Lupus Miliaris Disseminatus Faciei: A Venture into the Uncommon-Four Cases of Acne Mimicking Facial Dermatopathy – A Case Series

INTRODUCTION

Lupus miliaris disseminatus faciei (LMDF) is a rare idiopathic granulomatous skin disease, primarily affecting the eyelids and facial skin in young adults. It presents as multiple firm papules and is often misdiagnosed as acne.^[1] Early diagnosis and treatment are crucial to prevent progression and scarring.^[2,3] Here, we report four cases that presented to our dermatology outpatient department.

CASE SERIES

DISCUSSION

LMDF is a rare, chronic granulomatous skin condition affecting the face.^[4,5] It presents as discrete erythematous papules or nodules, primarily on the cheeks, periorbital areas, forehead and sometimes extending to the chin or neck.^[2,3] Despite its name, LMDF is not related to lupus vulgaris or tuberculosis.^[1,4] The lesions are typically non-suppurative but may be tender. Unlike acne or rosacea, LMDF follows a persistent course with episodic flare-ups and often leads to pitted scarring and post-inflammatory pigmentation.^[5,6] It predominantly affects young adults, with monomorphic, clustered lesions that can result in misdiagnosis.^[7]

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Figure 1:

(a-d) shows clinical images of the four patients at presentation. (e-h) shows clinical images of the patient after treatment with Capsule Isotretinoin.

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A comparison of two major studies on LMDF – one by Wang et al.^[8] in 2024 and another by Amiruddin et al.^[4] in 2011 – shows a mean age of 38.3 ± 12.1 years (range: 8–70) and 34.5 ± 14.9 years (range: 8–68), respectively. In contrast, our study found a mean age of 27 ± 6.6 years (range: 20–36). More females were affected in the previous studies (59.63% and 60%), whereas only 25% of our cases were female. Eyelid involvement was observed in 100% of cases in the first study, with 107 out of 109 showing bilateral involvement, and in 91% of cases in the second study. In our study, two out of four patients had bilateral eyelid involvement. The disease duration in the prior studies ranged from 1 to 120 months (average: 14.8 ± 30.9 months) and 18 months, whereas in our cases, it was significantly shorter, at 2.9 ± 1.6 months. Although generally asymptomatic, mild itching was reported in 29.55% of cases in past studies and in three out of four cases in our study. Healing often resulted in atrophic scars, affecting 88.63% of patients, which was also observed in two out of four of our cases.

The exact cause of LMDF remains unclear but is believed to involve a dysregulated T-cell-mediated immune response to foreign materials such as sebum and keratin within pilosebaceous units, leading to granulomatous inflammation.^[3,5] Some cases have shown a response to antituberculosis treatment, suggesting a possible Mycobacterium tuberculosis hypersensitivity reaction, though this is not universally observed.^[4,8]

Histopathological findings vary depending on the stage of the disease. Early lesions exhibit a lymphohistiocytic infiltrate around vessels and adnexal structures. Classic lesions often present with epithelioid cell granulomas, with or without central caseation, which was seen in all four of our cases.^[4,8,9] Abscess formation was noted in one of our cases. Late-stage lesions typically show perifollicular fibrosis and nonspecific inflammatory infiltrate.^[9]

LMDF can be distinguished from other conditions such as acne vulgaris (which presents with comedones), rosacea (characterised by erythema, telangiectasia and flushing), lupus vulgaris (which involves caseating granulomas and ulceration) and papular sarcoidosis (which presents as yellow-brown or violaceous plaques).^[5,9] The hallmark histopathological feature of LMDF is the presence of dermal epithelioid granulomas with caseous necrosis.^[8,9]

Dermoscopy findings consistently reveal an orange-yellow structureless background with follicular plugging, a feature considered specific to LMDF. In addition, white streaks, scar-like areas, arborising vessels and hairpin vessels are frequently observed.^[10]

The treatment of LMDF aims to reduce inflammation and prevent scarring. Various therapies, including topical and oral corticosteroids, antituberculosis treatment, oral retinoids, apremilast, tetracyclines, immunosuppressants such as methotrexate and cyclosporine, biologics and combination therapies, have been used with variable success.^[3,5,6,8,9] Intralesional and intramuscular corticosteroids have also been attempted.^[11] Laser therapies, including carbon dioxide laser, neodymium-doped yttrium aluminium garnet and pulsed dye laser, have been explored for scar treatment.^[12]

In the second study, different treatment responses were reported: 45.45% of patients responded to minocycline, 66.67% to doxycycline, 28.57% to rifampicin, 80% to roxithromycin and 66.67% to tranilast. In our study, all four patients were treated with isotretinoin.

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Figure 2:

(a) Epidermis appears normal. Dermis shows large multiple epithelioid granulomas (black arrows), few with central caseous necrosis (red stars) (×10) and (b) dermal granuloma showing caseation (red star) and giant cells (green arrows) along with inflammatory infiltrates (orange arrow) (×40).

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Figure 3:

Dermoscopy showing (a) reddish, orangish to yellowish structureless background (yellow arrow) (10x), (b) follicular plugging (orange circle) (10x), (c) arborising vessels (blue arrow) (10x), (d) white streaks/scar-like areas (brown arrow) associated with hairpin vessels (purple arrow) (10x).

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CONCLUSION

LMDF is a chronic granulomatous disorder with challenging treatment. We present four cases successfully treated with low-dose isotretinoin, showing clinical improvement within 4 months. Oral isotretinoin is a good option for treatment of LMDF with fast clinical response and should be initiated as soon as possible in hopes of preventing chronicity and scarring.