Palisaded neutrophilic and granulomatous dermatosis
Published Web Locationhttps://doi.org/10.5070/D36v431497
Palisaded neutrophilic and granulomatous dermatosisDepartment of Dermatology, New York University
Noushin Heidary MD, Stephanie Mengden MD, Miriam K Pomeranz MD
Dermatology Online Journal 14 (5): 17
A 22-year-old woman with mixed connective-tissue disease presented with a 5-month history of recurrent episodes of tender, erythematous papules, nodules, and edematous plaques on the upper extremities and thighs. Cutaneous lesions occurred in the setting of livedo reticularis. A biopsy specimen showed interstitial and perivascular inflammation with lymphocytes, macrophages, neutrophils, nuclear dust, collagen alteration, extravasated erythrocytes, and fibrin within small superficial blood vessels. These changes were consistent with a diagnosis of palisaded neutrophilic and granulomatous dermatosis, which is a rare entity that includes a combination of a neutrophilic infiltrate, abnormal or altered collagen, granuloma formation, and leukocytoclastic debris in the context of an immune-mediated collagen vascular or systemic disease. The underlying mechanism remains poorly understood. Treatment options are limited, and resolution of lesions typically occurs within several months to years.
A 22-year-old woman presented to the Dermatology Clinic at Bellevue Hospital Center in the fall of 2006 with a 5-month history of recurrent episodes of painful, tender, red and skin-colored nodules and ill-defined plaques on the arms, left hand, and thighs. The lesions, which were often associated with malaise and low-grade fevers, would last for several days and disappear with hyperpigmented macules. Past medical history included mixed connective-tissue disease. Review of systems included morning stiffness in the proximal interphalangeal and metacarpalphalangeal (MCP) joints, Raynaud phenomenon, and heartburn. No history of oral ulcers, alopecia, photosensitivity, chest pain, or shortness of breath was elicited. Medications included hydroxychloroquine and tolmentin for joint pain. The patient had a past history of being treated with prednisone for 2 years. A biopsy specimen was obtained at the site of a tender nodule near the left first digit of the MCP joint.
On the upper extremities, dorsum of the left hand, and thighs were erythematous, tender nodules and two, ill-defined, edematous, smooth plaques. On the upper extremities, several hyperpigmented macules were noted at the sites of prior lesions. Reticulated erythema was present on the thighs. Mild ulnar deviation of the MCP joints was noted.
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Antinuclear antibody titer was 1:2560 with a speckled pattern. Anti-U1RNP antibody was greater than 6.0. Anti-smooth muscle antibody was positive at 3.58. Rheumatoid factor was 19.8 IU/ml. Anti-Ro/La, anti-double-stranded DNA, anti-cardiolipin, anti-CCP, and anti-SCL-70 antibodies were negative. A complete blood count, basic metabolic panel, hepatic function tests, C3 and C4 levels, and urinalysis were normal. Hepatitis B antigen and C antibody were negative.
Within the superficial and deep dermis, there is a perivascular and diffuse, interstitial, mixed-cell infiltrate composed of histiocytes, neutrophils, neutrophil fragments, and lymphocytes. In some foci, the inflammatory infiltrate is palisaded and associated with basophilic alteration of the collagen. Fragments of neutrophils are present around blood-vessel walls, in which there are extravasated erythrocytes and fibrin deposition.
Palisaded neutrophilic and granulomatous dermatosis (PNGD) is a rare entity that has not been clearly defined either clinically or histopathologically. Other names include rheumatoid papules, Churg-Strauss granuloma, cutaneous extravascular necrotizing granuloma, and interstitial granulomatous dermatitis with arthritis. In 1983 cutaneous extravascular necrotizing granuloma was associated with a variety of disorders, such as systemic lupus erythematosus, rheumatoid arthritis, systemic vasculitis, lymphoproliferative conditions, Wegener granulomatosis, and inflammatory bowel disease . A single case report of PNGD occurring in a patient with Behçet disease has been reported . In 1994, the term palisaded neutrophilic and granulomatous dermatitis was proposed to encompass the clinical and histopathologic spectrum of lesions in the setting of systemic immune-complex diseases . Clinical lesions characteristically appear as firm papules or nodules that measure 2 mm to 2 cm in diameter and range from skin-colored to red to violaceous. The overlying epidermis may be normal, but ulcers and crusts have been described. Other cutaneous manifestations include petechiae, livedo reticularis, and urticaria. Lesions most commonly appear on the distal aspects of the upper extremities and fingers or elbows although they have been described on the thighs, trunk, and lower back . Most eruptions are asymptomatic but can be associated with tenderness or pruritus. The condition may persist for several months to a few years. The histopathologic features can be varied and depend on the age of the lesions. Early lesions are characterized by dense neutrophilic infiltrates, degenerated collagen, nuclear dust, broad cuffs of fibrin surround the venules, and extravasated erythrocytes. Fully-developed lesions show palisaded granulomas that surround leukocytoclastic debris, fibrin, and altered collagen. Late-stage lesions are characterized by palisaded granulomas with dermal fibrosis and scant neutrophilic debris . Clinical and histopathologic differential diagnoses include leukocytoclastic vasculitis, urticarial vasculitis, erythema elevatum diutinum, Sweet syndrome, granuloma annulare, rheumatoid nodules, and necrobiosis lipoidica. The etiology of PNGD is unknown although investigations have focused on the deposition of immune complexes in dermal vessels as the initial event. IgM and C3 have been frequently demonstrated in dermal vessel walls and at the dermoepidermal junction on direct immunofluorescence study . A pathogenetic model proposes that the deposition of immune complexes in dermal vessels leads to leukocytoclastic vasculitis, degeneration of collagen fibers, and palisaded granulomatous dermatitis with dermal fibrosis in the terminal stage . The most commonly reported beneficial therapy is oral glucocorticoids, which are either initiated for the cutaneous lesions or for the underlying systemic condition . Different treatment options that have been reported in the literature include colchicine, cyclosporine, cyclophosphamide, hydroxychloroquine, nonsteroidal anti-inflammatory agents, and dapsone with various degrees of success [5, 6].
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