Lupus Miliaris Disseminatus Faciei
- Author: Jeffrey Meffert, MD; Chief Editor: Dirk M Elston, MD more...
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] Note the image below.
Lupus miliaris disseminatus faciei central facial papules. Courtesy of San Antonio Uniformed Services Health Education Consortium. 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.
Epidemiology
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]
van de Scheur MR, van der Waal RI, Starink TM. Lupus miliaris disseminatus faciei: a distinctive rosacea-like syndrome and not a granulomatous form of rosacea. Dermatology. 2003;206(2):120-3. [Medline].
Skowron F, Causeret AS, Pabion C, Viallard AM, Balme B, Thomas L. F.I.GU.R.E.: facial idiopathic granulomas with regressive evolution. is 'lupus miliaris disseminatus faciei' still an acceptable diagnosis in the third millennium?. Dermatology. 2000;201(4):287-9. [Medline].
Hodak E, Trattner A, Feuerman H, et al. Lupus miliaris disseminatus faciei--the DNA of Mycobacterium tuberculosis is not detectable in active lesions by polymerase chain reaction. Br J Dermatol. Oct 1997;137(4):614-9. [Medline].
Shitara A. Lupus miliaris disseminatus faciei. Int J Dermatol. Oct 1984;23(8):542-4. [Medline].
Misago N, Nakafusa J, Narisawa Y. Childhood granulomatous periorificial dermatitis: lupus miliaris disseminatus faciei in children?. J Eur Acad Dermatol Venereol. Jul 2005;19(4):470-3. [Medline].
el Darouti M, Zaher H. Lupus miliaris disseminatus faciei--pathologic study of early, fully developed, and late lesions. Int J Dermatol. Jul 1993;32(7):508-11. [Medline].
Uesugi Y, Aiba S, Usuba M, Tagami H. Oral prednisone in the treatment of acne agminata. Br J Dermatol. Jun 1996;134(6):1098-100. [Medline].
Tokunaga H, Okuyama R, Tagami H, Aiba S. Intramuscular triamcinolone acetonide for lupus miliaris disseminatus faciei. Acta Derm Venereol. 2007;87(5):451-2. [Medline].
Berbis P, Privat Y. Lupus miliaris disseminatus faciei: efficacy of isotretinoin. J Am Acad Dermatol. Jun 1987;16(6):1271-2. [Medline].
Koike Y, Hatamochi A, Koyano S, Namikawa H, Hamasaki Y, Yamazaki S. Lupus miliaris disseminatus faciei successfully treated with tranilast: Report of two cases. J Dermatol. Jun 2011;38(6):588-92. [Medline].
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