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Acne agminata in Crohn's disease: A diagnostic and therapeutic challenge case for dermatologists

Dear Editor, We report the case of a unusual macuolo-papular eruption of the face in a young male patient, concomitant with asymptomatic relapse of Crohn's disease, that represented an interesting diagnostic and therapeutic challenge for the dermatologists. A 32 years old man affected by Crohn's disease from 10 years, in clinical remission for the last 2 years, presented to our clinical observation with asymptomatic yellowish dome-shaped micro-papular eruption of middle face, including eyelids, evolved over a period of about 6 months. As the patient had suffered from acne in his youth, vulgaris acne was suspected first, and patient received a combined therapy based on oral administration of systemic antibiotic minocycline 100 mg/die and topical application of adapalene 0.1% plus benzoyl peroxide 5% once a day for 8 weeks, with unsatisfactory therapeutic outcome. The patient was switched to oral isotretinoin 0.5 mg/kg/die for 16 weeks, with poor clinical response at the end of treatment period, thus suspecting a skin involvement from systemic granulomatous disease, we proceeded with skin biopsy and several instrumental and laboratory analyses. All data from laboratory—HBV, HCV, HIV serology, serum calcium, ACE, and lysozyme—were within the range of normality, except for fecal calprotectin whose levels were >800 mg/kg; mantoux test was negative and chest RX did not show any pleural parenchymal lesion. An asymptomatic relapse of Crohn's disease was suspected and then confirmed by CT enterography, which showed mucosal hyper-enhancement, wall thickening, mural stratification with a prominent vasa recta (comb sign), and mesenteric fat stranding. Histology revealed a granulomatous wide inflammation with giants multinucleated and epithelioid cells, lymphocytic infiltrate with central caseous necrosis around piliferous follicles (Figure 1 histological features of skin biopsy). Although histology could suggest a mycobacterial infection, as destructive vascular features were absent, Ziehl-neelsen staining and cultures for mycoses and mycobacteria were negative, the diagnosis of acne agminata was favored. Acne agminata (AA), also known as lupus miliaris disseminatus faciei, papular tuberculid, “Barthelemy acnitis” is a dermatosis whose nosological identity has been long debated, as the dermatosis has been confused with other granulomatous skin dermatoses. Getting a diagnosis was our first achievement: AA has been considered a clinical variant of rosacea granulomatous for several years, however the absence of telangiectatic component and a different histological profile drove us to distinguish AA from rosacea. Moreover, the presence of a central caseous necrosis allowed us to differentiate AA from the non-caseous granuloma of sarcoidosis. Both clinical and histopathological features could be indicative of cutaneous miliary tuberculosis, from which AA was differentiated since mantoux test and PCR analysis for the BK research on the tissue sample were negative. In addition, considering the patient's clinical history we hypothesized that the exacerbation of Crohn's disease in conjunction with the onset of acneiform eruption could not be a mere coincidence.