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Advances in dermatology and venereology Acta Dermato-Venereologica
Lupus Miliaris Disseminatus Faciei: Not Only a Facial Dermatosis
Marthe-Lisa SCHAARSCHMIDT 1, Michèle SCHLICH 2, Janina STAUB 3, Astrid SCHMIEDER 1, Sergij GOERDT 1 and Wiebke K. PEITSCH 4 *
1
Department of Dermatology, Venereology and Allergology, University Medical Center Mannheim, Heidelberg University, Mannheim,
2
Dermatological Practice Dr. Krekel, Speyer, 3 Department of Dermatology and Venereology, University Medical Center, Hamburg-Eppendorf, and 4 Department of Dermatology and Phlebology, Vivantes Klinikum im Friedrichshain, Landsberger Allee 49, DE-10249 Berlin, Germany. * E-mail: wiebke. ludwig-peitsch @ vivantes. de Accepted Jan 24, 2017; Epub ahead of print Jan 25, 2017
Lupus miliaris disseminatus faciei( LMDF), synonymously termed acne agminata( 1), is an uncommon granulomatous inflammatory disease usually affecting the central face and the eyelids( 1 – 3). Rare extrafacial manifestations have been reported( 1, 3 – 7). We present here a case of LMDF with involvement of the face, scalp and axillae and provide a summary on the characteristics of the disease and treatment options.
CASE REPORT
A 28-year-old man presented with a 3-month history of asymptomatic papules, nodules and pustules, which had initially appeared on his eyelids and in the axillae and subsequently spread over his face. Treatment with different topical agents, including antibiotics, nystatin, aciclovir and glucocorticoids, as well as oral doxycycline and oral valaciclovir, had been ineffective. The patient had no systemic symptoms or concomitant diseases and did not take any medications. He did not have any history of acne or rosacea, and denied use of aluminium-containing antiperspirants. Clinical examination showed multiple distinct red-brown, sometimes yellowish, papules and nodules, sized 2 – 10 mm, on the scalp, forehead, glabella, upper and lower eyelids, cheeks and chin( Fig. 1a) as well as in both axillae( Fig. 1b) with a light brown-yellowish infiltrate on diascopy. Diffuse erythema and telangiectasia were absent. Histopathological examination revealed dermal epithelioid cell granulomas with central caseating necrosis( Fig. 1c, d). Microscopy for pathogens with periodic acid – Schiff( PAS), Grocott and Ziehl-Neelsen stains, as well as cultures for Mycobacterium tuberculosis and atypical mycobacteria were negative. A bacterial swab yielded
Pseudomonas aeruginosa. Laboratory examinations including serum levels of angiotensin-converting enzyme and tuberculosis test with interferon gamma release assay( QuantiFERON-TB test, QIAGEN, Hilden, Germany) were unremarkable. Based on clinical and histopathological findings a diagnosis of LMDF with axillary involvement was established. Compatible with the results of antimicrobial susceptibility testing, a treatment attempt was made with ciprofloxacin 500 mg twice daily for 2 weeks, but without success. Subsequently isotretinoin 30 mg / day( 0.4 mg / kg) was initiated and continued for one year with regular laboratory monitoring. This resulted in clearing of the lesions on the face and scalp with some residual scars, but the axillary lesions remained.
DISCUSSION
LMDF usually affects young adults of both sexes, but it has also been reported in children( 8) and elderly patients( 9, 10). It typically presents with yellowishbrown dome-shaped papules and pustules localized in the central face, commonly involving the eyelids( 1 – 3). Moreover, extrafacial lesions in the axillae, scalp, trunk, genitalia, neck, hands and legs have been described( 1, 3 – 7). They may occur in addition to facial lesions( 1, 3, 4, 6, 7) or, rarely, as the only manifestation of LMDF( 4, 5). To make allowance for lesions in non-facial sites Nemer & McGirt recently suggested renaming LMDF“ lupus miliaris disseminatus”( 6).
The papules have a yellowish-brown(‘’ apple-jelly’’) infiltrate on diascopy. On histopathological examination
Fig. 1. Clinical and histopathological findings.( a, b) Multiple red-brown and yellowish papules and nodules in the face, on the scalp and in the axilla.( c, d) Haematoxylin-eosin stained skin biopsy specimen from the face showing dermal epithelioid cell granulomas with central areas of caseating necrosis. Magnification:( c, survey) × 20 or( d, detail) × 100. A biopsy from the axilla yielded identical findings. Written permission is given to publish these photographs.
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica. doi: 10.2340 / 00015555-2616 Acta Derm Venereol 2017; 97: 655 – 656