Lupus Miliaris Disseminatus Faciei

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 images below.

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Lupus miliaris disseminatus faciei central facial papules. Courtesy of San Antonio Uniformed Services Health Education Consortium.

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Lupus miliaris disseminatus faciei.

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).^[2] While the term LMDF is still widely used, the term FIGURE is now appearing in some publications.

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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.

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Etiology

The etiology of lupus miliaris disseminatus faciei (LMDF) 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. Onset has been reported after stem cell transplantation.^[4]

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Epidemiology

Frequency

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

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.^[5] 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.^[6]

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Prognosis

Prognosis for spontaneous resolution within 2 years is good. Therapy may help speed resolution. Recurrences are not described.

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Patient Education

Educate patients about the nature of the disease to help alleviate anxiety and to establish realistic treatment expectations.

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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.

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Physical Examination

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.^[7] Axillary lesions may be mistaken for antiperspirant-related granulomas. Lesions may present later as crusts, pustules, and, ultimately, scars.

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Imaging Studies

Imaging is not indicated for lupus miliaris disseminatus faciei (LMDF) unless sarcoidosis is suspected.

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Other Tests

Tests are not indicated for lupus miliaris disseminatus faciei (LMDF) unless sarcoidosis is considered likely.

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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.

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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.^[8] 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. Note the image below.

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Histopathology of lupus miliaris disseminatus faciei showing nodule with caseation necrosis. Image courtesy of Dr. Dirk Elston.

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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 prednisone^[9]
• Intralesional triamcinolone^[10]
• Intramuscular triamcinolone^[11]
• Dapsone (systemically and topically)^[12]
• Tetracycline products
• Antimalarials
• Pyridoxine hydrochloride
• Riboflavin
• Isotretinoin^[13]
• Tranilast^[14]

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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.

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Consultations

No consultations are indicated.

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Diet

No dietary association (excess or deficiency) with lupus miliaris disseminatus faciei (LMDF) is described.

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Activity

No physical activities or exposures are described that either improve or worsen lupus miliaris disseminatus faciei (LMDF).

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Complications

Scarring (occasionally severe) is the primary complication of lupus miliaris disseminatus faciei (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.

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Prevention

Since the pathogenesis of lupus miliaris disseminatus faciei (LMDF) is unknown, prevention methods are difficult to define.

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Medication Summary

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). In the setting of LMDF, isotretinoin has been used alone or with systemic corticosteroids. Corticosteroids should be added only as a last resort.^[15] Since LMDF spontaneously resolves within 1-2 years, the impact of therapy on the course of the disease is difficult to assess.

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Prednisone (Deltasone, Meticorten, Orasone)

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Clinical Context:  Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

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Class Summary

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.

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Tetracycline (Sumycin)

• Dosing, Interactions, etc.

Clinical Context:  Tetracycline's anti-inflammatory mechanism of action may differ from its antibacterial mechanism of action. Some studies indicate that tetracyclines inhibit inflammatory cell migration and transformation of lymphocytes. Tetracycline treats gram-positive and gram-negative organisms and mycoplasmal, chlamydial, and rickettsial infections. It inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).

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Doxycycline (Vibra-Tabs, Vibramycin, Bio-Tab)

• Dosing, Interactions, etc.

Clinical Context:  Doxycycline's anti-inflammatory effect may result from the inhibition of migration of inflammatory cells and transformation of lymphocytes. It inhibits protein synthesis and thus bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria.

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Minocycline (Dynacin, Minocin)

• Dosing, Interactions, etc.

Clinical Context:  Minocycline's anti-inflammatory effects may result from the inhibition of inflammatory cell migration and transformation of lymphocytes.

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Class Summary

Tetracyclines are used for their anti-inflammatory rather than antibiotic effects. Most reports describe limited therapeutic benefit. This class includes tetracycline, doxycycline, and minocycline.

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Isotretinoin (Accutane)

• Dosing, Interactions, etc.

Clinical Context:  Isotretinoin is an 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. Isotretinoin should be prescribed only by physicians experienced and/or trained in its use.

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Class Summary

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.

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