shows extra-articular manifesta- tions, especially in the skin. rheumatoid nodules, rheuma- toid vasculitis, pyoderma gangrenosum, interstitial granulo- matous dermatitis with arthritis, palisaded neutrophilic and granulomatous dermatitis, and rheumatoid neutrophilic der- matitis have been reported to be associated with the condi- tion [8]. In comparison with other cutaneous manifestations of the disease, however, rheumatoid neutrophilic dermatitis is a very rare skin manifestation in patients with rheuma- toid arthritis. In a relatively recent study of 215 patients with rheumatoid arthritis, only two were diagnosed with rheuma- toid neutrophilic dermatitis [9]. Treatment of rheumatoid neutrophilic dermatitis is often difficult. Skin lesions do not respond necessarily to systemic treatment of the arthritis. Usually, dramatic response to sys- temic corticosteroids is seen. However, lesions quickly reap- pear on withdrawal of that medication. references 1. Ackerman AB. Histologic Diagnosis of Inflammatory Skin Dis- eases. A Method by Pattern Analysis. Philadelphia: Lea & Febiger, 1978:437. 2. Brown TS, Fearneyhough PK, Burruss JB, et al. rheumatoid neu- trophilic dermatitis in a woman with seronegative rheumatoid arthritis. J Am Acad Dermatol 2001;45(4):596–600. 3. Ichikawa MM, Murata Y, Higaki Y, et al. rheumatoid neutro- philic dermatitis. Eur J Dermatol 1998;8(5):347–9. 4. Bevin AA, Steger J, Mannino S. rheumatoid neutrophilic derma- titis. Cutis 2006;78(2):133–6. 5. Lazarov A, Mor A, Cordoba M, et al. rheumatoid neutro- philic dermatitis: an initial dermatological manifestation of se- ronegative rheumatoid arthritis. J Eur Acad Dermatol Venereol 2002;16(1):74–6. 6. Ackerman AB, Böer A, Benin B, et al. Histologic Diagnosis of Inflammatory Skin Diseases. An Algorithmic Method Based on Pattern Analysis, 3rd Edition. New York City: Ardor Scribendi, Ltd., 2005. 7. MacAya A, Servitje o, Jucglà A, et al. rheumatoid neutrophilic dermatitis associated with pyoderma gangrenosum. Br J Derma- tol 2000;142(6):1246–8. 8. Sayah A, English JC 3rd. rheumatoid arthritis: a review of the cutaneous manifestations. J Am Acad Dermatol 2005;53(2):191– 209. 9. Ergun T, Inanc N, Tuney D, et al. Skin manifestations of rheu- matoid arthritis: a study of 215 Turkish patients. Int J Dermatol 2008;47(9):894–902. Quiz | Dermatol Pract Concept 2011;1(1):8 31 Figures 2a–P. Histopathology. A B E H J N M C F I K O D G L P