eMedicine Specialties > Dermatology > Diseases of the Adnexa
Lupus Miliaris Disseminatus Faciei
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.
Differential Diagnoses
Acne Vulgaris
Hydroa Vacciniforme
Rosacea
Sarcoidosis
Syringoma
Trichoepithelioma
Other Problems to Be Considered
Lupus vulgaris (see Cutaneous Tuberculosis)
Workup
Imaging Studies
Imaging is not indicated for lupus miliaris disseminatus faciei (LMDF) unless sarcoidosis is suspected.
Other Tests
Tests are not indicated for lupus miliaris disseminatus faciei (LMDF) unless sarcoidosis is considered likely.
Procedures
Skin biopsy may be necessary if the diagnosis of lupus miliaris disseminatus faciei (LMDF) is in doubt. Biopsy may help distinguish LMDF from the more common granulomatous rosacea, sarcoidosis, or benign adnexal neoplasms such as syringomas. Potential complications of biopsy are scarring, infection, or insufficient biopsy material for diagnosis.
Histologic Findings
Early lupus miliaris disseminatus faciei (LMDF) lesions show superficial perivascular and periappendiceal lymphocytic infiltrates with a few histiocytes and neutrophils. Fully developed lesions show round granulomas, often with caseation necrosis.6 The changes mimic miliary tuberculosis. Mixtures of sarcoidal and tuberculoid granulomas also may be seen. Late lesions show fibrosis with scattered lymphocytes, histiocytes, and neutrophils and also may be perifollicular and may show epidermal thinning.
Treatment
Medical Care
A variety of medical treatments reportedly are effective in lupus miliaris disseminatus faciei (LMDF), although controlled studies that support one treatment or group treatments as optimal are lacking. Reported therapies include the following:
- Low-dose prednisone7
- Intramuscular triamcinolone
- Dapsone
- Tetracycline products
- Antimalarials
- Pyridoxine hydrochloride
- Riboflavin
- Isotretinoin8
Surgical Care
- Scar revision procedures (laser resurfacing, dermabrasion, chemical peel) may benefit patients after the disease has run its course.
- Treatment with the 1450-nm diode laser has been reported to be effective.
- Pulse-dye laser has been used to successfully treat the erythema of rosacea, but its use in this condition has not been described.
Consultations
No consultations are indicated.
Diet
No dietary association (excess or deficiency) with LMDF is described.
Activity
No physical activities or exposures are described that either improve or worsen LMDF.
Medication
Medical treatment of lupus miliaris disseminatus faciei (LMDF) often is unsuccessful. Anecdotal reports describe improvement with a variety of therapies, including prednisone, isotretinoin, tetracyclines, and vitamins (eg, riboflavin, pyridoxine). Since LMDF spontaneously resolves within 1-2 years, the impact of therapy on the course of the disease is difficult to assess.
Corticosteroids
Both topical and systemic corticosteroids have been used for their anti-inflammatory properties. In the literature, topical agents usually are described as ineffective; low-dose systemic agents reportedly work rapidly and well. Since LMDF may represent a form of rosacea, corticosteroids may provide temporary improvement, followed by chronic flaring of the disease. Caution is advised, and corticosteroids should not be administered unless other treatment options have failed.
Prednisone (Deltasone, Meticorten, Orasone)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Dosing
Adult
Standard dose: 1 mg/kg/d; taper after LMDF resolves
Low dose: 10 mg qd for 2 wk, followed by 5 mg qd for 3 mo
Pediatric
Not established
Interactions
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Contraindications
Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections, GI disease
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur
Tetracyclines
Used for their anti-inflammatory rather than antibiotic effects. Most reports describe limited therapeutic benefit.
Class includes tetracycline, doxycycline, and minocycline.
Tetracycline (Sumycin)
Anti-inflammatory mechanism of action may differ from antibacterial mechanism of action. Some studies indicate that tetracyclines inhibit inflammatory cell migration and transformation of lymphocytes. Treats gram-positive and gram-negative organisms and mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).
Dosing
Adult
250-500 mg PO bid/qid
Pediatric
<8 years: Not recommended
>8 years: 25-50 mg/kg/d (10-20 mg/lb) PO qid; 1 h ac or 2 h pc
Interactions
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can increase hypoprothrombinemic effects of anticoagulants; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy
Contraindications
Documented hypersensitivity; severe hepatic dysfunction
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment, especially with doxycycline; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; use during tooth development (last one half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines
Doxycycline (Vibra-Tabs, Vibramycin, Bio-Tab)
Anti-inflammatory effect may result from inhibition of migration of inflammatory cells and transformation of lymphocytes. Inhibits protein synthesis and thus bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria.
Dosing
Adult
50-100 mg PO bid
Pediatric
<8 years old: Not recommended
>8 years old: Administer as in adults
Interactions
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; tetracyclines can increase hypoprothrombinemic effects of anticoagulants; tetracyclines can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy
Contraindications
Documented hypersensitivity; severe hepatic dysfunction
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last one-half of pregnancy through age 8) can cause permanent discoloration of teeth; Fanconi-like syndrome may occur with outdated tetracyclines
Minocycline (Dynacin, Minocin)
Anti-inflammatory effects may result from inhibition of inflammatory cell migration and transformation of lymphocytes.
Dosing
Adult
50-100 mg PO bid
Pediatric
<8 years: Not recommended
>8 years: 50 mg PO qd/tid
Interactions
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants
Contraindications
Documented hypersensitivity; severe hepatic dysfunction
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last one-half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines; hepatitis or lupus-like syndromes may occur
Retinoids
Histology at various stages of the disorder suggests a follicular-based disorder, although the pathogenesis is unclear, and the predilection for eyelids is difficult to explain. How retinoids help is difficult to explain except in general terms relating to proper maturation and function of the follicular epithelium. Use of topical retinoids is not described in the literature, but presumably, they have been tried without benefit. Systemic retinoids cause severe birth defects. Adhere to current prescribing guidelines.
Isotretinoin (Accutane)
Oral agent that treats serious dermatologic conditions. Isotretinoin is the synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to vitamin A.
Should be prescribed only by physicians experienced and/or trained in its use.
Dosing
Adult
0.5-1 mg/kg/d PO
Pediatric
Not established
Interactions
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine
Contraindications
Documented hypersensitivity; pregnancy or intended pregnancy within 3 mo
Precautions
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Causes skin and mucosal dryness; patient may not be able to wear contact lenses while undergoing therapy; depression and other behavior changes are associated with use; monitor lipids (particularly triglycerides) and LFTs; advise patients to avoid pregnancy while undergoing therapy and for a minimum of 3 mo after discontinuation; nay decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; diabetes patients may experience problems in controlling their blood sugar while on isotretinoin; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur
Follow-up
Deterrence/Prevention
Since the pathogenesis of lupus miliaris disseminatus faciei (LMDF) is unknown, prevention methods are difficult to define.
Complications
Scarring (occasionally severe) is the primary complication of LMDF. Efforts to control the disease and minimize scarring are laudable; however, take care that patients do not unduly experience the complications of therapy for this self-limiting disease.
Prognosis
Prognosis for spontaneous resolution within 2 years is good. Therapy may help speed resolution. Recurrences are not described.
Patient Education
Educate patients about the nature of the disease to help alleviate anxiety and to establish realistic treatment expectations.
Miscellaneous
Medicolegal Pitfalls
- Failure to properly diagnose cutaneous tuberculosis or sarcoidosis
- Failure to inform patients of or guard against the complications of therapy
Multimedia
Media file 1: Histopathology of lupus miliaris disseminatus faciei showing nodule with caseation necrosis. Image courtesy of Dr. Dirk Elston.
Media file 2: Lupus miliaris disseminatus faciei central facial papules. Courtesy of San Antonio Uniformed Services Health Education Consortium.
References
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].
Berbis P, Privat Y. Lupus miliaris disseminatus faciei: efficacy of isotretinoin. J Am Acad Dermatol. Jun 1987;16(6):1271-2. [Medline].
Mihara K, Isoda M. Immunohistochemical study of lysozyme in lupus miliaris disseminatus faciei. Br J Dermatol. Aug 1986;115(2):187-92. [Medline].
Keywords
lupus miliaris disseminatus faciei, LMDF, acne agminata, acnitis
Contributor Information and Disclosures
Author
Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.
Medical Editor
James Fulton Jr, MD, PhD, Medical Director, Fulton Skin Institute
James Fulton Jr, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Cosmetic Surgery, American Academy of Dermatology, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.
Pharmacy Editor
David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other
Managing Editor
Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.
CME Editor
Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.
Chief Editor
Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
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