Author(s): Kathryn Echols, Fiona Fang and James W. Patterson
Objective: To determine the clinical and histopathologic features of all lesions diagnosed as lupus miliaris disseminatus faciei via biopsy over the past 16 years at a single institution. Clinical features reviewed included age of patient, location and number of lesions, duration, description of primary lesion, size, and suspected clinical diagnosis or differential diagnosis. Histopathologic features reviewed included presence of caseation necrosis, depth of granuloma, presence of lymphocytic infiltrate, disruption of hair follicles, and presence of multinucleated giant cells.
Methods: The records of 10 patients (mean age, 50.4 years; range, 6 to 79 years) with characteristic histologic features of lupus miliaris disseminatus faciei were reviewed and the histopathologic findings and clinical features were analyzed. Formalin-fixed, paraffin-embedded specimens were examined by hematoxylin-eosin staining.
Results: The most common clinical appearance was a single papule located on the face. Two cases with solitary, extrafacial distributions were reported. All cases demonstrated epithelioid granulomas with a central area of caseation necrosis. The majority of granulomas were perifollicular in location and were comprised of histiocytes, lymphocytes, and multinucleated giant cells.
Conclusion: The 10 cases we report demonstrate the importance of recognizing the entity in solitary as well as extrafacial forms. Limiting the histologic diagnosis to fully developed lesions demonstrating epithelioid granulomas with caseation necrosis serves to clarify the diagnosis in the setting of diverse clinical presentations. Further information is needed to clarify the diagnosis, etiology, and pathogenesis of this disease, but an unusual host response to folliculitis or follicular injury likely plays a role in most cases.