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A Preauricular Ulcer in a Patient With Conjunctival Injection and Persistent Lacrimation: Answer.

DISCUSSION Granulomatosis with polyangiitis (GPA, previously Wegener granulomatosis) is a systemic antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis with frequent mucocutaneous involvement. Patients with GPA present with constitutional symptoms (fevers, malaise, arthralgias, fatigue, and weight loss), signs and symptoms of upper respiratory tract inflammation (ulcers, rhinorrhea, persistent sinusitis and/or otitis media), ocular involvement (conjunctivitis, episcleritis, uveitis), mononeuritis, and lower respiratory tract involvement (cough, hemoptysis, pleurisy). A minority of patients with GPA presents with glomerulonephritis, but the majority of patients eventually develop this disease manifestation. Upwards of 90% of patients with GPA have ANCA, the majority of which are anti-proteinase 3 (PR3). Patients with limited GPA have clinical manifestations that are limited to the upper respiratory tract or lungs. These patients are more likely to be young, female, and develop saddle nose deformities and less likely to be ANCA-positive. Mucocutaneous manifestations are very common in patients with GPA. Thirteen percent to 23% of patients with GPA will present with cutaneous signs of disease, and upwards of 50% will develop cutaneous manifestations at some point in their disease course. The most common cutaneous sign of GPA is palpable purpura on the lower extremities. Signs of medium vessel inflammation such as subcutaneous nodules, lower extremity ulcers, and digital infarcts are also common. Flesh colored papules on the extensor surfaces (previously referred to as rheumatoid papules or Churg–Strauss granulomas and now referred to palisaded and neutrophilic granulomatous dermatosis) may also be seen. Cutaneous manifestations in GPA are associated with a higher frequency of articular and renal involvement and correlate with active systemic vasculitis. Pyoderma gangrenosum-like ulcers are a rare cutaneous manifestation of GPA. Pyoderma gangrenosum-like ulcers in GPA often occur in atypical sites such as the perianal area and the head and neck (malignant pyoderma) and have nonspecific histological features such as epitheliomatous hyperplasia, granulation tissue, foreign body giant cells, fibrosis, and mixed diffuse inflammatory infiltrate. The clinical differential of pyoderma granulosum-like ulcers includes malignancies, infections (bacterial, deep fungal, typical mycobacterial, leishmaniasis), and pyoderma gangrenosum. Unfortunately, skin biopsy is rarely sufficient to make the diagnosis of GPA. The most common histological finding in GPA is leukocytoclastic vasculitis, which is reported to occur in 31%–80% of submitted biopsies. In contrast, granulomatous vasculitis, the sine qua non of GPA, is very rarely seen with reported frequencies of 0%– 11% of submitted GPA skin biopsies. Therefore, the absence of granulomatous vasculitis on skin biopsy does not rule out GPA. Other commonly reported histological diagnoses in GPA include chronic inflammation, granulomatous inflammation, nonspecific ulceration, and erythema nodosum. In conclusion, cutaneous manifestations in GPA are very common. However, pyoderma gangrenosum-like ulceration is an uncommon cutaneous presentation of GPA. Patients who present with pyoderma gangrenosum-like ulcers on the head and neck and perineum should be evaluated for GPA as their underlying etiology. Biopsies of the pyoderma gangrenosum-like lesions in GPA reveal nonspecific histopathological findings. Cutaneous manifestations in GPA are correlated with active systemic vasculitis and should prompt evaluation for disease activity.