Clinicopathological and Immunological Studies of Lupus Miliaris Disseminatus Faciei

Abstract

Eighty‐one cases of lupus miliaris disseminatus faciei were clinically and histologically studied, and some of them were also immunologically studied. Age at onset, distribution of skin lesions, and incidence by sex did not differ from the reports of various investigators. No apparent increase in incidence of this disease was noted. The histologic features of this disease could be divided into four types. Some relation between pilosebaceous units and epithelioid cell granulomas could be found in the lesions of 35 (43%) out of 81 cases. Central caseation may be coagulation necrosis caused by abscesses. Some immunological data were obtained from this study, but there were no abnormalities in delayed hypersensitivity reactions, e.g. tuberculin test, DNCB skin reaction, PHA skin reaction and the Kveim test, or in quantitative serum immunoglobulin surveys. Lymphocyte transformation tests using two mitogens, PHA‐P and Con‐A, showed normal responses. It was not possible to obtain enough data from the clinical data and histologic findings for this disease to be regarded as a tuberculid. But from the histologic findings, lupus miliaris disseminatus faciei was assumed to be a chronic inflammatory disease of pilosebaceous units and epithelioid cell granulomas in the lesions might be reactions to destroyed hair follicles or epithelial cysts. With these assumptions, the following hypotheses concerning the pathogenesis of this disease were proposed. 1) Epithelial cysts occur and rupture in the dermis for some reason resulting in formation of foreign‐body granulomas which break down epithelial cysts in the dermis. 2) Inflammatory infiltrates occur within the follicular epithelium or sebaceous glands for some reason and they are destroyed resulting in formation of epithelioid cell granulomas to follicular contents, such as keratin, sebum and Demodex folliculorum. Under the above assumptions, I would like to support the theory that lupus miliaris disseminatus faciei is not a tuberculid but a skin disorder similar to the papular type of acne rosacea.

The granulomatous reaction pattern

“…274 Biopsy appearances overlap those of both rosacea and perioral dermatitis. 275 Nuclear fragments may be seen in the necrotic foci. 7.5).…”

A Review of Lupus Miliaris Disseminatus Faciei-Like Histopathologic Changes in 10 Cases

“…It appears to be a granulomatous reaction most often related to destruction of the pilosebaceous unit and the resultant antigen exposure, but it remains clinically distinct from granulomatous rosacea and periorificial dermatitis, and in fact the etiology may be multifactorial. To prevent diagnostic confusion, we advocate limiting the histologic criteria to include only fully developed lesions demonstrating epithelioid granulomas with caseous necrosis [17]. The presence of solitary lesions in the majority of our patients and exclusively extrafacial involvement in 2 patients demonstrates the importance of histopathology in aiding diagnosis of LMDF.…”

“…The presence of nuclear dust and neutrophils within the zones of caseation necrosis suggests that neutrophils may indeed play a role in inducing this change [9]. Shitara found granulomas next to hair follicles in only 43 percent of 81 patients with LMDF, despite having performed multiple levels through the paraffin blocks [17]. Nevertheless, an immune response to the pilosebaceous unit seems highly likely to be involved in the granuloma formation in many cases of LMDF [9].…”

“…LMDF has been considered to be a form of granulomatous rosacea, supported by the frequent close association with pilosebaceous units [1,17]. However, LMDF is often clinically distinct from rosacea, since LMDF is self-limited with spontaneous resolution over 1-4 years (usually between 12-24 months), often leaves disfiguring scars, does not present with erythema, flushing, or telangiectasia, is not exacerbated by heat or spicy foods, and is often improved with oral corticosteroid treatment [1,3,5].…”

The treatment of acne agminata with clofazimine

“…1 Acne agminata occurs predominantly in young adults but is also reported in adolescents. 2,3 Histopathologically, the papules show dermal granulomas composed of epithelioid cells surrounding areas of caseation necrosis. There is no evidence for a tuberculous aetiology although, historically, the disease was named`lupus milaris disseminatus faciei'.…”