AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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.

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.

Pathophysiology

LMDF affects only the skin. Studies have failed to demonstrate Mycobacterium tuberculosis or other mycobacterial disease by culture or polymerase chain reaction. 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

Frequency is unknown in the United States, but the disease is considered rare.

International

Frequency is unknown internationally, but the disease may be more prevalent in Japan.

Mortality/Morbidity

• LMDF is not associated with mortality.
• Morbidity includes mild-to-severe scarring with resolution of the disease.

Race

LMDF may be more common in Asians, especially Japanese people.

Sex

Authors have stated that both sexes are affected; 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. Young adolescents also may be affected, and some authorities believe that facial granulomatous disease of children is a form of LMDF.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

Most often, young adults 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

• Red, brown, or yellow-brown papules appear singly or in crops.
• Papules appear on the central face, especially on and around the eyelids of young adults.
• Papules are found predominantly on the face in areas traditionally affected by rosacea.
• Lesions occasionally may be generalized and appear on the extremities or trunk.
• 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.

DIFFERENTIALS

Section 4 of 11  

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Other Problems to be Considered

Lupus vulgaris (see Cutaneous Tuberculosis)

WORKUP

Section 5 of 11  

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• Medication
• Follow-up
• Miscellaneous
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Imaging Studies

• Imaging is not indicated unless sarcoidosis is suspected.

Other Tests

• Tests are not indicated unless sarcoidosis is considered likely.

Procedures

• Skin biopsy may be necessary if the diagnosis 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 lesions show superficial perivascular and periappendiceal lymphocytic infiltrates with a few histiocytes and neutrophils. Fully developed lesions show round granulomas, often with caseation necrosis. 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

Section 6 of 11  

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• Follow-up
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• Multimedia
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Medical Care

A variety of medical treatments reportedly are effective in LMDF, although controlled studies that support one treatment or group treatments as optimal are lacking. Reported therapies include the following:

• Low-dose prednisone
• Dapsone
• Tetracycline products
• Antimalarials
• Pyridoxine hydrochloride
• Riboflavin
• Isotretinoin

Surgical Care

• Scar revision procedures (laser resurfacing, dermabrasion, chemical peel) may benefit patients after the disease has run its course.
• 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

Section 7 of 11  

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• Miscellaneous
• Multimedia
• References

Medical treatment 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.

Drug Category: 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.

Drug Category: Tetracyclines

Used for their anti-inflammatory rather than antibiotic effects. Most reports describe limited therapeutic benefit.

Class includes tetracycline, doxycycline, and minocycline.

Drug Name	Doxycycline (Vibra-Tabs, Vibramycin, Bio-Tab)
Description	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.
Adult Dose	50-100 mg PO bid
Pediatric Dose	<8 years old: Not recommended >8 years old: Administer as in adults
Contraindications	Documented hypersensitivity; severe hepatic dysfunction
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
Pregnancy	D - Unsafe in pregnancy
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

Drug Name	Minocycline (Dynacin, Minocin)
Description	Anti-inflammatory effects may result from inhibition of inflammatory cell migration and transformation of lymphocytes.
Adult Dose	50-100 mg PO bid
Pediatric Dose	<8 years: Not recommended >8 years: 50 mg PO qd/tid
Contraindications	Documented hypersensitivity; severe hepatic dysfunction
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
Pregnancy	D - Unsafe in pregnancy
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

Drug Category: 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.

FOLLOW-UP

Section 8 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Deterrence/Prevention:

• Since the pathogenesis of 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

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Failure to properly diagnose cutaneous tuberculosis or sarcoidosis
• Failure to inform patients of or guard against the complications of therapy

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Histopathology of lupus miliaris disseminatus faciei showing nodule with caseation necrosis. Image courtesy of Dr. Dirk Elston.
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Media file 2:  Lupus miliaris disseminatus faciei central facial papules. Courtesy of San Antonio Uniformed Services Health Education Consortium.
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
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• Follow-up
• Miscellaneous
• Multimedia
• References

• Berbis P, Privat Y. Lupus miliaris disseminatus faciei: efficacy of isotretinoin. J Am Acad Dermatol. Jun 1987;16(6):1271-2. [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].
• Mihara K, Isoda M. Immunohistochemical study of lysozyme in lupus miliaris disseminatus faciei. Br J Dermatol. Aug 1986;115(2):187-92. [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].
• Shitara A. Lupus miliaris disseminatus faciei. Int J Dermatol. Oct 1984;23(8):542-4. [Medline].
• Skowron F, Causeret AS, Pabion C, et al. 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].
• 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].
• 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].
• 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].

Article Last Updated: Feb 7, 2007