Acne fulminans in a young man with granulomatosis with polyangiitis (Wegener's granulomatosis): A chance association or marker of serious systemic disease?

A 19-year-old man with granulomatosis with polyangiitis (Wegener's disease) presented with hemorrhagic facial nodules mimicking severe inflammatory acne (acne fulminans) as one of the first symptoms of the disease. The lesions were earlier treated as nodulocystic acne with isotretinoin without any benefit. Complete resolution was seen with pulsed methylprednisolone and oral prednisolone and mycophenolate mofetil thereafter. He also developed acute onset of severe pustular eruption of the face and a destructive ulcer of the auricle on two separate occasions. Facial lesions mimicking severe inflammatory acne, not responsive to standard treatment, may be a marker for more severe systemic disease such as Wegener's disease/granulomatosis with polyangiitis.

Nasal septal ulceration.

Nasal septal ulceration can have multiple etiologies. Determining the exact cause depends on who the consulting specialist is, who could either be the ENT surgeon or the dermatologist. The common causes are infections (tuberculosis, leprosy, leishmaniasis), vasculitis (Wegener's granulomatosis and Churg-Strauss syndrome), and lupus erythematosus. Traumatic causes and malignancy can also be seen in tertiary referral centers. The diagnosis often requires thorough investigations and multiple tissue specimens from various sites, and in chronic cases, a suspicion of lymphoma should be considered. Apart from disease-specific therapy, a multidisciplinary approach is required in most cases to tackle the cosmetic disfigurement.

Background noise of infection for using ANCA as a diagnostic tool for vasculitis in tropical and developing countries.

Antineutrophil cytoplasmic antibody (ANCA) is often used in the laboratory to confirm paucicellular vasculitis like Wegener's granulomatosis, Churg Strauss syndrome or polyarteritis nodosa in the presence of suggestive clinical features. In tropical countries, tuberculosis, leprosy and, occasionally, malaria can produce clinical features similar to a vasculitic illness and all the three infections are known to be associated with auto antibodies. We tested 318 patients suffering from malaria, tuberculosis or leprosy for ANCA positivity. ANCA positivity was found in 19%, 32% and 30% of malaria, tuberculosis and leprosy patients (Pradhan V, Badakere S, Shankarkumar V, Iyer Y, Ghosh K, Karnad D, Indian J Malariol, 39:51-59, 2002; Pradhan V, Badakere S, Ghosh K, Pawar A, Indian J Med Sci, 58:283-288, 2004a; Pradhan V, Badakere S, Shankarkumar V, Lepr Rev, 75:50-56, 2004b), respectively, raising the possibility that ANCA positivity with clinical features suggestive of vasculitis in tropical countries may even be related to the background noise of this seropositivity caused by one of these three infections rather than confirming the diagnosis of paucicellular vasculitis. Hence, one should be careful about the background noise of ANCA positivity caused by these infections while diagnosing a vasculitic illness.

Nasal manifestations of granulomatous disease.

Granulomatous disease frequently affects the head and neck region, particularly the nose and sinuses. This article describes the most common infectious and non-infectious conditions and their clinical features.

Doenças granulomatosas da laringe / Granulomatous diseases of the larynx

Realizamos uma revisäo sobre as principais doenças granulomatosas que podem acometer a laringe, devido à grande ocorrência dessas afecçöes no Brasil e em outros países de clima tropical e subtropical. Ressaltamos os aspectos histopatológicos para o seu diagnóstico, assim como o seu tratamento.

Anti-neutrophil cytoplasmic antibodies (ANCA) in the clinical forms of leprosy.

Anti-neutrophil cytoplasmic antibodies (ANCA) are autoantibodies against enzymes present in primary granules of neutrophils and lysosomes of monocytes detected in systemic vasculitis and in other diseases, including infections. ANCA are markers of active Wegener granulomatosis, which presents some anatomo-pathologic and immune response features similar to those of leprosy. Thus, we raised the hypothesis that ANCA may be present in leprosy as markers specifically linked to the presence of vasculitis. The aim of this study was to determine the presence of ANCA in leprosy and its correlation with the clinical forms of the disease. Sera from 60 normal individuals and from 59 patients with different clinical forms of leprosy were studied. The patients were also allocated into reactional and nonreactional groups. By indirect immunofluorescence, ANCA were positive, an atypical pattern (A-ANCA), in 28.8% of the patient sera. A-ANCA predominated, although not significantly (p > 0.05), in the reactional groups 37.9% vs 20.0%), and in those at the lepromatous pole (41.6% vs 20.0%). There was no correlation between ANCA positivity and either disease duration, disease activity, or therapeutic regimen (p > 0.05). An interesting finding was the correlation between ANCA and gender: 94.1% of ANCA-positive patients were males (p < 0.01), a feature that so far has not been reported in ANCA-related diseases and for which there is no explanation at the moment. By ELISA, the sera of the lepromatous leprosy patients did not show activity against either PR3, MPO, HLE, the most common ANCA antigens. Because A-ANCA are nonspecific, this finding requires further investigation for the determination of the responsible antigen(s). In conclusion, A-ANCA are present in 28.8% of leprosy patients but are not related to vasculitis in the erythema nodosum leprosum reaction and are not a marker of a specific clinical form.

Granulomatous diseases of the larynx

The inflammatory processes that involve the larynx can take many forms, with the granulomatous disorders invariably representing the end consequences of chronic disease. In the past, laryngeal granulomas were more prevalent, primarily because of the frequency of tuberculosis.More recently such lesions often have been diagnosed by clinicians attempting to rule out carcinoma.

Granulomatous lesions in nasal biopsies.

A series of 19 cases has been reviewed in which biopsy of an intra-nasal lesion revealed a granulomatous pathology. These have been classified on an aetiological basis. They include infections, Wegener's granuloma and neoplasms with a granulomatous stroma. One patient with sarcoidosis first presented with lesions in the nasal cavity. Cholesterol granulomata were seen in four lesions removed from the paranasal sinuses. In six cases clinical and histological examination failed to show a cause for the granulomata; in all of these patients the nasal cavity was free from disease at a subsequent examination.

Medical and surgical aspects of the granulomatous diseases of the larynx

Granulomatous diseases of the larynx are often clinically unususpected and may present a wide spectrum of symptoms and physical findings. A thorough otolaryngologic examination is imperative because granulomatous infiltration of the entire upper respiratory tract may precede or coexist with laryngeal involvement.

The cutaneous manifestations in wegeners granulomatosis / ver

Wegeners granulomatosis is a rare puzzling disease generally characterized by necrotizing granulomatous lesions in the lungs and upper respiratory tract generalized necrotising vasculitis and acute glomerulitis Pathologically tehre is focal necrotizing vasculitis involving the small arteries and veins of the respiratory tract as well as those of the kidney spleen heart intestines liver mucous membranes and skin. In this rport we wish to stress that the cutaneous manifestations are often a prominent feature of wegeners granulomatosis and occasionally are the first sign of the desease. The basis for this report is II8 patients with unequivocal wegener granulomatosis screened from the word literature. The authors cheched al the reported cases of wegeners granulomatosis as well as the conditions frequently confused with it such as periarteritis nodosa lethal medline granuloma the stevens johson syndrome and several others. With but few exceptions patientes were included in this series only if an apparent diagnosis of wegeners granulomatosis was confirmed by gross autopsy examination or by microscopy biopsy examination using the widely accepted pathologic criteria of Godman and Churg (2). If there were any doubt about the diagnosis in any patient in any series or if the pathologic evidence were only presumptive that patient wasexcluded from this study. Our object was to review as many patients as possiblewith clear cut wegeners granulomatosis in order to study the extent of the cutaneos involvement in this disease. In addition to illustrate some of the problems inherent in distinguishing wegener granulomatosis from other conditions characterized by necrotizing angiitis with granuloma we have included five cases form our personal experience. All of these had mucosal lesions and four of the five had atypical pneumonias. Autopsy examination of the four who have died indicated that there had authentic wegeners granulomatosis while the evidence in the other two patients suggests their condition possibily could be due to this entity yet there are many atypical features that cast doubt on the diagnosis.