eMedicine Specialties > Dermatology > Diseases of the Adnexa

Lupus Miliaris Disseminatus Faciei

Author: Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
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Updated: Jan 29, 2009

Introduction

Background

Lupus miliaris disseminatus faciei (LMDF) is an uncommon, chronic, inflammatory dermatosis characterized by red-to-yellow or yellow-brown papules of the central face, particularly on and around the eyelids. Lesions may occur singly or in crops. Once considered a tuberculid because of the histology, many authors now consider LMDF to be an extreme variant of granulomatous rosacea. Others believe it is a distinct entity because of its characteristic histopathology and occasional involvement of noncentral facial areas.¹

A variety of treatments are reportedly of some benefit, but controlled studies to establish the best treatment are lacking. Most clinicians find LMDF difficult to control; LMDF may result in disfiguring scarring. Etiology and pathogenesis are unknown. Active disease usually involves a 1- to 3-year course and resolves spontaneously.

In 2000, Skowron et al proposed a name change from LMDF to FIGURE (facial idiopathic granulomas with regressive evolution); to date, this name change does not appear to have been widely accepted.²

Pathophysiology

Lupus miliaris disseminatus faciei (LMDF) affects only the skin. Studies have failed to demonstrate Mycobacterium tuberculosis or other mycobacterial disease by culture or polymerase chain reaction.³ Extrapolating from theories of the pathogenesis of other forms of rosacea, some authors suggest that LMDF is a reaction to Demodex folliculorum. While the usual distribution coincides with that of most rosacea cases, an association with Demodex has not been confirmed. Others suggest that LMDF is a granulomatous reaction to hair follicle destruction or ruptured epidermal cysts.

Frequency

United States

Lupus miliaris disseminatus faciei (LMDF) frequency is unknown in the United States, but the disease is considered rare.

International

Lupus miliaris disseminatus faciei (LMDF) frequency is unknown internationally, but the disease may be more prevalent in Japan.

Mortality/Morbidity

Lupus miliaris disseminatus faciei (LMDF) is not associated with mortality. Morbidity includes mild-to-severe scarring with resolution of the disease.

Race

Lupus miliaris disseminatus faciei (LMDF) may be more common in Asians, especially Japanese people.

Sex

Authors have stated that both sexes can be affected with lupus miliaris disseminatus faciei (LMDF); however, most published reports reviewed for this discussion and cited in the Bibliography describe solely or predominantly male patients.

Age

Young adults in their 20s most often are affected, although one report by Shitara described a woman in her early 70s.⁴ Young adolescents also may be affected, and some authorities believe that chronic granulomatous periorificial disease of children (CGPD) is a form of lupus miliaris disseminatus faciei (LMDF) because histology and treatment response are the same.⁵

Clinical

History

Most often, young adults with lupus miliaris disseminatus faciei (LMDF) have papules singly or in crops that are red, brown, or yellow-brown and appear on the central face, especially on and around the eyelids. Spontaneous resolution after crusting or pustulation within 1-3 years is standard. Residual scarring after individual papules regress may be disfiguring. Lesions occasionally may be generalized and appear on the extremities or trunk.

Physical

Lupus miliaris disseminatus faciei (LMDF) manifests red, brown, or yellow-brown papules that appear singly or in crops. The papules appear on the central face, especially on and around the eyelids of young adults. They are found predominantly on the face in areas traditionally affected by rosacea.

Lesions occasionally may be generalized and appear on the extremities or trunk. Axillary lesions may be mistaken for antiperspirant-related granulomas. Lesions may present later as crusts, pustules, and, ultimately, scars.

Causes

Cause is unknown, but suggestions have included infection by M tuberculosis, atypical mycobacteria, tuberculids, foreign body granuloma (particularly to zirconium), reaction to cyst contents, and reaction to D folliculorum.

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