You are in: eMedicine Specialties > Dermatology > DISEASES OF THE ADNEXA Lupus Miliaris Disseminatus FacieiArticle Last Updated: Feb 7, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio Jeffrey Meffert is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society Editors: James Fulton Jr, MD, PhD, Medical Director, Fulton Skin Institute; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: acne agminata, acnitis INTRODUCTIONSection 2 of 11
  BackgroundLupus 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. PathophysiologyLMDF 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. FrequencyUnited StatesFrequency is unknown in the United States, but the disease is considered rare. InternationalFrequency is unknown internationally, but the disease may be more prevalent in Japan. Mortality/Morbidity
RaceLMDF may be more common in Asians, especially Japanese people. SexAuthors 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. AgeYoung 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. CLINICALSection 3 of 11
HistoryMost 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
CausesCause 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. DIFFERENTIALSSection 4 of 11
Acne Vulgaris Hydroa Vacciniforme Rosacea Sarcoidosis Syringoma Trichoepithelioma
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| Drug Name | Prednisone (Deltasone, Meticorten, Orasone) |
|---|---|
| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
| Adult Dose | 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 Dose | Not established |
| Contraindications | Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections, GI disease |
| 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 |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| 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 |
Used for their anti-inflammatory rather than antibiotic effects. Most reports describe limited therapeutic benefit.
Class includes tetracycline, doxycycline, and minocycline.
| Drug Name | Tetracycline (Sumycin) |
|---|---|
| Description | 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). |
| Adult Dose | 250-500 mg PO bid/qid |
| Pediatric Dose | <8 years: Not recommended >8 years: 25-50 mg/kg/d (10-20 mg/lb) PO qid; 1 h ac or 2 h pc |
| Contraindications | Documented hypersensitivity; severe hepatic dysfunction |
| 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 |
| Pregnancy | D - Unsafe in pregnancy |
| 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 |
| 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 |
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.
| Drug Name | Isotretinoin (Accutane) |
|---|---|
| Description | 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. |
| Adult Dose | 0.5-1 mg/kg/d PO |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; pregnancy or intended pregnancy within 3 mo |
| Interactions | Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine |
| Pregnancy | X - Contraindicated in pregnancy |
| 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 |
Deterrence/Prevention:
Complications:
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Patient Education:
| 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. | |
 | View Full Size Image | Media type: Photo |
Lupus Miliaris Disseminatus Faciei excerpt
Article Last Updated: Feb 7, 2007
