Cutaneous manifestation of Kikuchi's histiocytic necrotizing lymphadenitis

A young man who presented with cervical Kikuchi's histiocytic necrotizing lymphadenitis later developed a cutaneous plaque lesion. Histologic study of the cutaneous lesion revealed dermal patchy infiltrates composed of large lymphoreticular cells and scattered cells resembling Hodgkin or Reed-Sternberg cells. This condition was initially mistaken for a large-cell lymphoma. But it was later discovered that the dermal cellular infiltrates were similar to that of the involved lymph node. Many of the large cells in the infiltrates were found to be histiocytes. Immunohistochemical study revealed that the cells resembling Hodgkin or Reed-Sternberg cells were activated fibroblasts. The presence of foamy histiocytes and the absence of neutrophils in the skin lesions were also similar to the involved lymph node. Cutaneous Kikuchi's disease may be mistaken for cutaneous lymphoma. Cutaneous involvement by Kikuchi's disease may also denote a worse clinical course.     

The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases.

Kikuchi's disease (KD) is an idiopathic, self-limited necrotizing lymphadenitis that can clinically and histologically mimic high-grade lymphoma, including Hodgkin's disease, or can be mistaken for the lymphadenitis of systemic lupus erythematosus (SLE). Involvement of extranodal sites is unusual but well documented, especially in Asia, where KD is more common than in North America or Europe. The successful distinction of KD from malignant lymphoma and SLE is imperative for the appropriate treatment of affected patients. We describe five patients with cutaneous involvement by KD, all of whom presented with fever, lymphadenopathy, and an eruption on the skin of the upper body, which in one case was clinically suspected to be due to SLE and in another, polymorphous light eruption. The patients ranged in age from 10 months to 42 years (median, 33 years) and included three females and two males. All five patients had negative serologic studies for collagen vascular disease. Each patient had a lymph node biopsy showing the typical necrotizing lymphadenitis of KD. Skin biopsies from all five patients shared a specific constellation of histologic features: vacuolar interface change with necrotic keratinocytes, a dense lymphohistiocytic superficial and deep perivascular and interstitial infiltrate, varying amounts of papillary dermal edema, and abundant karyorrhectic debris with a conspicuous absence of neutrophils and a paucity of plasma cells, paralleling the nodal histology in KD. CD68 immunohistochemistry on paraffin-embedded sections showed many histiocytes and plasmacytoid monocytes in all cases, whereas CD3, CD4, and CD8 showed highly variable staining among the cases. There was only rare staining with TIA-1 and CD30. We believe that the papular eruption of KD has recognizable histopathologic features and that a CD68 stain that marks many cells that initially seem to be lymphocytes can be performed to confirm the diagnosis.     

Necrotizing histiocytic lymphadenitis (Kikuchi-Fujimoto disease) in a 7-year-old girl

Kikuchi-Fujimoto's disease is a rare self-limited condition of young adults that usually involves the cervical lymph nodes and is associated with fever, rash and some haematological alterations. Diagnosis is based on characteristic pathologic findings that permit differentiation of this disease from lymphoma, systemic lupus erythematous and infectious lymphadenopathies. We describe a case of 7 year-old female presenting with cervical localization of Kikuchi-Fujimoto's disease. To our knowledge, this case results the earliest affection of this disease. Our proposal with this article is to remind the pediatricians and pediatric surgeons of this poorly recognized entity when children ask for cervical masses and fever.     

Kikuchi's disease: a review and analysis of 61 cases.

OBJECTIVE: Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a rare self-limiting disorder that typically affects the cervical lymph nodes. It has occasionally been misdiagnosed as malignant lymphoma or another serious diseases; hence, clinicians should be made more aware of this disease. METHODS: From January 1986 to January 2001, a series of 61 patients who underwent a biopsy of a cervical lymph node and proved histologically to have KD were enrolled in this study. We retrospectively reviewed the clinical records and pathologic parameters of each patient. The patients were followed up from 6 months to 14.3 years (mean, 6.9 years). RESULTS: There were 34 women and 27 men (1.26:1 ratio; age range, 6 to 46 years; mean age, 21 years). The affected cervical lymph nodes were commonly located in the posterior cervical triangle (54 of 61, or 88.5%). Unilateral and bilateral cervical lymph nodes were affected in 54 and 7 patients, respectively. The dimensions of affected lymph nodes were commonly in the range of 0.5 to 4 cm (57 of 61, or 93.4%). In 2 patients, the size of the enlarged lymph nodes was greater than 6 cm. Leukopenia was observed in 14 patients (23%); fever, in 18 patients (29.5%). Five patients had a past history of tuberculosis. Two patients developed systemic lupus erythematosus, 1 month and 5 years later, respectively. The cervical lymphadenopathy usually resolved without any medical treatment within 6 months after definite diagnosis was made. In 3 patients, the enlarged lymph nodes eventually disappeared after more than 1 year. No recurrence has since been noted. CONCLUSION: The results of this study support the theory that KD is a self-limiting disorder that does not require any specific management. The female predominance was not as striking as in the studies performed in Western countries. We suggest that the patients with KD require a systemic survey and regular follow-up for several years; 2 of our patients developed systemic lupus erythematosus. An effective communication between the otolaryngologist and pathologist is needed because the clinical and pathologic characteristics of KD are essential in making an accurate diagnosis.     

A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis

Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.     

Management of perineal necrotizing fasciitis (Fournier's gangrene)

Eleven cases of perineal necrotizing fasciitis were treated in the Department of Surgery, University of Turku, Finland. In 10 cases the diagnosis was made on the basis of the fulminating progression of the infection to scrotal gangrene, identification of multiple underlying pathogenic organisms and toxaemia. In one female patient a corresponding infection developed in the labia majora. Each patient suffered nonspecific symptoms before the gangrene became evident. The management included surgical debridement of the necrotic tissue with incisions and drainage of the involved areas, antibiotic therapy and hyperbaric oxygen (HBO) treatment. The infection originated from the anorectal area in 5 patients, 1 patient had sustained scrotal trauma and in 5 cases the underlying condition was unknown. Colostomy was performed in 6 patients. One patient died 2 days after the admission. All the infections proved to be multimicrobial. Hyperbaric oxygenation was employed as a therapeutic adjunct in the present series, but it should neither replace nor delay surgical intervention. The key points in the management include early diagnosis with prompt surgical debridement and antibiotic therapy. After healthy granulation has appeared, the healing time can be shortened with reconstructive surgical procedures.     

Histiocytic lymphoma (reticulum-cell sarcoma) of bone. Current strategy for orthopaedic surgeons

We retrospectively reviewed the cases of eight patients with histiocytic lymphoma (reticulum-cell sarcoma) appearing as a primary bone lesion. Histological findings were classified according to the criteria of Rappaport. Lesions were staged as suggested by the Ann Arbor Conference. After thorough staging, four of the eight patients were shown to have more extensive disease than was initially thought, which altered the therapeutic approach used. Thorough staging is necessary for improved survival of patients with histiocytic lymphoma of bone. We suggest a diagnostic strategy for patients with histiocytic lymphoma presenting in bone that leads to appropriate therapy.     

A forgotten old disease: mediastinal tuberculous lymphadenitis in children.

OBJECTIVE: The purpose of the study was two-fold: (1) to highlight the varied presentation of mediastinal tuberculous lymphadenitis (MTL) in children and (2) to identify parameters, that may help in the early diagnosis of this condition. METHODS: Between January 1995 and December 2002, 13 children with histological diagnosis of MTL were retrospectively assessed for age at presentation, history of exposure to TB, presenting symptoms, investigations, initial diagnosis, surgical treatment and outcome. Stepwise multiple linear regression analysis was used to determine potential risk factors for early diagnosis of MTL. RESULTS: Thirteen children presented with: (a) fever, night sweats and weight loss (4); (b) acute respiratory distress (2); (c) cough and shortness of breath (SOB) (5); (d) stridor (1); and (e) chest pain (1). TB was suspected only in 6 children (46%) at presentation. In the other 7 cases (54%) the presumed diagnoses were: neuroblastoma (n=1), metastatic malignancy (n=1), bronchial polyp (n=1), bronchogenic cyst (n=2), and presumed foreign body (n=2). Bronchoscopy was diagnostic in identifying cheesy material within the bronchus and organisms on lavage in 4 (30%) and in identifying external compression in 2 (15%). Thoracotomy and excision of the lymph node mass was necessary to treat the mediastinal compression and to ascertain the diagnosis of TB in 3 children (23%). All 13 children had complete resolution of tuberculous lymphadenitis following anti-tuberculous treatment. The diagnostic clues in this cohort of patients were cough and SOB with history of exposure to tuberculosis (P=0.0001) and bronchoscopy and lavage with positive staining for acid-fast bacilli (P=0.0001). CONCLUSIONS: Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms.