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Protracted superficial Wegener granulomatosis in a child

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Protracted superficial Wegener granulomatosis in a child
Imed Ben Ghorbel MD, Asma Sioud Dhrif MD, Mohamed Miled MD, Mohamed Habib Houman, MD
Dermatology Online Journal 13 (4): 14

Department of Internal Medicine, La Rabta hospital, Tunis, Tunisia.


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.

Wegener granulomatosis (WG) is a rare systemic vasculitis of unknown etiology characterized by involvement of the upper airway, lung, and kidneys; although, almost any organ can be affected [1]. Variants of WG also exist. A lengthy course has been reported in some patients.

Clinical synopsis

A 16-year old boy presented with a 3-month history of an ulceration of the fifth left finger and the first left toe. The disease started at the age of 7 years with recurrent digital necrotic ulcers. In addition, the patient had experienced many flares of purulent rhinorrhea during the last year.

Figure 1Figure 2
Figure 1. Ulceration of the plantar face of the first left toe
Figure 2. Multiple ulcerated erythematosus papules and nodules of the right scapular area

Physical examination revealed an ulceration of the plantar surface of the first left toe (Fig. 1) and multiple ulcerated erythematosus papules and nodules on the right scapular area (Fig. 2). The ear, nose, and throat examination showed congestion with superficial ulcerations of the nasal mucosa (Fig. 3) without nasal deformation or nasal septal abnormalities. Keratitis was noted during an ocular examination.

Figure 3
Figure 3. Superficial ulcerations of the nasal mucosa.

Respiratory, cardiac, and neurological examinations were unremarkable. Laboratory investigations revealed an erythrocyte sedimentation rate of 70 mm, total leucocyte count of 12500/mm3, gammaglobulinemia of 21 g/l, and C-reactive protein of 8 mg/l. Urinalysis and renal function tests were normal. C-ANCA done by indirect immunofluorescence was positive. Rheumatoid factor, antinuclear antibodies, and cryoglobulins were negative. Chest X-ray was normal. The sinus computerized tomographic scan demonstrated occluded ethmoidal sinuses. The histopathology of the biopsy specimens of the nasal mucosa showed superficial ulcerations underlying a granulomatous infiltration (Fig. 4). The histopathology of the biopsy specimens from the scapular nodule revealed granulomatous vasculitis (Fig. 5).

Figure 4Figure 5
Figure 4. Superficial ulceration of the nasal mucosa underlying a granulomatous infiltration (H&E X 10)
Figure 5. Granulomatous vasculitis on the scapular nodule (H&E X 10)

Stains with PAS and Fite were negative for fungi and acid fast bacilli. Tissue cultures for bacteria, fungi, and mycobacteria were negative.

The diagnosis of WG was made according to the American College of Rheumatology criteria [2]. The patient was treated with oral prednisone (1 mg/kg/day) for 2 months. The symptoms decreased during the following year and medication was tapered. Similar cutaneous and mucosal lesions occurred during 2 relapses of the disease, after 9 months and 11 months, respectively, without renal or respiratory involvement. A trial of cotrimoxazol for 3 months did not yield any improvement; systemic corticosteroids were again required to promote clearing.


WG is a systemic disease characterized by necrotizing and granulomatous vasculitis that involves predominantly the upper and lower respiratory tract and kidneys. The disease is not limited to those manifestations and can affect many organs including the skin [1].

Protracted superficial WG is a rare variant consisting of necrotic ulcers primarily localized in the skin and mucosa with much milder internal disease that can continue for months and even years before severe multiorgan system involvement develops [3]. Our patient had cutaneous ulcerations 8 years before nasal mucosa involvement and 9 years before the diagnosis was made. No internal organ involvement was present.

The skin involvement is common in WG and its frequency ranges from 16 to 77 percent [4]; however, the presence of cutaneous disease is rarely the initial or the only sign of WG [5]. In cases with initial skin manifestations, the definitive diagnosis is made after an average period of 12 months. The duration of the protracted superficial variant varies and can range from 9 months to 18 years [3, 6, 7, 8, 9].

The clinical presentation of cutaneous lesions is highly variable. The findings may include palpable purpura, papules, subcutaneous nodules, ulcers, pustules, and vesicles. Fewer cases have exhibited a macular eruption, livedo, splinter hemorrhages of fingernails, and pyoderma-gangrenusum-like ulcers [3, 4, 8, 9]. The extremities, especially legs and feet, are the most common sites. However, in the protracted variant the face is often affected [6, 8, 9].

Upper airway symptoms are frequent in WG and consist of nasal, sinus, tracheal, and ear inflammatory abnormalities. Nasal involvement occurs with rhinorrhea or nasal obstruction and can lead to many complications such as mucosal ulcerations, nasal septal perforation or nasal deformity. WG in children and adolescents results in a high frequency of nasal deformity and subglottic stenosis.

Accurate pathologic interpretation of tissue biopsies is an essential aspect of the diagnosis of WG. Because of the accessibility of the cutaneous and mucosal lesions for biopsies, their pathologic findings must be recognized. In a majority of cases, the histopathology is nonspecific and in 25 percent of cases, skin lesions demonstrate characteristic findings including leukocytoclastic vasculitis, palisading granulomas, and granulomatous vasculitis. Necrotizing vasculitis is usually associated with purpuric lesions and less frequently with subcutaneous nodules. Granulomatous inflammation is usually associated with nodules and cutaneous and mucosal ulcerations [10]. In our patient, the histopathological findings established the diagnosis. In these protracted and superficial cases, the diagnosis of WG is aided by the demonstration of antineutrophilic antibodies.

If left untreated, the disease may produce severe tissue damage and eventual impairment of renal function along with other sequelae. Cytotoxic therapy is often required but does not necessarily prevent the development of multisystem involvement. In fact, in patients with limited or protracted superficial forms of WG there appears to be no way to predict which individuals will experience progression; these patients need careful follow up. Low-dose methotrexate combined with prednisone has been shown to induce remission in 71 percent of patients [12].


Because the triad of necrotizing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and small vessel vasculitis is not seen initially in the protracted superficial variant of WG, the correct diagnosis may be overlooked. However, this rare form must been recognized and treated; immunosuppressive therapy can prevent multiorgan system involvement and renal failure. Long-term surveillance is essential.


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