Correspondence Clinical Letter
Clinical letter Segmental lichen aureus in a young woman with spontaneous improvement
DOI: 10.1111/ddg.12236
Dear Editors, Lichen aureus (LA), first described by Martin in 1958 and termed as “lichen purpuricus”, is a rare subtype of pigmented purpuric dermatoses, characterized by isolated golden to rust-brown patches or plaques [1–4]. It is always chronic and asymptomatic, and often affects young adults [1–4]. The lesions are frequently localized on the legs, but can also involve the arms, hands, trunk and even the eyelids [1–4]. We report a case with segmental distribution on the left hip and leg which improved spontaneously. A 30-year-old Chinese woman presented with a 4-month history of asymptomatic purpuric eruptions on her left leg, which had increased gradually in number and size; some lesions had already shown spontaneous regression. The patient
denied any other medical problems. There was no history of prior trauma at this site. No family history of similar disorders was noted. Cutaneous examination showed irregular, discrete, lichenoid erythematous-purpuric macules and plaques in a segmental distribution located on the left hip and the backside of the left leg from thigh to calf (Figure 1a, b, c). The lesional distribution did not follow a dermatome or the lines of Blaschko. Routine laboratory tests including complete blood counts, urine analysis were unremarkable. Histopathology of a macule revealed normal epidermis, band-like infiltrate of lymphocytes and extravasated erythrocytes with hemosiderin deposition in the papillary dermis (Figure 2a–c), and deep dermal perivascular, perineural and peri-appendageal lymphocytic infiltrates (Figure 2d, e). The lesions responded poorly to a methylprednisolone 8 mg t.i.d. for 2 weeks along with topical halcinonide ointment. The patient refused further treatment, but the lesions tended to slowly resolve over the next 13 months (Figure 1d, e) although a few new lesions occurred occasionally. LA has a predilection for young men, and less frequently for children [2–4]. The lesions always present as unilateral, discrete or confluent golden to rust-brown lichenoid macules and papules, which may persist unchangeably for many years
Figure 1 Golden-brown macules and patches on the left hip and the back aspect of left leg (a, b), close view (c). Most of the lesions resolved after 13 months (d, e).
260
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
Correspondence Clinical Letter
Figure 2 Histopathology showing band-like lymphocytic infiltrate (a) and extravasated erythrocytes (b) with hemosiderin deposition (c) in the papillary dermis, and deep perivascular/ perineural (d) and peri-appendageal (e) lymphocytic infiltrates in the dermis. (a, b, d, e: hematoxylin-eosin stain; c: Perls’ stain. a: the length of the white bar is 500 μm; b–e: original magnifications, x400)
[2–4]. The involved sites are most commonly the lower legs. Segmental patterns, as in our case, have been reported following the lines of Blaschko or the course of an underlying vein [2]. Although most of the cases are asymptomatic, some may be associated with intense itching and severe pain [2]. The pathogenesis remains unclear though several theories including venous insufficiency, trauma, infection and adverse effects of drugs have been proposed [1, 2, 4, 5] Histopathologically, LA presents an inflammatory infiltrate with a lichenoid pattern mainly located on the superficial dermis, and associated with extravasated erythrocytes. In rare instance, perivascular and perineural lymphocytic infiltrates in the dermis have also been described [3]. The lymphocytic infiltrates in our case were also peri-appendageal, a phenomenon that is rarely described. The differential diagnostic considerations include purpuric mycosis fungoides, lichen striatus, adult Blaschkitis, Schamberg disease, Majocchi disease and Gougerot-Blum syndrome. Purpuric mycosis fungoides is similar to pigmented purpuric dermatoses clinically [6], and LA may show histological features which may not be easily distinguished from mycosis fungoides and may show monoclonality, although the relationship between the two
entities is still controversial [2, 4, 5]. Patients with LA should have regular follow-up. Lichen striatus is a common dermatosis among children and is characterized by erythematous or red-brown papules with smooth or mild scaly surface in a linear distribution following lines of Blaschko, usually on the extremities. Adult Blaschkitis consists of relapsing pruritic papules and vesicular eruptions along the lines of Blaschko, generally on the chest. Its histopathology is more spongiotic than lichenoid, and the entity remains controversial [7]. Schamberg disease generally affects the lower legs in men and has a classical ‘cayenne pepper’ appearance. Majocchi disease presents with blue-red annular macules with darkred telangiectatic puncta and can occur at any site. Gougerot-Blum syndrome is characterized by lichenoid papules that coalesce into symmetrical plaques of various hues, and always affects the legs. Treatment of LA is challenging [1, 2, 4, 5]. Spontaneous remission is uncommon, even over long periods of time [1, 2, 4], and seems more likely in children than in adults [2, 4]. Fortunately, the present patient showed spontaneous regression, though her course was long. Potent topical and systemic steroids are generally ineffective, although 0.1%
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
261
Correspondence Clinical Letter
methylprednisolone aceponate ointment was helpful in 2 cases [2]. Topical pimecrolimus and tacrolimus, PUVA, narrowband UVB, as well as combination therapy with pentoxifylline and prostacyclin, have shown variable success [1, 4]. Most reports on therapy involve case reports; larger series of observations are needed to confirm the results. Conflicts of interest None.
Yu-Kun Zhao1, Di-Qing Luo1, Rashmi Sarkar2, Wen-Lin Xie3 (1) Department of Dermatology, Huangpu Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China (2) Department of Dermatology, Maulana Azad Medical college and LNJP Hospital, New Delhi, India (3) Department of Pathology, Huangpu Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
References 1 2
3
4 5
6
7
Murota H, Katayama I. Lichen aureus responding to topical tacrolimus treatment. J Dermatol 2011; 38: 823–5. Moche J, Glassman S, Modi D, Grayson W. Segmental lichen aureus: a report of two cases treated with methylprednisolone aceponate.Australas J Dermatol 2011; 52: e15–18. Aung PP, J Burns S, Bhawan J. Lichen aureus: an unusual histopathological presentation: a case report and a review of literature. 2013 May 7 [Epub ahead of print]. Kim MJ, Kim BY, Park KC, Youn SW. A case of childhood lichen aureus. Ann Dermatol 2009; 21: 393–5. Fink-Puches R, Wolf P, Kerl H, Cerroni L. Lichen aureus clinicopathologic features, natural history, and relationship to mycosis fungoides. Arch Dermatol 2008; 144: 1169–73. Hanna S, Walsh N, D’Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol 2006; 23: 350–4. Fogagnolo L, Barreto JA, Soares CT, Marinho FC, Nassif PW. Lichen striatus on adult. An Bras Dermatol. 2011; 86: 142–5.
Correspondence to Dr. Di-Qing Luo Department of Dermatology Huangpu Hospital of The First Affiliated Hospital Sun Yat-sen University 183 Huangpu Rd. E. Guangzhou 510700, China E-mail: [email protected]
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© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
Clinical letter Segmental lichen aureus in a young woman with spontaneous improvement
DOI: 10.1111/ddg.12236
Dear Editors, Lichen aureus (LA), first described by Martin in 1958 and termed as “lichen purpuricus”, is a rare subtype of pigmented purpuric dermatoses, characterized by isolated golden to rust-brown patches or plaques [1–4]. It is always chronic and asymptomatic, and often affects young adults [1–4]. The lesions are frequently localized on the legs, but can also involve the arms, hands, trunk and even the eyelids [1–4]. We report a case with segmental distribution on the left hip and leg which improved spontaneously. A 30-year-old Chinese woman presented with a 4-month history of asymptomatic purpuric eruptions on her left leg, which had increased gradually in number and size; some lesions had already shown spontaneous regression. The patient
denied any other medical problems. There was no history of prior trauma at this site. No family history of similar disorders was noted. Cutaneous examination showed irregular, discrete, lichenoid erythematous-purpuric macules and plaques in a segmental distribution located on the left hip and the backside of the left leg from thigh to calf (Figure 1a, b, c). The lesional distribution did not follow a dermatome or the lines of Blaschko. Routine laboratory tests including complete blood counts, urine analysis were unremarkable. Histopathology of a macule revealed normal epidermis, band-like infiltrate of lymphocytes and extravasated erythrocytes with hemosiderin deposition in the papillary dermis (Figure 2a–c), and deep dermal perivascular, perineural and peri-appendageal lymphocytic infiltrates (Figure 2d, e). The lesions responded poorly to a methylprednisolone 8 mg t.i.d. for 2 weeks along with topical halcinonide ointment. The patient refused further treatment, but the lesions tended to slowly resolve over the next 13 months (Figure 1d, e) although a few new lesions occurred occasionally. LA has a predilection for young men, and less frequently for children [2–4]. The lesions always present as unilateral, discrete or confluent golden to rust-brown lichenoid macules and papules, which may persist unchangeably for many years
Figure 1 Golden-brown macules and patches on the left hip and the back aspect of left leg (a, b), close view (c). Most of the lesions resolved after 13 months (d, e).
260
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
Correspondence Clinical Letter
Figure 2 Histopathology showing band-like lymphocytic infiltrate (a) and extravasated erythrocytes (b) with hemosiderin deposition (c) in the papillary dermis, and deep perivascular/ perineural (d) and peri-appendageal (e) lymphocytic infiltrates in the dermis. (a, b, d, e: hematoxylin-eosin stain; c: Perls’ stain. a: the length of the white bar is 500 μm; b–e: original magnifications, x400)
[2–4]. The involved sites are most commonly the lower legs. Segmental patterns, as in our case, have been reported following the lines of Blaschko or the course of an underlying vein [2]. Although most of the cases are asymptomatic, some may be associated with intense itching and severe pain [2]. The pathogenesis remains unclear though several theories including venous insufficiency, trauma, infection and adverse effects of drugs have been proposed [1, 2, 4, 5] Histopathologically, LA presents an inflammatory infiltrate with a lichenoid pattern mainly located on the superficial dermis, and associated with extravasated erythrocytes. In rare instance, perivascular and perineural lymphocytic infiltrates in the dermis have also been described [3]. The lymphocytic infiltrates in our case were also peri-appendageal, a phenomenon that is rarely described. The differential diagnostic considerations include purpuric mycosis fungoides, lichen striatus, adult Blaschkitis, Schamberg disease, Majocchi disease and Gougerot-Blum syndrome. Purpuric mycosis fungoides is similar to pigmented purpuric dermatoses clinically [6], and LA may show histological features which may not be easily distinguished from mycosis fungoides and may show monoclonality, although the relationship between the two
entities is still controversial [2, 4, 5]. Patients with LA should have regular follow-up. Lichen striatus is a common dermatosis among children and is characterized by erythematous or red-brown papules with smooth or mild scaly surface in a linear distribution following lines of Blaschko, usually on the extremities. Adult Blaschkitis consists of relapsing pruritic papules and vesicular eruptions along the lines of Blaschko, generally on the chest. Its histopathology is more spongiotic than lichenoid, and the entity remains controversial [7]. Schamberg disease generally affects the lower legs in men and has a classical ‘cayenne pepper’ appearance. Majocchi disease presents with blue-red annular macules with darkred telangiectatic puncta and can occur at any site. Gougerot-Blum syndrome is characterized by lichenoid papules that coalesce into symmetrical plaques of various hues, and always affects the legs. Treatment of LA is challenging [1, 2, 4, 5]. Spontaneous remission is uncommon, even over long periods of time [1, 2, 4], and seems more likely in children than in adults [2, 4]. Fortunately, the present patient showed spontaneous regression, though her course was long. Potent topical and systemic steroids are generally ineffective, although 0.1%
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
261
Correspondence Clinical Letter
methylprednisolone aceponate ointment was helpful in 2 cases [2]. Topical pimecrolimus and tacrolimus, PUVA, narrowband UVB, as well as combination therapy with pentoxifylline and prostacyclin, have shown variable success [1, 4]. Most reports on therapy involve case reports; larger series of observations are needed to confirm the results. Conflicts of interest None.
Yu-Kun Zhao1, Di-Qing Luo1, Rashmi Sarkar2, Wen-Lin Xie3 (1) Department of Dermatology, Huangpu Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China (2) Department of Dermatology, Maulana Azad Medical college and LNJP Hospital, New Delhi, India (3) Department of Pathology, Huangpu Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
References 1 2
3
4 5
6
7
Murota H, Katayama I. Lichen aureus responding to topical tacrolimus treatment. J Dermatol 2011; 38: 823–5. Moche J, Glassman S, Modi D, Grayson W. Segmental lichen aureus: a report of two cases treated with methylprednisolone aceponate.Australas J Dermatol 2011; 52: e15–18. Aung PP, J Burns S, Bhawan J. Lichen aureus: an unusual histopathological presentation: a case report and a review of literature. 2013 May 7 [Epub ahead of print]. Kim MJ, Kim BY, Park KC, Youn SW. A case of childhood lichen aureus. Ann Dermatol 2009; 21: 393–5. Fink-Puches R, Wolf P, Kerl H, Cerroni L. Lichen aureus clinicopathologic features, natural history, and relationship to mycosis fungoides. Arch Dermatol 2008; 144: 1169–73. Hanna S, Walsh N, D’Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol 2006; 23: 350–4. Fogagnolo L, Barreto JA, Soares CT, Marinho FC, Nassif PW. Lichen striatus on adult. An Bras Dermatol. 2011; 86: 142–5.
Correspondence to Dr. Di-Qing Luo Department of Dermatology Huangpu Hospital of The First Affiliated Hospital Sun Yat-sen University 183 Huangpu Rd. E. Guangzhou 510700, China E-mail: [email protected]
262
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1203
