Pediatric Dermatology Vol. 32 No. 1 e33–e35, 2015
Linear Cutaneous Lupus Erythematosus with Calcinosis Cutis and Milia Han Ma, M.D.,*,1 Mengsi Liao, M.D.,*,1 Shu Qiu, B.N.,† Rongbiao Lu, M.D.,* and Chun Lu, M.D.* Departments of *Dermatology and †Surgery, Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong, China
Abstract: Linear cutaneous lupus erythematosus (LCLE) is an unusual manifestation of cutaneous lupus erythematosus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. Calcinosis cutis and secondary milia are very uncommon in lupus erythematosus. We present a rare case of LCLE with calcinosis cutis and milia.
Linear cutaneous lupus erythematosus (LCLE) is a rare subtype of cutaneous lupus erythematosus (CLE) that presents as linear lesions following the lines of Blaschko. Lesions are more commonly observed on the face and occur less frequently on the limbs and trunk (1). We describe a rare case of LCLE with calcinosis cutis and milia. A 13-year-old boy presented with a linear, slightly pruritic, scaly, reddish-brown plaque on the left lower margin of the mandible, submandibular triangle, and anterior auricle for 7 years (Fig. 1A). Multiple small milia-like lesions were scattered on the plaque and sporadic atrophic fuchsia macules were observed on the mental region and marginal mandible (Fig. 1B). The patient reported no fever, chills, or night sweats. His past medical history was unremarkable and there was no similar condition reported in his family members. Physical examination revealed no sign of superficial lymph node enlargement in the bilateral
parts of the neck. An examination of the patient’s serum showed 2.33 mmol/L calcium (normal 2.20– 2.75 mmol/L), 1.47 mmol/L phosphorus (normal 1.20–1.90 mmol/L), 315 U/L alkaline phosphatase (normal 40–375 U/L), and 57 ng/L parathyroid hormone (normal 14–72 ng/L). Serum 25-hydroxy vitamin D levels also fell within the normal range. There were no abnormalities found during routine tests, including blood count, urinalysis, and liver and kidney function tests. Antinuclear and other autoantibodies, including anti-SS-A, Sm, U1-RNP, dsDNA, ssDNA, and cardiolipin, were all negative. Serum complement levels were normal. A skin biopsy specimen revealed hyperkeratosis, acanthosis, and multifocal liquefaction of basal cells (Fig. 1C insert). Severe banded lymphocyte infiltration in the superficial dermis and around the appendages, such as hair follicles and eccrine glands, was also observed (Fig. 1C). Multiple cysts with a squamous epithelial
Address correspondence to Chun Lu, M.D., Department of Dermatology, Third Affiliated Hospital, Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou, Guangdong 510630, China, or e-mail: [email protected]. 1 Han Ma and Mengsi Liao contributed equally to the article. DOI: 10.1111/pde.12496
© 2014 Wiley Periodicals, Inc.
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e34 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
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Figure 1. (A) Linear red-brown plaque on the left lower margin of the mandible, submandibular triangle and anterior auricle. (B) Multiple milia-like lesions and atrophic fuchsia macules. (C) A band of massive lymphocyte infiltration in the superficial dermis and around the appendages (hematoxylin and eosin, 409) and multifocal liquefaction of basal cells (insert; hematoxylin and eosin, 1009). (D) Multiple cysts (Von Kossa stain, 409). (E) Calcium deposits (Von Kossa stain, 4009). (F) Foreign body giant cells (Von Kossa stain, 4009).
cell layer and flakelike keratinous inclusions in the middermis (Fig. 1D) and calcium deposits (Fig. 1E) surrounded by foreign body giant cells (Fig. 1F) were found in one paraffin section treated with Von Kossa stain. Based on the clinical manifestations, histopathologic features, and observations made after Von Kossa stain, the patient was diagnosed with facial LCLE with calcinosis cutis and milia. He was treated with several topical corticosteroids intermittently for approximately 6 months. Tacrolimus 0.1% ointment was administered to mitigate concerns about the longterm safety of corticosteroids and to enhance treatment efficacy. Treatment using this ointment was found to be cost prohibitive, and it was administered for only 1 month, but during this short course of treatment, the lesion improved slightly and has remained stable for 8 months.
DISCUSSION LCLE is a highly unusual variation of discoid lupus erythematosus (DLE). It occurs mainly in children and young adults, with a similar incidence in both sexes, without ethnic preference. In most cases the age of onset is younger than 15 years. Lesions appear as linear unilateral erythematous plaques and are observed most frequently on the face, although the neck, trunk, and extremities may also be affected (2). Neither photosensitivity nor disease progression to systemic lupus erythematosus is observed (1). Antinuclear antibodies are usually negative or slightly positive, but direct immunofluorescence of autoantibodies is observed in most cases (2). These described histologic characteristics are compatible with a diagnosis of DLE.
Ma et al: LCLE with Calcinosis Cutis and Milia and Anti-SS-A
Lines of Blaschko, first described in 1901, are observed in many skin disorders, including lichen striatus, lichen planus, scleroderma, and psoriasis. In the case described in this article, lesions affected the left facial region of the patient and extended along the lines of Blaschko. Disorders that occur along the lines of Blaschko are believed to result from two different cell clones that evolve early in embryogenesis. Lyonization or random inactivations in X-linked disorders, postzygotic somatic mutations in somatic conditions, and gametic half-chromatid mutations have been proposed as mechanisms for the manifestation of the disorders that occur along the lines of Blaschko (3). The exact mechanism of the formation of calcifications has not been elucidated. High alkaline phosphatase concentrations in local tissues induced by chronic inflammation or injury and tissue necrosis may participate in the process of calcification. Weinberger et al (4) suggested that a small concentration of organic phosphate is sufficient to inhibit crystal formation and that alkaline phosphatase can remove such an inhibitor. This hypothesis supports the observation that soft tissue calcification is more commonly observed in progressive systemic sclerosis, dermatomyositis or polymyositis, and lupus erythematosus (LE). In the case presented in this article, high alkaline phosphatase concentrations in the skin or subcutaneous tissue, lichenoid progression to the basal membrane induced by the chronic inflammatory course of LE, or trauma as a result of scratching may cause dystrophic calcinosis and secondary milia. Topical corticosteroids are first-line treatment for CLE. Some findings support the greater efficacy of
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high-dose versus low-dose steroids (5). Topical calcineurin inhibitors have emerged in recent years as an alternative topical option for the treatment of various subtypes of CLE (6). CLE can also be treated topically using physical treatments, including laser therapy, cryotherapy, and dermabrasion. The efficacy of pulsed dye and argon lasers has been shown in several case reports and studies. Systemic therapies, including antimalarial drugs, systemic corticosteroids, immunosuppressants, rituximab, dapsone, and oral retinoids, can be used in cases in which there is widespread or scarring disease or in cases that remain refractory to topical treatments (6). REFERENCES 1. Aiyama A, Muro Y, Sugiura K et al. Extraordinarily long linear cutaneous lupus erythematosus along the lines of Blaschko. Dermatol Online J 2013;19:18960. 2. Alc antara-Gonz alez J, Fernandez-Guarino M, CarrilloGijon R et al. Linear cutaneous lupus erythematosus. Indian J Dermatol Venereol Leprol 2011;77:717–719. 3. Kar BR, Dash K. Co-existence of lichen sclerosus et atrophicus and morphoea along lines of Blaschko. Indian J Dermatol 2014;59:77–79. 4. Weinberger A, Kaplan JG, Myers AR. Extensive soft tissue calcification (calcinosis universalis) in systemic lupus erythematosus. Ann Rheum Dis 1979;38:384– 386. 5. Jessop S, Whitelaw DA, Delamere FM. Drugs for discoid lupus erythematosus. Cochrane Database Syst Rev 2009;7:CD002954. 6. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol 2013;27:391–404.
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
Linear Cutaneous Lupus Erythematosus with Calcinosis Cutis and Milia Han Ma, M.D.,*,1 Mengsi Liao, M.D.,*,1 Shu Qiu, B.N.,† Rongbiao Lu, M.D.,* and Chun Lu, M.D.* Departments of *Dermatology and †Surgery, Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong, China
Abstract: Linear cutaneous lupus erythematosus (LCLE) is an unusual manifestation of cutaneous lupus erythematosus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. Calcinosis cutis and secondary milia are very uncommon in lupus erythematosus. We present a rare case of LCLE with calcinosis cutis and milia.
Linear cutaneous lupus erythematosus (LCLE) is a rare subtype of cutaneous lupus erythematosus (CLE) that presents as linear lesions following the lines of Blaschko. Lesions are more commonly observed on the face and occur less frequently on the limbs and trunk (1). We describe a rare case of LCLE with calcinosis cutis and milia. A 13-year-old boy presented with a linear, slightly pruritic, scaly, reddish-brown plaque on the left lower margin of the mandible, submandibular triangle, and anterior auricle for 7 years (Fig. 1A). Multiple small milia-like lesions were scattered on the plaque and sporadic atrophic fuchsia macules were observed on the mental region and marginal mandible (Fig. 1B). The patient reported no fever, chills, or night sweats. His past medical history was unremarkable and there was no similar condition reported in his family members. Physical examination revealed no sign of superficial lymph node enlargement in the bilateral
parts of the neck. An examination of the patient’s serum showed 2.33 mmol/L calcium (normal 2.20– 2.75 mmol/L), 1.47 mmol/L phosphorus (normal 1.20–1.90 mmol/L), 315 U/L alkaline phosphatase (normal 40–375 U/L), and 57 ng/L parathyroid hormone (normal 14–72 ng/L). Serum 25-hydroxy vitamin D levels also fell within the normal range. There were no abnormalities found during routine tests, including blood count, urinalysis, and liver and kidney function tests. Antinuclear and other autoantibodies, including anti-SS-A, Sm, U1-RNP, dsDNA, ssDNA, and cardiolipin, were all negative. Serum complement levels were normal. A skin biopsy specimen revealed hyperkeratosis, acanthosis, and multifocal liquefaction of basal cells (Fig. 1C insert). Severe banded lymphocyte infiltration in the superficial dermis and around the appendages, such as hair follicles and eccrine glands, was also observed (Fig. 1C). Multiple cysts with a squamous epithelial
Address correspondence to Chun Lu, M.D., Department of Dermatology, Third Affiliated Hospital, Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou, Guangdong 510630, China, or e-mail: [email protected]. 1 Han Ma and Mengsi Liao contributed equally to the article. DOI: 10.1111/pde.12496
© 2014 Wiley Periodicals, Inc.
e33
e34 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
A
B
C
D
E
F
Figure 1. (A) Linear red-brown plaque on the left lower margin of the mandible, submandibular triangle and anterior auricle. (B) Multiple milia-like lesions and atrophic fuchsia macules. (C) A band of massive lymphocyte infiltration in the superficial dermis and around the appendages (hematoxylin and eosin, 409) and multifocal liquefaction of basal cells (insert; hematoxylin and eosin, 1009). (D) Multiple cysts (Von Kossa stain, 409). (E) Calcium deposits (Von Kossa stain, 4009). (F) Foreign body giant cells (Von Kossa stain, 4009).
cell layer and flakelike keratinous inclusions in the middermis (Fig. 1D) and calcium deposits (Fig. 1E) surrounded by foreign body giant cells (Fig. 1F) were found in one paraffin section treated with Von Kossa stain. Based on the clinical manifestations, histopathologic features, and observations made after Von Kossa stain, the patient was diagnosed with facial LCLE with calcinosis cutis and milia. He was treated with several topical corticosteroids intermittently for approximately 6 months. Tacrolimus 0.1% ointment was administered to mitigate concerns about the longterm safety of corticosteroids and to enhance treatment efficacy. Treatment using this ointment was found to be cost prohibitive, and it was administered for only 1 month, but during this short course of treatment, the lesion improved slightly and has remained stable for 8 months.
DISCUSSION LCLE is a highly unusual variation of discoid lupus erythematosus (DLE). It occurs mainly in children and young adults, with a similar incidence in both sexes, without ethnic preference. In most cases the age of onset is younger than 15 years. Lesions appear as linear unilateral erythematous plaques and are observed most frequently on the face, although the neck, trunk, and extremities may also be affected (2). Neither photosensitivity nor disease progression to systemic lupus erythematosus is observed (1). Antinuclear antibodies are usually negative or slightly positive, but direct immunofluorescence of autoantibodies is observed in most cases (2). These described histologic characteristics are compatible with a diagnosis of DLE.
Ma et al: LCLE with Calcinosis Cutis and Milia and Anti-SS-A
Lines of Blaschko, first described in 1901, are observed in many skin disorders, including lichen striatus, lichen planus, scleroderma, and psoriasis. In the case described in this article, lesions affected the left facial region of the patient and extended along the lines of Blaschko. Disorders that occur along the lines of Blaschko are believed to result from two different cell clones that evolve early in embryogenesis. Lyonization or random inactivations in X-linked disorders, postzygotic somatic mutations in somatic conditions, and gametic half-chromatid mutations have been proposed as mechanisms for the manifestation of the disorders that occur along the lines of Blaschko (3). The exact mechanism of the formation of calcifications has not been elucidated. High alkaline phosphatase concentrations in local tissues induced by chronic inflammation or injury and tissue necrosis may participate in the process of calcification. Weinberger et al (4) suggested that a small concentration of organic phosphate is sufficient to inhibit crystal formation and that alkaline phosphatase can remove such an inhibitor. This hypothesis supports the observation that soft tissue calcification is more commonly observed in progressive systemic sclerosis, dermatomyositis or polymyositis, and lupus erythematosus (LE). In the case presented in this article, high alkaline phosphatase concentrations in the skin or subcutaneous tissue, lichenoid progression to the basal membrane induced by the chronic inflammatory course of LE, or trauma as a result of scratching may cause dystrophic calcinosis and secondary milia. Topical corticosteroids are first-line treatment for CLE. Some findings support the greater efficacy of
e35
high-dose versus low-dose steroids (5). Topical calcineurin inhibitors have emerged in recent years as an alternative topical option for the treatment of various subtypes of CLE (6). CLE can also be treated topically using physical treatments, including laser therapy, cryotherapy, and dermabrasion. The efficacy of pulsed dye and argon lasers has been shown in several case reports and studies. Systemic therapies, including antimalarial drugs, systemic corticosteroids, immunosuppressants, rituximab, dapsone, and oral retinoids, can be used in cases in which there is widespread or scarring disease or in cases that remain refractory to topical treatments (6). REFERENCES 1. Aiyama A, Muro Y, Sugiura K et al. Extraordinarily long linear cutaneous lupus erythematosus along the lines of Blaschko. Dermatol Online J 2013;19:18960. 2. Alc antara-Gonz alez J, Fernandez-Guarino M, CarrilloGijon R et al. Linear cutaneous lupus erythematosus. Indian J Dermatol Venereol Leprol 2011;77:717–719. 3. Kar BR, Dash K. Co-existence of lichen sclerosus et atrophicus and morphoea along lines of Blaschko. Indian J Dermatol 2014;59:77–79. 4. Weinberger A, Kaplan JG, Myers AR. Extensive soft tissue calcification (calcinosis universalis) in systemic lupus erythematosus. Ann Rheum Dis 1979;38:384– 386. 5. Jessop S, Whitelaw DA, Delamere FM. Drugs for discoid lupus erythematosus. Cochrane Database Syst Rev 2009;7:CD002954. 6. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol 2013;27:391–404.
This document is a scanned copy of a printed document. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material.
