Two Cases of Lupus Miliaris Disseminatus Faciei Treated with Oral Mini-pulse Steroid Therapy / 대한피부과학회지

Lupus miliaris disseminatus faciei (LMDF) is a rare granulomatous skin disease mainly affecting the central area of the face. A variety of treatments are reportedly of some benefit; however, controlled studies to establish the best treatment are lacking. Here, we report the cases of a 33-year-old man who presented with multiple, various-sized, erythematous maculopapules on the face and a 19-year-old man who presented multiple, reddish papuloplaques distributed on the face. Histopathological examinations of the two cases revealed large clear-boundary epithelioid cell granulomas with central necrosis surrounded by lymphocytic infiltration. Based on the clinical and histological findings, diagnoses of LMDF were made. As oral tetracycline and antimalarials were not fully effective in our cases, oral mini-pulse steroid therapy (dexamethasone, 5 mg bid for 2 days per week) was initiated. After several months, the eruption significantly improved, and most lesions were resolved. Here, we report two cases of LMDF successfully treated with oral mini-pulse steroid therapy.

Lupus miliar diseminado facial: presentación de un caso clínico y revisión de la literatura / Lupus miliar disseminated facial: Presentation of a clinical case and review of the literature

El lupus miliar diseminado facial es una enfermedad inflamatoria granulomatosa de etiología incierta, caracterizada por pápulas eritemato-amarillentas que afectan predominantemente la región facial. Luego de 1 a 4 años puede involucionar sin tratamiento pero dejando secuelas estéticas. Existen múltiples tratamientos con respuestas variables. Presentamos un caso con respuesta favorable al tratamiento con isotretinoína, sin recaídas a la fecha, y realizamos una revisión de la literatura (AU)

Lupus miliaris disseminatus faciei is a granulomatous inflammatory disease of unknown etiology, characterized by erythematous yellowish papules predominantly affecting facial region. After 1-4 years it may involute without treatment but leaving scarring sequelae. There are multiple treatments with variable responses. A case with favorable response to isotretinoin treatment, with no relapses to date, is reported and literature review is made (AU)

Lupus Miliaris Disseminatus Faciei Associated with Milium / 대한피부과학회지

Lupus miliaris disseminatus faciei (LMDF) is a chronic inflammatory disorder primarily affecting the face. A 22-year-old man presented with facial skin lesions. Physical examination revealed scattered pin-head sized erythematous to yellowish papules. Skin biopsy from the erythematous papule showed keratinous material in the layers of the thin epithelium and caseous necrosis with adjacent epitheloid granuloma and giant cells. After mechanical extractions of milium-like inflamed papules, skin lesions improved leaving an atrophic scar. LMDF was initially thought to be associated with tuberculosis infection but today it is considered a variant of rosacea. However, there have been reports of LMDF in association with epidermal cysts or milium, suggesting an abnormal immune response to the pilosebaceous unit may play a role. Damage to the pilosebaceous unit that is further complicated by milium rupture may induce inflammation resulting in LMDF. We suggest that milium or epidermal cysts may also attribute to the pathogenesis of LMDF although further studies are needed.

The Clinical and Histopathologic Features of 30 Cases of Lupus Miliaris Disseminatus Faciei / 대한피부과학회지

BACKGROUND: Lupus miliaris disseminatus faciei (LMDF) is a rare granulomatous disease characterized by eruption of discrete red-brown, dome-shaped papules, predominantly in the lower eyelid. Histopathologically, LMDF could present as caseous necrosis in the central area with granulomas composed of epithelioid cells and giant cells. The pathogenesis of LMDF remains controversial. Some suspect LMDF as a kind of cutanoeus tuberculosis, but others believe LMDF to be a variant of granulomatous rosacea or sarcoidosis. OBJECTIVE: This study aimed to investigate the clinical and histopathologic features of LMDF. METHODS: We reviewed the medical records, clinical photographs, and histopathologic slides of 30 cases of LMDF that were evaluated in our department between February 1991 and May 2009. RESULTS: The average age of the affected patient was 34.6+/-10.9 years and the ratio of male-to-female patients was 3.3 : 1. The most common sites of involvement with LMDF was the eyelid (86.7%). cheek (76.7%), philtrum (60.0%), and glabella (56.7%). On histopathologic examination, the epithelioid granulomas had a connection to pilosebaceous structures in 66.7% of the cases. Follicular rupture (33.3%), perifollicular granulomas composed of naked tubercles (53.2%), and sarcoidal granulomas with abscesses (23.3%) were noted. Characteristic caseous necrosis occurred in 7 cases (23.3%). The duration of LMDF was 2 years in 26 cases (86.7%) and the lesion improved leaving scar in 21 cases (70%). CONCLUSION: We identified the histopathologic changes of LMDF as a function of disease duration. Connections of pilosebaceous units with granulomas were commonly observed. It will be helpful to understand the pathophysiology of LMDF.

A Case of Lupus Miliaris Disseminatus Faciei on the Neck / 대한피부과학회지

Lupus miliaris disseminatus faciei (LMDF) is an eruption of discrete red-brown, dome-shaped papules, histologically characterized by epithelioid cell granulomas. Although it is thought that LMDF might occur by an unusual reaction to pilosebaceous units, the exact reason for facial predominant occurrence is not clear. There have been extrafacial reports of LMDF on the axillae, shoulders, arms, hands, groins and legs together with facial lesions, but reports on LMDF solely involving the extrafacial areas have not been made. Herein we report a rare case of LMDF on the neck of a patient without any facial manifestations.

A Case of Facial and Axillary Lupus Miliaris Disseminatus Faciei / 대한피부과학회지

Lupus miliaris disseminatus faciei (LMDF) is a papulo-pustular eruption typically affecting the face of young adults. It is characterized histologically by the presence of caseating granuloma in the dermis. The lesions are typically described to affect the periorbital and muzzle skin of the face, however, only three cases with axillary involvement have been described in the literature. We report a case of LMDF on the face and left axilla of a 44-year-old female. She presented with a 7-month history of a mildly-pruritic papulopustular eruption on the face. The histologic examination showed the homogenous necrosis surrounded by granulomatous inflammation containing epithelioid macrophages, lymphocytes, and multinucleated giant cells in the dermis. Two months later, a solitary palpable nodule developed on the left axilla and its histological features showed similarity with the eruption on the face. A diagnosis of LMDF was made based on the clinical and histological findings. We hereby report an interesting and rare case of LMDF with axillary involvement.

A Case of Fungal Folliculitis Clinically Mimicking Lupus Miliaris Disseminatus Faciei / 대한피부과학회지

Dermatophytes generally cause infection of the stratum corneum and superficial layers of skin. They are, therefore, found less frequently in the rete layer and do not ordinarily penetrate deeper than the basal cell layer. Nevertheless, under special circumstances such as the pressure generated during trauma or long term topical steroid application, the fungus elements of some dermatophytes will be forced into the dermis where they instigate a chronic granulomatous reaction. We report a 43-year-old male who presented with multiple erythematous brown colored dome-shaped papules on both periorbital areas, which were clinically similar to lupus miliaris disseminatus faciei (LMDF). Histologic examination revealed follicular and perifollicular infiltration by inflammatory cells, and perifollicular granuloma. Trichophyton species were isolated from the fungus culture of the lesional tissue. The patient was treated with an antifungal agent, resulting in clearance of the skin lesions, and there was no recurrence of skin lesions during a 13-month follow-up period. We suggest that it is important to perform biopsies and cultures on periorbital papules which mimick LMDF, refractory to conventional therapy.

Three Cases of Lupus Miliaris Disseminatus Faciei Treated by Dapsone / 대한피부과학회지

Lupus miliaris disseminatus faciei(LMDF) is a erythematous to yellowish asymptomatic papular eruption affecting the cental of the face, which runs a chronic course and terminating spontaneously with scarring. Histopathologically, epithelioid cell granuloma with caseation necrosis is seen in the dermis. Although the disease is self limited, the etiology and pathogenesis are unknown, so there is no effective treatment for LMDF. We report 3 cases of LMDF patients treated with Dapsone with effective results compared to natural history of this disease.

Two Cases of Lupus Miliaris Disseminatus Faciei Treated with Dapsone / 대한피부과학회지

Lupus miliaris disseminatus faciei (LMDF) is an asymptomatic papular eruption affecting the central area of the face. It runs a chronic course and can involute spontaneously with scarring. The treatment of LMDF has not been satisfactory despite the use of many different kinds of topical and systemic agents. In the early 1980s, Kumano et al. reported their experiences with this disease using dapsone; the efficacy of dapsone was described as excellent. With this report in mind, we have used dapsone(50-100mg/d) in 2 patients with LMDF, and a marked irnprovement or almost cure of the skin disease were recognized.