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Lupus Miliaris Disseminatus Faciei: A case report

  • Author(s): Esteves, Tiago;
  • Faria, Anabela;
  • Alves, Rubina;
  • Marote, Jorge;
  • Viana, Isabel;
  • Vale, Esmeralda
  • et al.
Main Content

Lupus Miliaris Disseminatus Faciei: A case report
Tiago Esteves, Anabela Faria, Rubina Alves, Jorge Marote, Isabel Viana, Esmeralda Vale
Dermatology Online Journal 16 (5): 10

Department of Dermatology, Hospital Central do Funchal, Funchal, Madeira, Portugal. tiago.castroesteves@gmail.com

Abstract

A 33-year-old man presented to our clinic with asymptomatic red-brown, dome-shaped papules, distributed bilaterally on the central area of the face (forehead, lower portions of the eyelids, nasolabial folds, and perioral areas) these had evolved over a period of about 1 year. A skin biopsy, taken from a lesion on the forehead, revealed an epithelioid cell granuloma with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells. The chest X-ray and the results of the laboratory studies were within normal limits; the Mantoux test was negative. The patient was treated with minocycline 100 mg/day for 4 months. There was significant clinical improvement, but papular lesions remained on the forehead. Later on, treatment with systemic steroids for 7 months resulted in the resolution of most lesions. Comment: 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. Originally, LMDF was considered to be a variant of lupus vulgaris or a tuberculid because of the histology, but there has been no evidence to date supporting a link to tuberculosis. Some authors consider LMDF to be a granulomatous forms of rosacea. However, our case supports the concept that it is a distinct entity.



Introduction

Lupus miliaris disseminatus faciei (LMDF), also termed acne agminata or acnitis, is a rare granulomatous skin disease mainly affecting the central area of the face. It shows a characteristic tendency to involve the lower eyelids. The eruption is characterized by small, discrete, red-brown or yellow papules, which occur singly or in crops, may be follicular or nonfollicular, develop rapidly, and in many cases show a pustular top. Extrafacial involvement is occasionally observed [1]. The eruption runs a chronic course and usually involutes spontaneously in about 12-24 months leaving small pitted scars [2]. Histopathology shows caseating and noncaseating epithelioid cell granulomas, which may resemble tuberculosis, sarcoidosis, granulomatous rosacea, and other granulomatous disorders. However, the histopathogenesis of this disorder is still unknown.


Case report


Figure 1Figure 2

A 33-year-old man presented to our clinic with asymptomatic red-brown, dome-shaped papules, varying between 2 and 4 mm in size. The papules were distributed bilaterally on the central area of the face (forehead, lower portions of the eyelids, nasolabial folds and perioral areas) (Figures 1 and 2) and had evolved over about about 1 year. The patient had no additional cutaneous lesions. Erythema, telangiectasias, and flushing were absent. The patient denied the application of topical steroids on the face at any time and there was no family history of similar eruptions. A skin biopsy taken from a lesion on the forehead revealed an epithelioid cell granuloma with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells (Figure 3). The chest X-ray and the results of routine laboratory studies were within normal limits; the Mantoux test was negative. A diagnosis of LMDF was made and oral minocycline treatment (100 mg/day) was initiated. The treatment was continued for 4 months. The eruption improved significantly, but some papules on the forhead remained. Later on, treatment with oral deflazacort for 7 months (30 mg/day and gradual decrease to 7.5 mg/day) resulted in the resolution of most lesions; some of them healed leaving depressed scars (Figure 4).


Figure 3Figure 4

Discussion

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, which can affect adults and adolescents of both sexes.

The histopathological hallmark of the disease is an epithelioid cell granuloma with central necrosis. However, the histological pattern can vary according to the timing of the biopsy. Early lesions may show superficial perivascular infiltrates of lymphocytes, histiocytes, and occasional neutrophils. Late lesions have these changes together with dermal fibrosis, particularly around hair follicles. Established lesions may show tuberculoid or suppurative granulomas.

Originally, LMDF was considered to be a variant of lupus vulgaris or a tuberculid because of the histology, but there has been no evidence to date supporting a link to tuberculosis [3]. Evidence against this association includes: a variable cutaneous hypersensitivity response to tuberculin, the absence of concomitant tuberculosis, the absence of bacilli in lesions, the failure to respond to antituberculous therapy in many cases, and the failure of PCR techniques to demonstrate the DNA of M. tuberculosis in lesional skin [3].

The pathogenesis of LMDF remains controversial. Some authors view it as a particular form of granulomatous rosacea (GR) [4]. Support for this, comes primarily from the observation that in LMDF the granulomas appear in association with pilosebaceous units [5, 6] and epithelioid granulomas have been found in some patients with rosacea. However, there appear to be many differences between the two, making it hard to accept this concept. The predominance in young adults or adolescents, the involvement of extrafacial sites and areas typically spared by rosacea (lower eyelids), and the absence of erythema, flushing, and telangiectasia argue against LMDF being a form of rosacea. Furthermore, LMDF has an inconsistent response to oral tetracyclines, an occasional response to oral steroids and a self-limited course with scarring, inconsistent with the diagnosis of rosacea. Although dermal epithelioid cell granulomas are observed in both histologically, caseation necrosis is rarely observed in GR. In view of these striking differences, LMDF should be regarded as separate entity and not as part of the spectrum of rosacea [7].

Grosshans, Kremer and Maleville (1974) regarded this granuloma formation as a delayed-type hypersensitivity reaction to the mite. Demodex folliculorum, but the Demodex association has not been confirmed [8, 9, 10]. Conversely, others suggest that LMDF is a granulomatous reaction to hair follicle destruction or ruptured epidermal cysts [11, 12].

Lastly, it has also been proposed that LMDF may represent a micropapular form of sarcoidosis [13]. The clinical features, histopathologic features (sarcoidal-type granulomas), and course of LMDF are often similar to cutaneous sarcoidosis. However, sarcoidosis can generally be excluded by the physical examination, chest Xray, and laboratory tests.

Actually, LMDF may be considered a distinct entity. Nevertheless, because the currently used name may be confusing, Skowron et al. proposed, in 2000, a name change from LMDF to FIGURE (facial idiopathic granulomas with regressive evolution) [14]; to date, this name change does not appear to have been widely accepted.

Usually, this disease is difficult to control. Because LMDF spontaneously resolves within 1-2 years, the impact of therapy on the course of the disease is difficult to assess. A variety of treatments are reportedly of some benefit, but controlled studies to establish the best treatment are lacking. Tetracycline, glucocorticoids, antituberculous medications, and antimalarial drugs have been found to be effective in some patients. Tetracyclines are the usual first-line treatment in acne agminata, whereas dapsone has been shown to prevent new lesions and shorten the total duration of disease [15]. Prevention of scarring may be possible with early intervention using low dose corticosteroids over a 3-month period [16]. In addition, isotretinoin has been reported to be effective in cases of LMDF [17, 18]. The efficacy of isotretinoin in LMDF may be related to its effects on the immune system.

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