CED
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Dermoscopic evaluation of two patients with lupus miliaris disseminatus faciei E. Ayhan,1 U. Alabalik2 and Y. Avci2 1 Department of Dermatology, Zile State Hospital, Tokat, Turkey; and 2Department of Pathology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey
doi:10.1111/ced.12331
Summary
Lupus miliaris disseminatus faciei (LMDF) is a chronic and uncommon inflammatory dermatosis, characterized by yellowish-red papules, especially on the face and around the eyelids. It is considered to be a reaction to destroyed hair follicles. We dermoscopically evaluated two patients diagnosed with LMDF. Dermoscopy revealed follicular keratotic plugs and vascular structures. We discuss these cases and other diseases dermoscopically showing keratotic plugs.
Lupus miliaris disseminatus faciei (LMDF) is an uncommon, inflammatory, and granulomatous disease of unknown aetiology.1 Clinically, reddish-yellow or yellowish-brown papules are seen on the face, typically on the eyelids.2 Some researchers consider LMDF to be a variation of granulomatous rosacea, while others disagree. We dermoscopically evaluated two patients diagnosed with LMDF.
Report The two patients were both admitted to our clinic with papillary lesions on the face and neck. Patient 1 was a 38-year-old man (Fig. 1). On physical examination, he was found to have erythematous papules, some with ulceration at the centre, on his forehead, cheeks, eyelids and neck. Patient 2 was a 52-year-old woman (Fig. 2), who had numerous papules on her cheeks, some of which were skin-coloured and others erythematous. Skin biopsies were taken from the faces of both patients, and histological examination revealed a granulomatous reaction with caseification necrosis Correspondence: Dr Erhan Ayhan, Cumhuriyet Caddesi 22. Sokak No: 34 € Yozgat Yolu Uzeri /Zile/Tokat, Turkey E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 December 2013
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Clinical and Experimental Dermatology (2014) 39, pp500–502
indicative of LMDF, along with follicular hyperkeratosis. Dermoscopy was performed, and revealed follicular plugs in the lesions, some resembling a target surrounding the centre, with hairpin-like and linear vessels on an orange-yellow and erythematous background. Patient 1 received tetracycline treatment, which produced no benefit. He was prescribed isotretinoin, but severe photocontact dermatitis appeared after this treatment. Patient 2 did not want any treatment. It is believed that LMDF is that the result of an autoimmune reaction resulting from the rupture of hair follicles, which causes granulomatous disease.3 Because LMDF has a granulomatous histopathological pattern, it has been considered a variation of lupus vulgaris or a form of tuberculid.4 However, this theory is no longer considered correct, because of factors such as the self-healing nature of the disease, lack of identification of a causative organism, and lack of response to anti-tuberculosis treatments.5 It has also been suggested that LMDF might be a form of sarcoidosis because of its clinical appearance; however the accompaniment of caseification necrosis and lack of other findings indicating sarcoidosis dissuaded Skowron et al. 6 from making this diagnosis. Another theory is that LMDF is a variation of granulomatous rosacea (GR). However, some features of GR, including its chronic nature, incidence of flushing,
ª 2014 British Association of Dermatologists
Dermoscopic evaluation of two patients with LMDF E. Ayhan et al.
(a)
(b)
(c)
(d)
Figure 1 (a) Patient 1 had a large num-
ber of erythematous papules on his face. (b) Linear and hairpin-like vessels, targetoid flame-like follicular plugs and ellipsoid follicular plugs were visible around the ulceration (original magnification 920). (c) Targetoid follicular plugs (white arrows), along with linear and hairpinlike vessels, surrounded the centre of a few of these papules (original magnification 910). (d) A schematic representation of the appearance of (c).
(a)
(b)
(c)
(d)
Figure 2 (a) Patient 2 had a few papules
on her face, with follicular plugs (white arrows). (b) Linear vessels (black arrows) and follicular plugs (white arrows). (c) Follicular plugs (thin white arrows). (d) hairpin vessels (thick white arrows) and linear vessel (black arrow). Original magnification (b–d) 910.
worsening with alcohol and spicy foods, incidence of Demodex parasites, inability to regress, and production of scarring indicate that LMDF is a different disease.7 Skowron et al. have stated that it should be renamed as a separate entity, proposing the acronym FIGURE (facial idiopathic granulomas with regressive evolution).6
ª 2014 British Association of Dermatologists
In the current study, dermoscopy showed keratotic follicular plugs and vascular structures in the papillary lesions. Keratotic plugs develop as a result of lateral pressure on hair follicles.8 Keratotic follicular plugs lead to a ‘strawberry pattern’ appearance on a background of nonpigmented actinic keratosis (AK). In this pattern, a noticeable pinkish-red pseudo-network develops
Clinical and Experimental Dermatology (2014) 39, pp500–502
501
Dermoscopic evaluation of two patients with LMDF E. Ayhan et al.
around the hair follicles intially, then oval, yellow-white keratotic plugs, mostly targetoid in appearance, start to appear.9 During the transformation of AK to intraepidermal carcinoma, the appearance is of a keratin mass, with small ulcerations in the middle, and surrounding oval, elliptic targetoid keratotic plugs, an appearance described by Zalaudek et al.10 as a red starburst pattern. Keratotic plugs have also been found in squamous cell carcinoma (SCC) and in keratoacanthoma.10 In addition, two independent studies on skin leishmaniasis have also found keratotic plugs, described as ‘teardrop-like’ structures.8,11 Although this feature was not described, the images in these studies show that these structures are targetoid in appearance. Similar keratotic plugs were seen in the lesions of patients with discoid lupus erythematosus.12 Keratotic plugs appear in the facial forms of DLE, AK, SCC, intraepidermal carcinoma and cutaneous leishmaniasis. Other skin diseases that should be considered in the differential diagnoses of LMDF, such as sarcoidosis13 and lupus vulgaris,13 reveal no follicular plugs. In conclusion, we report two patients with LMDF, which to our knowledge, are the first reported cases where dermoscopy has been used for evaluation. Dermoscopy may be of help in differentiating LMDF from other diseases that may be considered in the differential diagnosis.
Learning points ● LMDF is a granulomatous disease of unknown
aetiology. ● A similar condition, GR, is thought to be a var-
iant form of sarcoidosis and tuberculosis, and the clinical findings of GR are very similar to LMDF, leading to the possiblity of misdiagnosis. ● Dermoscopy has been used recently for diagnosis of nonmelanocytic lesions. ● Vascular and nonvascular structures can be identified by dermoscopy, and could be helpful in the differentiation of LMDF from clinically similar skin diseases.
502
Clinical and Experimental Dermatology (2014) 39, pp500–502
References 1 Fox T. Disseminated follicular lupus (similating acne). Lancet 1878; 112: 75–6. 2 Borhan R, Vignon-Pennamen MD, Morel P. Lupus miliaris disseminatus faciei: 6 cases. Ann Dermatol Venereol 2005; 132: 526–30. 3 El Darouti M, Zaher H. Lupus miliaris disseminatus faciei – pathologic study of early, fully developed, and late lesions. Int J Dermatol 1993; 32: 508–11. 4 Darier J. Les tuberculides cutanees. Ann Dermatol Syphilol 1986; 7: 1431–6. 5 Hodak E, Trattner A, Feuerman H et al. Lupus miliaris disseminatus faciei—the DNA of Mycobacterium tuberculosis is not detectable in active lesions by polymerase chain reaction. Br J Dermatol 1997; 137: 614–19. 6 Skowron F, Causeret AS, Pabion C et al. F.I.GU.R.E.: facial idiopathic granulomas with regressive evolution. Is ‘lupus miliaris disseminatus faciei’ still an acceptable diagnosis in the third millennium? Dermatology 2000; 201: 287–9. 7 Khokhar O, Khachemoune A. A case of granulomatous rosacea: sorting granulomatous rosacea from other granulomatous diseases that affect the face. Dermatol Online J 2004; 10: 6. 8 Llambrich A, Zaballos P, Terasa F et al. Dermoscopy of cutaneous leishmaniasis. Br J Dermatol 2009; 60: 756–61. 9 Zalaudek I, Giacomel J, Argenziano G, et al. Dermoscopy of facial nonpigmented actinic keratosis. Br J Dermatol 2006; 155: 951–6. 10 Zalaudek I, Giacomel J, Schmid K et al. Dermatoscopy of facial actinic keratosis, intraepidermal carcinoma, and invasive squamous cell carcinoma: a progression model. J Am Acad Dermatol 2012; 66: 589–97. 11 Yucel A, Gunasti S, Denli Y, Uzun S. Cutaneous leishmaniasis: new dermoscopic findings. Int J Dermatol 2013; 52: 831–7. 12 Lallas A, Apalla Z, Lefaki I, Sotiriou E et al. Dermoscopy of discoid lupus erythematosus. Br J Dermatol 2013; 168: 284–8. 13 Lallas A, Argenziano G, Apalla Z et al. Dermoscopic patterns of common facial inflammatory skin diseases. J Eur Acad Dermatol Venereol 2013; doi:10.1111/jdv. 12146 [Epub ahead of print].
ª 2014 British Association of Dermatologists
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Dermoscopic evaluation of two patients with lupus miliaris disseminatus faciei E. Ayhan,1 U. Alabalik2 and Y. Avci2 1 Department of Dermatology, Zile State Hospital, Tokat, Turkey; and 2Department of Pathology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey
doi:10.1111/ced.12331
Summary
Lupus miliaris disseminatus faciei (LMDF) is a chronic and uncommon inflammatory dermatosis, characterized by yellowish-red papules, especially on the face and around the eyelids. It is considered to be a reaction to destroyed hair follicles. We dermoscopically evaluated two patients diagnosed with LMDF. Dermoscopy revealed follicular keratotic plugs and vascular structures. We discuss these cases and other diseases dermoscopically showing keratotic plugs.
Lupus miliaris disseminatus faciei (LMDF) is an uncommon, inflammatory, and granulomatous disease of unknown aetiology.1 Clinically, reddish-yellow or yellowish-brown papules are seen on the face, typically on the eyelids.2 Some researchers consider LMDF to be a variation of granulomatous rosacea, while others disagree. We dermoscopically evaluated two patients diagnosed with LMDF.
Report The two patients were both admitted to our clinic with papillary lesions on the face and neck. Patient 1 was a 38-year-old man (Fig. 1). On physical examination, he was found to have erythematous papules, some with ulceration at the centre, on his forehead, cheeks, eyelids and neck. Patient 2 was a 52-year-old woman (Fig. 2), who had numerous papules on her cheeks, some of which were skin-coloured and others erythematous. Skin biopsies were taken from the faces of both patients, and histological examination revealed a granulomatous reaction with caseification necrosis Correspondence: Dr Erhan Ayhan, Cumhuriyet Caddesi 22. Sokak No: 34 € Yozgat Yolu Uzeri /Zile/Tokat, Turkey E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 December 2013
500
Clinical and Experimental Dermatology (2014) 39, pp500–502
indicative of LMDF, along with follicular hyperkeratosis. Dermoscopy was performed, and revealed follicular plugs in the lesions, some resembling a target surrounding the centre, with hairpin-like and linear vessels on an orange-yellow and erythematous background. Patient 1 received tetracycline treatment, which produced no benefit. He was prescribed isotretinoin, but severe photocontact dermatitis appeared after this treatment. Patient 2 did not want any treatment. It is believed that LMDF is that the result of an autoimmune reaction resulting from the rupture of hair follicles, which causes granulomatous disease.3 Because LMDF has a granulomatous histopathological pattern, it has been considered a variation of lupus vulgaris or a form of tuberculid.4 However, this theory is no longer considered correct, because of factors such as the self-healing nature of the disease, lack of identification of a causative organism, and lack of response to anti-tuberculosis treatments.5 It has also been suggested that LMDF might be a form of sarcoidosis because of its clinical appearance; however the accompaniment of caseification necrosis and lack of other findings indicating sarcoidosis dissuaded Skowron et al. 6 from making this diagnosis. Another theory is that LMDF is a variation of granulomatous rosacea (GR). However, some features of GR, including its chronic nature, incidence of flushing,
ª 2014 British Association of Dermatologists
Dermoscopic evaluation of two patients with LMDF E. Ayhan et al.
(a)
(b)
(c)
(d)
Figure 1 (a) Patient 1 had a large num-
ber of erythematous papules on his face. (b) Linear and hairpin-like vessels, targetoid flame-like follicular plugs and ellipsoid follicular plugs were visible around the ulceration (original magnification 920). (c) Targetoid follicular plugs (white arrows), along with linear and hairpinlike vessels, surrounded the centre of a few of these papules (original magnification 910). (d) A schematic representation of the appearance of (c).
(a)
(b)
(c)
(d)
Figure 2 (a) Patient 2 had a few papules
on her face, with follicular plugs (white arrows). (b) Linear vessels (black arrows) and follicular plugs (white arrows). (c) Follicular plugs (thin white arrows). (d) hairpin vessels (thick white arrows) and linear vessel (black arrow). Original magnification (b–d) 910.
worsening with alcohol and spicy foods, incidence of Demodex parasites, inability to regress, and production of scarring indicate that LMDF is a different disease.7 Skowron et al. have stated that it should be renamed as a separate entity, proposing the acronym FIGURE (facial idiopathic granulomas with regressive evolution).6
ª 2014 British Association of Dermatologists
In the current study, dermoscopy showed keratotic follicular plugs and vascular structures in the papillary lesions. Keratotic plugs develop as a result of lateral pressure on hair follicles.8 Keratotic follicular plugs lead to a ‘strawberry pattern’ appearance on a background of nonpigmented actinic keratosis (AK). In this pattern, a noticeable pinkish-red pseudo-network develops
Clinical and Experimental Dermatology (2014) 39, pp500–502
501
Dermoscopic evaluation of two patients with LMDF E. Ayhan et al.
around the hair follicles intially, then oval, yellow-white keratotic plugs, mostly targetoid in appearance, start to appear.9 During the transformation of AK to intraepidermal carcinoma, the appearance is of a keratin mass, with small ulcerations in the middle, and surrounding oval, elliptic targetoid keratotic plugs, an appearance described by Zalaudek et al.10 as a red starburst pattern. Keratotic plugs have also been found in squamous cell carcinoma (SCC) and in keratoacanthoma.10 In addition, two independent studies on skin leishmaniasis have also found keratotic plugs, described as ‘teardrop-like’ structures.8,11 Although this feature was not described, the images in these studies show that these structures are targetoid in appearance. Similar keratotic plugs were seen in the lesions of patients with discoid lupus erythematosus.12 Keratotic plugs appear in the facial forms of DLE, AK, SCC, intraepidermal carcinoma and cutaneous leishmaniasis. Other skin diseases that should be considered in the differential diagnoses of LMDF, such as sarcoidosis13 and lupus vulgaris,13 reveal no follicular plugs. In conclusion, we report two patients with LMDF, which to our knowledge, are the first reported cases where dermoscopy has been used for evaluation. Dermoscopy may be of help in differentiating LMDF from other diseases that may be considered in the differential diagnosis.
Learning points ● LMDF is a granulomatous disease of unknown
aetiology. ● A similar condition, GR, is thought to be a var-
iant form of sarcoidosis and tuberculosis, and the clinical findings of GR are very similar to LMDF, leading to the possiblity of misdiagnosis. ● Dermoscopy has been used recently for diagnosis of nonmelanocytic lesions. ● Vascular and nonvascular structures can be identified by dermoscopy, and could be helpful in the differentiation of LMDF from clinically similar skin diseases.
502
Clinical and Experimental Dermatology (2014) 39, pp500–502
References 1 Fox T. Disseminated follicular lupus (similating acne). Lancet 1878; 112: 75–6. 2 Borhan R, Vignon-Pennamen MD, Morel P. Lupus miliaris disseminatus faciei: 6 cases. Ann Dermatol Venereol 2005; 132: 526–30. 3 El Darouti M, Zaher H. Lupus miliaris disseminatus faciei – pathologic study of early, fully developed, and late lesions. Int J Dermatol 1993; 32: 508–11. 4 Darier J. Les tuberculides cutanees. Ann Dermatol Syphilol 1986; 7: 1431–6. 5 Hodak E, Trattner A, Feuerman H et al. Lupus miliaris disseminatus faciei—the DNA of Mycobacterium tuberculosis is not detectable in active lesions by polymerase chain reaction. Br J Dermatol 1997; 137: 614–19. 6 Skowron F, Causeret AS, Pabion C et al. F.I.GU.R.E.: facial idiopathic granulomas with regressive evolution. Is ‘lupus miliaris disseminatus faciei’ still an acceptable diagnosis in the third millennium? Dermatology 2000; 201: 287–9. 7 Khokhar O, Khachemoune A. A case of granulomatous rosacea: sorting granulomatous rosacea from other granulomatous diseases that affect the face. Dermatol Online J 2004; 10: 6. 8 Llambrich A, Zaballos P, Terasa F et al. Dermoscopy of cutaneous leishmaniasis. Br J Dermatol 2009; 60: 756–61. 9 Zalaudek I, Giacomel J, Argenziano G, et al. Dermoscopy of facial nonpigmented actinic keratosis. Br J Dermatol 2006; 155: 951–6. 10 Zalaudek I, Giacomel J, Schmid K et al. Dermatoscopy of facial actinic keratosis, intraepidermal carcinoma, and invasive squamous cell carcinoma: a progression model. J Am Acad Dermatol 2012; 66: 589–97. 11 Yucel A, Gunasti S, Denli Y, Uzun S. Cutaneous leishmaniasis: new dermoscopic findings. Int J Dermatol 2013; 52: 831–7. 12 Lallas A, Apalla Z, Lefaki I, Sotiriou E et al. Dermoscopy of discoid lupus erythematosus. Br J Dermatol 2013; 168: 284–8. 13 Lallas A, Argenziano G, Apalla Z et al. Dermoscopic patterns of common facial inflammatory skin diseases. J Eur Acad Dermatol Venereol 2013; doi:10.1111/jdv. 12146 [Epub ahead of print].
ª 2014 British Association of Dermatologists
