Acne fulminans is a rare disease, which most often affects teenagers and young adults, being described associated with exogenous testosterone use and with elevated adrenal androgens in congenital adrenal hyperplasia. We describe a case of acne fulminans in a toddler with adrenocortical carcinoma.
A 2.3 years-old male diagnosed with adrenocortical tumor presenting pubarche, increased penis and body hair, severe acne lesions in face, chest and back and also arterial hypertension. He was previously healthy, with normal development at his first year. The investigation showed adrenal hyperandrogenism and hypercortisolism. Serum values of Total Testosterone >1500 ng/dL (<7,00 - 25,91 ng/dL), ACTH: 7,2 pg/mL (<46 pg/mL), DHEAS: 682 µg/dL (<15 µg/dL), Aldosterone: 11 ng / dL (2.5-39.2 ng /dL), Serum Cortisol 8AM: 30.65µg/dL (5.27 to 22.45 µg/dL), Salivary midnight cortisol: 29.5nmol/L (11:30PM-00:30AM < 7.6 nmol/L). The abdominal computed tomography (CT) showed the left suprarenal gland with a 5,1 x 3,9 cm lesion. Left adrenalectomy was performed, whose histology confirmed adrenocortical carcinoma, without exceeding the capsule, but with a vascular invasion focus and Ki-67 rate of 20%. Hormonal levels in the early follow up were normal and prophylactic corticosteroid therapy was progressively removed. At the three months after surgery outpatient follow-up, worsening of his skin acneiform lesions was observed. He presented painful papules, pustules and crusts at face, chest and back, with purulent exudation and bleeding lesions, associated with axillary and inguinal adenomegaly and acne fulminans was diagnosed. Clinical and ultrasonographic examination also showed the presence of hepatosplenomegaly,. The treatment was initially made with oral corticosteroids and antibiotics. At this time, laboratory tests of androgens, DHEAS and salivary cortisol were normal, but inflammatory markers were high. A new postoperative abdominal CT showed, at the left adrenal topography, two lesions, 47HU attenuation, 1.5 x 1.2 x 1.1cm and 2.3 x 1.7 x 2.0cm, and also confirmed mild hepatosplenomegaly. Biopsy of inguinal lymph nodes was performed, and histology showed lymphoid follicles hyperplasia.
This case report showing the association between acne fulminans and adrenocortical carcinoma, at postoperative time, when the child already had normal serum androgens and after hypercortisolism resolution, without oral corticosteroid therapy. Disordered immune response and/or hypersensitivity reaction to Propionibacterium acnes antigens, are also considered as possible triggering factors, although the etiology for this cases is not well established. The description of an acne fulminans case in such a young patient with adrenocortical carcinoma seems unusual and such association deserves further elucidation.