Lupus miliaris disseminatus faciei with unusual distribution of lesions.

Sir,

Lupus miliaris disseminatus faciei (LMDF) is a granulomatous eruption characterized by monomorphic, reddish-brown papules and nodules predominantly localized on the face.[1] It was initially thought to be a hypersensitivity reaction to tuberculosis, but a conclusive relationship to tuberculosis has not been established. Now a days, it is considered to be a variant of rosacea. Extrafacial involvement in LMDF has been rarely reported in the literature. We hereby report a case of LMDF with typical morphology of the lesions with extensive facial lesions and extrafacial localization.

A 36-year-old male presented with 4 months history of asymptomatic, reddish-raised lesions over the face, earlobes, and the neck. The lesions initially appeared on the chin and left side of the neck, which progressively involved his forehead, both the cheeks, earlobes, upper eyelids, and whole of his neck over a period of 2 months. He works in biscuit wrapper manufacturing unit; but there is no history of contact with any chemical or fumes. His wife was treated (6 months treatment) for tuberculous lymphadenitis a year back; but he himself never had cough, expectoration, hemoptysis, or weight loss. There was no history of sexual promiscuity or any genital ulcer disease in the past. He did not suffer from any medical problem. On examination, multiple, erythematous, firm papules and nodules of size ranging from 0.5 to 2 cm were seen on forehead, upper eyelids, malar region, nose, beard area, chin, earlobes, submandibular region, the entire neck and shoulder [Figure 1a–b]. A few lesions showed scaling on the surface. These lesions were larger and more coalescent on the neck. Diascopy revealed apple-jelly appearance in some of the lesions. There was no cervical lymphadenopathy. Tuberculin test was negative. Chest X-ray was unremarkable. Venereal disease research laboratory (VDRL) test was non-reactive. Biopsy from a representative skin nodule showed mild epidermal hyperplasia and granulomatous reaction pattern in the dermis. Granulomas were composed of epithelioid cells, plenty of Langhan's and foreign body giant cells surrounded by a lymphocytic cuff [Figure 2]. Extensive caseous necrosis was seen in the center of multiple granulomas [Figures 3 and 4]. A tendency toward periappendageal localization of the granulomas was also noted. Stain for acid-fast bacilli and fungi were negative. A final diagnosis of lupus miliaris disseminatus faciei was made based on the above-mentioned features. A trial of anti-tubercular therapy was given for 2 months, but no response was seen, rather he had developed some new lesions (including one papule on the right upper chest). He was put on minocycline 100 mg daily and is under follow up.

Figure 1

(a) Papules and nodules of LMDF over the face and neck. (b) Close up of neck

Figure 2

Photomicrograph showing epidermis and dermis with granulomatous infiltrate with caseous necrosis in superficial dermis around appendages as well as deep dermis (hematoxylin and eosin ×40)

Figure 3

Photomicrograph showing caseous necrosis with collection of epithelioid cells around the caseous necrosis, and a few giant cells (hematoxylin and eosin ×200)

Figure 4

Photomicrograph demonstrating epithelioid cell granulomas with many giant cells (hematoxylin and eosin ×400)

Typical skin lesions of LMDF are multiple discrete, smooth, 1-3 mm, monomorphic, symmetrical reddish-brown or brown-to-yellowish dome-shaped translucent papules and nodules occurring on the chin, forehead, cheeks, and eyelids.[1] Tiny pustules may rarely accompany the papules. Surrounding erythema is not a characteristic feature, but may be present. Diascopy of larger lesions often reveals an apple-jelly nodule-like appearance.[2] The eruption tends to be self-limiting, resolving completely over a few months or up to 2 years often leaving behind pock-like scars.[1]

LMDF typically occurs as symmetrically distributed eruptions in the center of the face.[2] The lower portions of the eyelids, the lower portions of the forehead, the nasolabial folds, the cheeks, and the perioral areas are preferentially involved, but occasionally more widespread dissemination occurs.[2] Several other sites like axillae,[3] chest,[4] neck, arms, hands, legs, and groin[1] have been reported to be rarely involved in LMDF. Our patient had extensive involvement of the face, with unusual involvement of the ear lobes, neck, and the shoulder girdle.

Characteristic histopathology includes a tubercle consisting of aggregates of epithelioid histiocytes and occasional multinucleate giant cells, which surround a usually large area of caseous necrosis.[5] This typical histopathological feature, however, is not consistently seen. Caseation necrosis is variable and usually seen in fully developed LMDF lesions.[6] A sparse lymphoid collection surrounds the tubercle in the periphery giving a sarcoidal rather than tuberculoid appearance to the granuloma.[56] Shitara[2] delineated the histopathological findings into four groups: epithelioid cell granuloma with central necrosis, epithelioid cell granuloma without central necrosis (sarcoid/foreign body reaction), epithelioid cell granuloma with abscesses, and nongranulomatous nonspecific inflammatory infiltrate.

Therapy is difficult with variable efficacy and several therapeutic modalities, e.g., dapsone, doxycycline, minocycline, isotretinoin, clofazimine, isoniazid, and corticosteroids have been used to effectively induce remission.[17] Topical agents such as psoralen with ultraviolet A therapy (PUVA), erythromycin and metronidazole have also been used.[1] Considering LMDF a condition related to rosacea, it may be worthwhile trying oral nicomide containing nicotinamide and zinc, which has been found to be an effective therapy in rosacea.[8] Topical tacrolimus, on the other hand may induce rosacea like dermatitis.[910]