Lupus Miliaris Disseminatus Faciei – Lest we Forget: A Report of Two Cases

Sir, Lupus Miliaris Disseminatus Faciei (LMDF) is an uncommon chronic inflammatory dermatosis of ambiguous etiology. It is clinically characterized by monomorphic papules predominantly on the face, which leave pigmented scars and is usually self-limiting. It is one of the intriguing granulomatous disorders in dermatology with numerous synonyms. Here, we discuss two such cases, who presented to us in the past 1 year. A 31-year-old male presented to our outpatient department with multiple asymptomatic skin-colored raised lesions on the face for 2 months, which were progressively increasing in size and number. There was no history of photosensitivity. There was no history suggestive of Hansen’s disease. Systemic examination was normal. Cutaneous examination revealed multiple, discrete, firm skin-colored to erythematous papules and nodules over infraorbital region, malar area, nose, and earlobes [Figure 1a and b]. Apple-jelly nodule-like appearance was not appreciated on diascopy. There was no perilesional erythema or telangiectasia. There were no other lesions elsewhere on the body.Figure 1: (a) Multiple discrete erythematous to skin-colored papules and nodules over cheeks and nose in the first patient, (b) papules over earlobe, (c and d) multiple skin-colored dome-shaped papules distributed symmetrically over periorbital area, nose, and earlobes in the second patientA 56-year-old male came with similar complaints of 6-month duration. On examination, there were multiple firm nontender dome-shaped skin-colored papules of size ranging from 2 to 4 mm diameter over forehead, periorbital region, nose, and ears [Figure 1c and d]. Surrounding skin was normal. Our differential diagnosis included acneiform eruption, sarcoidosis, LMDF, granulomatous rosacea, pseudolymphoma, and histoid Hansen’s disease. Routine investigations were within normal limits for both patients. Slit skin smear did not reveal any acid-fast bacilli. Mantoux test was negative, and chest X-ray was normal. Biopsy of a nodule from the right cheek revealed multiple well-defined granulomata in the dermis with a few Langhans giant cells in the first patient [Figure 2a-c]. Histopathology of papule from the second patient showed multiple well-defined granulomata with Langhans and foreign-body giant cells in the dermis with central caseation necrosis [Figure 3a-c]. Ziehl–Neelsen staining and periodic acid-Schiff staining were negative for mycobacteria and fungi, respectively, in both patients. A diagnosis of LMDF was made, and both patients were started on systemic steroids 0.5 mg/kg per day which was gradually tapered over a few months. Most of the lesions resolved in about a year in the first patient and the second patient was lost for follow-up.Figure 2: (a) Multiple well-defined granulomata in the dermis with lymphohistiocytic infiltrate (H and E, ×40), (b) granuloma with multiple epithelioid cells, histiocytes, giant cells, and lymphocytes (H and E, ×100), (c) Langhans giant cell inside granuloma (H and E, ×400)Figure 3: (a) Multiple granulomata in the dermis with central caseation necrosis (H and E, ×40), (b) epithelioid cell granuloma with histiocytes and lymphocytes (H and E, ×100), (c) foreign-body giant cell inside granuloma (H and E, ×400)LMDF was first described as “disseminated follicular lupus” by Fox in 1878. Later, the term “acne agminata” was used for this entity by Radcliffe-Crocker in 1903. The exact etiopathogenesis of LMDF is still not fully understood. Demodex folliculorum was suggested as its etiology by few authors which was not proven. Later, it was considered a tuberculid because of its histology, while some authors proposed it to be an extreme variant of granulomatous rosacea. Propionibacterium acnes was also suggested as a possible factor in etiology. A recent popular school of thought is that it is a granulomatous or a foreign-body reaction caused by immune response to follicular antigen released by damaged hair follicles or ruptured epidermal cysts.[1,2] Currently, majority of authors are of the opinion that LMDF is a distinct entity with characteristic histology, and the name Facial Idiopathic Granulomas with Regressive Evolution was proposed in 2000.[3] LMDF usually occurs in third or fourth decade of life with no clear gender predilection. It clinically presents with symmetrical skin-colored or reddish-brown papules which can be translucent, dome–shaped, and predominantly over centrofacial area. Lesions may cluster around eyelids, perioral area, cheeks, chin, and forehead. The involvement of lower eyelid which is characteristic of LMDF was seen in our second patient. New lesions can occur in crops, and they can be follicular or nonfollicular. Sometimes, few pustules or surrounding erythema are also noted. Although it may last for a few years if not treated, it is usually a self-limiting condition and resolution of papules leave pitted or pock-like scars and pigmentation. Extrafacial involvement is unusual and may involve scalp, ear lobes, neck, chest, axilla, arms, groin, legs, and genitalia. Our first patient had lesions on his ear lobes which is also an uncommon site for LMDF, previously reported by Nath et al.[4] Isolated extrafacial involvement of trunk and extremities without involvement of face was reported by Kou et al.[5] Diascopy of papules in LMDF can demonstrate apple-jelly nodule-like appearance. Dermoscopy can help in narrowing down the differential diagnosis. Orangish-brown background with central targetoid follicular plug surrounded by radiating white streaks is seen in dermoscopy in LMDF. The orangish-brown background corresponds to granulomatous inflammation and is seen in other granulomatous disorders also. The keratotic follicular plugs occurring due to lateral pressure on hair follicles are absent in other granulomatous conditions such as sarcoidosis and lupus vulgaris.[6] The classic histopathology picture of LMDF consists of dermal epithelioid cell granulomata with central caseous necrosis. However, there may be variations in this depending on the clinical stage of the lesions. Early or developing lesions may have only some lymphohistiocytic infiltrate around vessels and adnexa. Classic lesions which are fully developed may have epithelioid cell granulomata with or without central necrosis, epithelioid cell granulomata with abscess, or nonspecific nongranulomatous inflammatory infiltrate. Late lesions can present with perifollicular fibrosis and nonspecific inflammatory infiltrate in histopathology.[1,4] The various differential diagnosis to be considered include nodulocystic acne (polymorphic lesions, presence of comedones, and response to antibiotics), acneiform eruption (history of drug intake before onset of lesions), papular sarcoidosis (specific histopathology with naked granulomas and asteroid bodies, systemic involvement), granulomatous rosacea (symptoms triggered by spicy food, stress, photosensitivity, presence of surrounding erythema, and telangiectasia), histoid leprosy (characteristic histology with spindle-shaped cells and presence of acid-fast bacilli), granuloma faciale (solitary papule or few in number, telangiectasia, and follicular prominence on the surface), post-kala-azar dermal leishmaniasis (PKDL) (papules and nodules over infiltrated skin, associated hypopigmented lesions, and pseudolymphomas (characteristic lymphoproliferation in histology).[1] Treatment options include steroids, dapsone, doxycycline, minocycline, isotretinoin, clofazimine, anti-tuberculosis drugs, metronidazole, nicotinamide, and zinc.[4] Our first patient had already taken around 6 weeks of doxycycline with a diagnosis of acne vulgaris, with no response. However, he showed excellent response to systemic steroids within 4 weeks. A poor response to tetracyclines and mixed response to isotretinoin has been reported in literature.[1] Tranilast is a relatively new addition to this armamentarium and was first used in LMDF by Sato et al. in 2006.[7] It works by inhibiting collagen synthesis and fibroblast proliferation and has been proven to be efficacious in other granulomatous diseases such as sarcoidosis, granuloma annulare, and granulomatous cheilitis. Few topical agents have been tried including tacrolimus, erythromycin, and metronidazole. psoralen with ultraviolet A therapy (PUVA) and laser therapy using a 1450 nm diode laser or 1565 nm nonablative fractionated laser are also treatment options. Scarring may be prevented, and clinical course can be shortened by treatment with systemic steroids in early stages. In later stages, treatment of scarring can be attempted using 100% trichloroacetic acid and carbon dioxide laser.[1,4] Early diagnosis and appropriate treatment in LMDF can prevent sequelae like scarring. Our knowledge on etiopathogenesis and therapeutic interventions in LMDF are still evolving. It is imperative to keep the diagnosis of LMDF in mind while dealing with patients with papules over face. 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.