Localized Wegener's granulomatosis

Background Wegener’s granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease. Objectives Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG. Methods The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied. Results All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti‐neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide. Limitations The limited number of patients is counterbalanced by the rarity of the disease. Conclusions Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.     

Protracted superficial Wegener granulomatosis in a child

Wegener granulomatosis is a rare, chronic, multisystemic vasculitis affecting mainly the upper and lower respiratory tracts together with glomerulonephritis, but the disease may involve any other organ. Protracted superficial form is a rare variant of the disease. We report a case of protracted superficial Wegener granulomatosis in a 16-year-old boy in whom the disease started with recurrent digital ulcers at age 7 years. Later, he developed nodules and papules associated with upper airway involvement and ocular keratitis without lung or renal involvement. C-ANCA was positive. The patient was treated with oral prednisone. Similar cutaneous and mucosal lesions developed during two relapses of the disease without renal or respiratory involvement.     

Superficial granulomatous pyoderma: an idiopathic granulomatous cutaneous ulceration

A 70-year-old male with a superficial granulomatous ulcer is reported. Histopathological findings were the same as those described for superficial granulomatous pyoderma, a recognized variant of classic pyoderma gangrenosum. The differences between pyoderma gangrenosum and its variant superficial granulomatous pyoderma are highlighted.     

Palisading neutrophilic granulomatous dermatitis in a Japanese patient with Wegener's granulomatosis

Wegener's granulomatosis (WG) is an etiologically obscure entity with multiple systemic manifestations. Recently, cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) has become recognized as a valuable adjunct in the diagnosis of this disorder. WG typically involves the upper airway, lungs, and kidneys, but any other organ can be involved, including the skin. We encountered a unique case in which a 27-year-old Japanese man with WG presented with various typical cutaneous manifestations. Purpuric skin lesions and erythematous rash on the lower extremities progressively involved and changed into a necrotizing ulceration on his toe. Additionally, several nodules developed on the extensor surfaces of his elbows. His serum C-ANCA level increased remarkably. Leukocytoclastic vasculitis, the most common histopathological finding in WG patients, was detected in a purpuric lesion on his hand. A biopsy of a nodule on his elbow revealed palisading epithelioid histiocyte granulomas with features of leukocytoclastic vasculitis. The distinctive pattern of papules has been referred to as "palisading neutrophilic granulomatous dermatitis". An open lung biopsy confirmed WG with focal necrotizing granuloma. A renal biopsy demonstrated necrotizing vasculitis and crescentic glomerulonephritis. He showed a good response to oral corticosteroids and cyclophospamide with total remission of symptoms. We believe that a careful balance between the clinical manifestations and the histopathological evidence allows for timely treatment of WG, which may prevent serious morbidity or death. Although uncommon, WG can present with various types of accompanying cutaneous lesions. Therefore, clinicians should keep this diagnosis in mind when presented with these manifestations.     

Wegener's granulomatosis

A case of an unusual development of Wegener's granulomatosis is described. A long-term immunosuppressive therapy led to intermittent involution of the process, but finally a transformation to a new disease, for present qualified as "malignant histiocytosis" was the result. The cause of the transformation and the differentiation from other necrotising granulomas are discussed.     

Wegener's granulomatosis

This report deals with a female patient with Wegener's granulomatosis, exhibiting characteristic asymptomatic lung disease and palpable purpura but also a granulomatous vasculitis of the intestine which led to multiple perforations. Therapy with cyclophosphamide and corticosteroids brought remission of all lesions.     

Superficial granulomatous pyoderma: a case report of two Japanese patients and clinical comparison with foreign patients

Superficial granulomatous pyoderma (SGP) is an unusual variant type of pyoderma gangrenosum characterized by a benign course and specific histological features; it is quite a rare cutaneous disorder in Japan. We reported two Japanese cases of SGP and compared the clinical features of 13 Japanese cases with those of 42 foreign cases. Case 1 was a 53-year-old female who presented with three indolent and ulcerative plaques with elevated edges on the back and the posterior portion of the left thigh. Case 2 was a 74-year-old female who presented with crusted and vegetative erythematous plaques on the left shoulder and left upper arm. Histological examinations revealed a characteristic three-layer granuloma in each case. Laboratory and physical examinations found no abnormalities. Topical corticosteroid, oral administration of minocycline, and/or additional supplementation with corticosteroid were effective. In comparison with foreign cases, Japanese cases show a possible differentiation in the ratio of sex and distribution of the ulcer. Females have a high incidence and the extremities are frequently involved area in Japanese cases. Up to now, 11 cases of SGP have been reported in Japan. To our best knowledge, our patients are the first Japanese cases described in the English literature.     

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Lymphomatoid granulomatosis (LG) is a rare Epstein–Barr virus (EBV)‐associated B‐cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one‐third of patients and may precede the development of lung lesions in as many as 10–15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B‐lymphocytes in a polymorphous T‐cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.     

ANCA-negative limited Wegener's granulomatosis

A 26-year-old man presented with epistaxis, nasal obstruction and a subcutaneous swelling over the left malar region with radiological evidence of a mass in the right nasal cavity. Histology of the lesions showed necrotizing granuloma with evidence of vasculitis. There was no other systemic involvement and the patient was ANCA-negative. Excellent response to systemic steroid and cyclophosphamide therapy was noted.     

A case of Wegener's granulomatosis

The described case of Wegener's granulomatosis evidences that this condition is difficult to diagnose and that it should be differentiated from tuberculosis, syphilis, pemphigus, deep mycosis. Late identification and beginning of therapy result in generalization of the process.     

Clinical variants of Wegener's granulomatosis

A female patient is described, suffering from a lingering superficial form of Wegener's granulomatosis. The disease has been diagnosed on the basis of its prolonged (7 yrs) relatively benign course, isolated involvements presenting as skin and nasal mucosa ulcers, hard palate and nasal septum defect, and histologic shifts (necrotizing paling granulomas, combined with vasculitis). Low prednisolone doses have been quite effective in therapy of this condition.     

Chronic superficial pyoderma of the face: an unusual male case of rosacea fulminans

A 62-year-old Japanese male developed acneiform papules on the face in November of 2002. The eruptions progressed to exudative erythema with central necrosis, and then to thick-crusted superficial abscesses in a short period. The shallow ulcers exposed by removal of the crusts and abscesses immediately re-epithelized without leaving scars. The histology of the eruption was a dense infiltration of neutrophils associated with granulomatous changes in the upper to middle dermis. Histology of the tiny white particles in the abscess showed an irregularly proliferated mass of keratinocytes including accumulated neutrophils. The skin lesions resisted intravenous injection of antibiotics but responded to systemic administration of a corticosteroid agent. Colchicine did not work well, but the additional administration of etretinate was effective. The patient is currently receiving combined therapy with prednisolone and etretinate, but eruptions are still episodically observed. We diagnosed this case as an unusual male case of rosacea fulminans.     

Wegener's granulomatosis presenting as pyoderma gangrenosum

Half of the patients with Wegener's granulomatosis develop skin lesions due to the systemic vasculitis. Wegener's granulomatosis should be included in the differential diagnostic considerations for necrotic ulcers, including leg ulcers. We present a case which demonstrates the importance of histological evaluation of a skin biopsy from the margin of the ulcer for establishing the diagnosis. Antineutrophil cytoplasmic antibodies with antigen specificity for proteinase 3 (PR3‐ANCA) were detected supporting the diagnosis of Wegener granulomatosis. Further evaluation showed involvement of the eyes and kidneys. The ulcer rapidly healed under treatment with cyclophosphamide and corticosteroids.     

Cutaneous involvement in Wegener's granulomatosis.

A case of Wegener's granulomatosis is presented. The major features of the disease, with special emphasis on the cutaneous findings, are reviewed.