Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum

Abstract

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15-year-old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis. | CASE REPORTA 15-year-old boy presented with a pustular, warty eruption of 4 months duration. Cutaneous lesions involving the scalp, trunk, limbs, and genitals were characterized by numerous pustules surrounded by an erythematous halo and vegetative and keratotic plaques with crusts and purulent discharge overlying ulcerated, slightly painful plaques ( Figure 1A,B). Ocular examination showed erythema, conjunctival injection, and scleral nodules (Figure 2A). Several 2-to 3-mm pustules were seen on the hard palate. He also had marked digital clubbing and cyanosis ( Figure 2B), despite pulmonary auscultation with no remarkable findings, absence of dyspnea, and pulse oximetry of 92%.The patient's disease had started 9 months before with cough and he was treated for chronic bronchitis, with partial improvement.Five months before the onset of the dermatosis, he was empirically treated for pulmonary tuberculosis, but the cough persisted.During examination, a pathergy test was negative, and chest Xrays revealed a reticulonodular pattern and probable bronchiectases.Computed tomography (CT) showed pleural fibrosis, the vascular pattern was more apparent, and noncavitated nodules. Paranasal sinus CT was normal. Lung function tests showed a mild restrictive pattern. Bronchoscopy revealed mild tracheoendobronchitis with a diffuse nodular pattern. Tests were negative for microorganisms. Pulmonary hypertension was excluded. Complete blood cell count showed leukocytosis (12 9 10 3 /mm) with neutrophilia (77%). Erythrocyte sedimentation rate (22 mm/h) and C-reactive protein

Granulomatosis and Polyangiitis Presenting as Superficial Granulomatous Pyoderma

“…Clinical appearance of SGP is usually a sterile, well-defined superficial ulcer with a clean base and no undermining borders most commonly occurring on the trunk and rarely on the face [1,3,[6][7][8]. In contrast to classic PG, SGP typically presents as slowly progressing, more superficial granulomatous lesions and as such has a more favorable prognosis and is not associated with underlying systemic or autoimmune disease [1,3,[6][7][8][9][10][11]. SGP usually resolves with local, topical or intralesional anti-inflammatory or anti-microbial agents and does not require more aggressive systemic immunosuppressive agents that may be required in the treatment PG lesions or GPA, although a handful of cases of more aggressive SGP variants on the face, head, and neck have been reported [9][10][11].…”

Neutrophilic Dermatoses and Their Implication in Pathophysiology of Asthma and Other Respiratory Comorbidities: A Narrative Review

“…Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis most commonly presenting with a disfiguring deep necrotic laceration with a dusky, indeterminate edge, typically in the lower limb. In addition to the classic ulcerative presentation, four other variants are identified: pustular, bulbous, peristomal, and vegetative [38–41]. Since neither the clinical nor histological manifestations are pathognomonic, PG is usually diagnosed by a combination of features only after exclusion of other conditions [16].…”