- Top of page
- Case Report
A 19-year-old girl presented with hemorrhagic acneiform lesions on the face for several months that was unresponsive to conventional acne treatment. A biopsy revealed a noninfectious suppurative granulomatous dermatitis with hemorrhage, possibly representing a ruptured folliculitis. A second biopsy revealed chronic granulomatous dermal inflammation and hemorrhage with foreign body giant cells non-infectious by stains. No vasculitis was noted in either biopsy. Later in her course she developed a severe sinusitis and eventually presented with severe fevers, rapid weight loss, sinusitis, and cough. Further workup produced the diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis). She rapidly improved with intravenous steroids and rituximab. To date, acneiform lesions have only been reported in young adult patients and may represent a clinical manifestation of granulomatosis with polyangiitis unique to this age group, as illustrated in our patient.
- Top of page
- Case Report
A 19-year-old girl presented complaining of “blood under her skin” in hemorrhagic acneiform papules distributed across her chin (Fig. 1). Without manipulation, the painless, nonpruritic papules would spontaneously bleed under the surface of her skin. She denied any history of fevers or bleeding disorders, and complete blood count, prothrombin time, and partial thromboplastin time were within normal limits. Conventional treatment with a topical combination of a retinoid and benzoyl peroxide, topical clindamycin, and oral doxycycline was unsuccessful.
Test treatments using a pulsed dye laser were partially successful in improving the lesions, but she continued to develop more hemorrhagic acneiform papules, including a few on her arms.
Approximately 6 months after her initial presentation, she developed a severe sinus infection with malaise. Two months later she presented to the hospital with fevers, weight loss of 10 pounds over 1 to 2 months, severe sinus congestion, and cough. Chest radiograph showed a pulmonary nodule with cavitation in the right lower lobe.
A small biopsy specimen from the chin at this time revealed a suppurative, granulomatous dermatitis with hemorrhage. No organisms were detected on special stains. A second biopsy of the cheek revealed chronic granulomatous dermal inflammation and hemorrhage, with some fragments of foreign nonpolarizable material (Fig. 2). Acid-fast and periodic acid-Schiff stains failed to show any organisms, and CD34 demonstrated only small vessels within the papillary dermis. CD31 immunostain was noncontributory and no vasculitis was noted in either biopsy.
Serology showed a positive cytoplasmic antineutrophil cytoplasmic antibody (cANCA) and high proteinase 3 titer, confirming clinical suspicion of Wegener's granulomatosis (WG). Pulsed high-dose intravenous steroids rapidly improved her condition. Rituximab and prednisone were initiated and she has since been controlled with azathioprine.
Renamed granulomatosis with polyangiitis (GPA) , WG is an antineutrophil cytoplasmic antibody (ANCA)-associated primary vasculitis characterized by necrotizing granulomatous inflammation of small and medium-size blood vessels and glomerulonephritis .
The spectrum of lesions from 75 cases reported by Frances et al  consisted of purpura, nodules, skin ulcers, oral ulcers, gingival hyperplasia, pustules, and necrotic papules. The histopathologic findings were varied; necrotizing or granulomatous vasculitis could be present, and granulomatous inflammation was associated with nonpurpuric lesions. Another study showed that the cutaneous features were not limited to only leukocytoclastic vasculitis; other findings included acneiform perifollicular and dermal granulomatous inflammation and palisaded neutrophilic granulomatous inflammation (with or without vasculitis) .
Our patient is not only a rare example of pediatric-onset GPA, but also a rare case in which GPA presented initially with cutaneous findings. Although nodular and papular skin lesions are common in GPA, they are rarely the initial symptom and are typically found on the limbs. A review of the literature shows that acneiform lesions have been reported only in patients younger than 20 years of age, and we believe that acneiform skin findings may be an initial presentation unique to pediatric-onset GPA [5-9].
GPA is rare in children. Since the clinical and dermatopathologic findings are broad and nonspecific, and untreated prognosis is poor, it represents a condition in which the dermatologist can prevent devastating sequelae, particularly in adolescents, in whom coexisting acne may confound the clinical picture. We propose that acneiform lesions may be a novel presentation in pediatric-onset GPA.