Lupus miliaris disseminatus faciei: An uncommon dermatologic entity

Anjali Madan; Vineet Rehlan; Vijay Kumar Garg; Nidhi Mahajan

doi:10.4103/0975-9727.212411

CASE REPORT

Year : 2017 | Volume : 8 | Issue : 2 | Page : 91-93

Lupus miliaris disseminatus faciei: An uncommon dermatologic entity

Anjali Madan¹, Vineet Rehlan¹, Vijay Kumar Garg¹, Nidhi Mahajan²
¹ Department of Dermatology, Maulana Azad Medical College, New Delhi, India
² Department of Pathology, Maulana Azad Medical College, New Delhi, India

Date of Web Publication

7-Aug-2017

Correspondence Address:
Anjali Madan
Department of Dermatology, Maulana Azad Medical College, New Delhi
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0975-9727.212411

Abstract

Lupus miliaris disseminatus faciei (LMDF) first described in 1878 is an uncommon dermatosis of unknown etiology with characteristic clinicopathological features. It mainly affecting the central area of the face showing a characteristic tendency to involve the lower eyelids. Various treatments have been tried with tetracyclines, isotretinoin, dapsone, prednisolone, clofazimine, isoniazid and corticosteroids, with variable results. We present the case of a 30 year old patient with characteristic complaints of eruption of multiple, discrete reddish raised asymptomatic lesion all over face with histopathology suggestive of Lupus miliaris disseminatus faciei

Keywords: Lupus miliaris disseminatus faciei (LMDF), morphea, tuberculosis

How to cite this article:
Madan A, Rehlan V, Garg VK, Mahajan N. Lupus miliaris disseminatus faciei: An uncommon dermatologic entity. Muller J Med Sci Res 2017;8:91-3

How to cite this URL:
Madan A, Rehlan V, Garg VK, Mahajan N. Lupus miliaris disseminatus faciei: An uncommon dermatologic entity. Muller J Med Sci Res [serial online] 2017 [cited 2023 Jun 2];8:91-3. Available from: https://www.mjmsr.net/text.asp?2017/8/2/91/212411

Introduction

Lupus miliaris disseminatus faciei (LMDF) is an uncommon dermatologic entity, characterized clinically by the presence of discrete, red-brown, dome-shaped papules on the face that resolve with pitted scars.^[1] It is a granulomatous skin disease mainly affecting the central area of the face showing a characteristic tendency to involve the lower eyelids.^[2]

It was once thought to be a tuberculid because of its histology; however, many authors now consider LMDF to be an extreme variant of granulomatous rosacea (GR). Many are of the opinion that it is a distinct entity because of its characteristic histology and occasional involvement of the noncentral facial areas.^[3]

It responds unsatisfactorily to treatment with tetracycline-class antibiotics and oral isotretinoin, which are considered the mainstay of treatment.

Case Report

A 30-year-old lady presented to the outpatient department, with complaints of eruption of multiple, discrete reddish raised asymptomatic lesion all over face since past 4 months. These lesions used to appear in crops. History of photosensitivity was absent. There was no history of fever, weight loss, or chest infection. There was no significant family or past history. Patient was not on any medication for the illness or any topical application.

Physical examination revealed multiple, discrete erythematous monomorphic papules over bilateral cheeks, forehead, chin, and neck region. The surface of lesions was smooth and the surrounding skin was normal. Some of the lesions were tender to touch [Figure 1].

Figure 1: Multiple monomorphic papules over face

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Diascopy of lesions showed apple-jelly nodule-like appearance. Rest of the physical examination was normal and no systemic abnormality was detected. We kept the differential diagnosis of LMDF, papular sarcoidosis, papular granuloma annulare, and Hansen's disease.

Slit skin smear examination showed no bacilli. Smears for Leishman–Donovan (LD) bodies were negative. On laboratory investigations, complete blood count, erythrocyte sedimentation rate (ESR), serum angiotensin converting enzyme (ACE) level, and calcium were normal. Mantoux and chest x-ray was normal.

Histopathology of the lesion showing mild focal acanthosis with focal follicular plugging. The upper dermis shows few epithelioid cell granulomas with presence of giant cells, surrounded by chronic inflammatory cell infiltrate [Figure 2] and [Figure 3]. Special stains such as acid-fast bacillus AFB and periodic acid — Schiff (PAS) were both negative.

Figure 2: Fragmented skin biopsy showing mild focal acanthosis with focal follicular plugging. The upper dermis shows few epithelioid cell granulomas with the presence of giant cells, surrounded by chronic inflammatory cell infiltrate. (Scanner view, hematoxylin & eosin stain, 40×)

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Figure 3: High power view of the epithelioid cell granuloma, showing presence of Langhans type giant cell and lymphocytic cuffing (hematoxylin & eosin stain, 600×)

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The patient was given dapsone (100 mg daily) for five months. Regular follow-up was done and disease resolved with no eruption of new skin lesions after starting the treatment and previous lesions healed without significant scarring [Figure 4].

Figure 4: Improvement in lesions after dapsone therapy

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Based on the typical clinical findings, histopathology, and treatment response to dapsone, a final diagnosis of LMDF was made.

Discussion

LMDF first described by Fox et al. in 1878 is a rare dermatosis with characteristic clinicopathological features but of unknown etiology.^[4] The onset tends to be fairly rapid, and lesions may persist for months to years without treatment. Residual pitted scars are a characteristic feature of this disease. The periorbital area, the nasolabial folds, the cheeks, and the perioral areas are commonly involved that is similar to that seen in acne vulgaris and rosacea as well. Extrafascial involvement in LMDF has rarely been reported in the literature.

In 2000, Skowron et al. proposed a change of name from LMDF to facial idiopathic granulomas with regressive evolution (FIGURE); however, this name-change does not appear to have been widely accepted throughout the dermatologist community.^[5]

LMDF is known to affect only the skin. Multiple theories of its pathogenesis have been proposed. While some authors have suggested it to be a reaction to Demodex folliculorum, studies also suggest it to be a granulomatous reaction to hair follicle destruction or ruptured epidermal cyst.^[6] Studies have failed to demonstrate Mycobacterium tuberculosis or other mycobacterial disease by culture or polymer reaction studies.^[7] Sarcoidosis can present similarly like LMDF. However, sarcoidosis may be associated with systemic manifestations, mediastinal lymphadenopathy, lung lesions, and increased levels of the angiotensin-converting enzyme, features that are not seen in LMDF.^[8]

LMDF has characteristic histopathological features: Epithelioid granulomas with or without central necrosis and occasional multinucleated giant cells in the dermis, epithelioid cell granuloma without central necrosis (sarcoidal-type granuloma); epithelioid cell granuloma with abscess; and nongranulomatous, nonspecific inflammatory infiltration.^[1] Diascopy of larger lesions often reveals an apple-jelly nodule-like appearance. Caseation necrosis is variable and usually seen in fully developed LMDF lesions.^[9]

LMDF has long been viewed as a variant of GR. However, the predominance of LMDF in younger adults, involvement of facial areas typically spared by rosacea (in the lower eyelids), involvement of extrafascial sites and the absence of erythema and telangiectasia go against this entity being a form of rosacea.^[10]

Both sexes can be affected with LMDF; however, males predominate in the many studies cited by us in this case report. Young adults in their 20s most often are seen to be affected by this dermatological disorder.^[11]

Differential diagnosis include clinical entities such as acne, lupus vulgaris, GR, sarcoidosis, and histoid leprosy.

Therapy is difficult with variable efficacy. The treatment options for LMDF are tetracyclines, isotretinoin, dapsone, prednisolone, clofazimine, isoniazid, and corticosteroids. Standard treatment is oral tetracycline; however, clinical improvement may take 3-6 months, and long-term maintenance therapy is needed.^[12] LMDF has a variable response to oral tetracyclines, an occasional response to oral steroids and a self-limited course with scarring. This feature also goes against its relation with rosacea.^[13]

The 1,450-nm diode laser has also recently been shown to be fairly effective in the treatment.^[14] It has been recommended to treat the lesions early in order to prevent the development of depressed scars.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

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