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Blaschkolinear Lupus Erythematosus: Strategies for Early Detection and Management
To the Editor:
Chronic cutaneous lupus erythematosus (CCLE) is an inflammatory condition with myriad cutaneous manifestations. Most forms of CCLE have the potential to progress to systemic lupus erythematosus (SLE).1
Blaschkolinear lupus erythematosus (BLE) is an exceedingly rare subtype of cutaneous lupus erythematosus that usually manifests during childhood as linear plaques along the lines of Blaschko.2,3 Under normal conditions, Blaschko lines are not noticeable; they correspond to the direction of ectodermal cell migration during cutaneous embryogenesis.4,5 The embryonic cells travel ventrolaterally, forming a V-shaped pattern on the back, an S-shaped pattern on the trunk, and an hourglass-shaped pattern on the face with several perpendicular intersections near the mouth and nose.6 During their migration, the cells are susceptible to somatic mutations and clonal expansion, resulting in a monoclonal population of genetically heterogenous cells. This phenomenon is known as somatic mosaicism and may lead to an increased susceptibility to an array of congenital and inflammatory dermatoses, such as cutaneous lupus erythematosus.4 Blaschkolinear entities tend to manifest in a unilateral distribution following exposure to a certain environmental trigger, such as trauma, viral illness, or UV radiation, although a trigger is not always present.7 We report a case of BLE manifesting on the head and neck in an adult patient.
A 46-year-old man presented with a pruritic rash of 3 months’ duration on the right cheek that extended inferiorly to the right upper chest. He had a medical history of well-controlled psoriasis, and he denied any antecedent trauma, fevers, chills, arthralgia, or night sweats. There had been no improvement with mometasone ointment 0.1% applied daily for 2 months as prescribed by his primary care provider. Physical examination revealed indurated, red-brown, atrophic plaques in a blaschkolinear distribution around the nose, right upper jaw, right side of the neck, and right upper chest (Figure, A).
Histopathology of punch biopsies from the right jaw and right upper chest showed an atrophic epidermis with scattered dyskeratotic keratinocytes and vacuolar alteration of the basal cell layer. A superficial and deep perivascular and periadnexal lymphocytic infiltrate was observed in both biopsies. Staining with Verhoeff-van Gieson elastin and periodic acid–Schiff highlighted prominent basement membrane thickening and loss of elastic fibers in the superficial dermis. These findings favored a diagnosis of CCLE, and the clinical blaschkolinear distribution of the rash led to our specific diagnosis of BLE. Laboratory workup for SLE including a complete blood cell count; urine analysis; and testing for liver and kidney function, antinuclearantibodies, complement levels, and erythrocyte sedimentation rate revealed no abnormalities.
The patient started hydroxychloroquine 200 mg twice daily and methotrexate 25 mg weekly along with strict photoprotection measures, including wearing photoprotective clothing and avoiding sunlight during the most intense hours of the day.
Linear lichen planus is an important differential diagnosis to consider in patients with a blaschkolinear eruption.7 Although the clinical manifestations of BLE and linear lichen planus are similar, they differ histopathologically. One study found that only 33.3% of patients (6/18) who clinically presented with blaschkolinear eruptions were correctly diagnosed before histologic examination.7 Visualization of the adnexa as well as the superficial and deep vascular plexuses is paramount in distinguishing between linear lichen planus and BLE; linear lichen planus does not have perivascular and periadnexal infiltration, while BLE does. Thus, in our experience, a punch biopsy—rather than a shave biopsy—should be performed to access the deeper layers of the skin.
Because these 2 entities have noteworthy differences in their management, prognosis, and long-term follow-up, accurate diagnosis is critical. To start, BLE is treated with the use of photoprotection, whereas linear lichen planus is commonly treated with phototherapy. Given the potential for forms of CCLE to progress to SLE, serial monitoring is indicated in patients with BLE. As the risk for progression to SLE is highest in the first 3 years after diagnosis, a review of systems and laboratory testing should occur every 2 to 3 months in the first year after diagnosis (sooner if the disease presentation is more severe).9 Also, treatment with hydroxychloroquine likely delays transformation to SLE and is important in the early management of BLE.10 On the other hand, linear lichen planus tends to self-resolve without progression to systemic involvement, warranting limited follow-up.9
Blaschkolinear lupus erythematosus typically manifests in childhood, but it also can be seen in adults, such as in our patient. Adult-onset BLE is rare but may be underrecognized or underreported in the literature.11 However, dermatologists should consider it in the differential diagnosis for any patient with a blaschkolinear eruption, as establishing the correct diagnosis is key to ensuring prompt and effective treatment for this rare inflammatory condition.
- Grönhagen CM, Fored CM, Granath F, et al. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol. 2011;164:1335-1341. doi:10.1111/j.1365-2133.2011.10272.x
- Requena C, Torrelo A, de Prada I, et al. Linear childhood cutaneous lupus erythematosus following Blaschko lines. J Eur Acad Dermatol Venereol. 2002;16:618-620. doi:10.1046/j.1468-3083.2002.00588.x
- Lim D, Hatami A, Kokta V, et al. Linear cutaneous lupus erythematosus in children-report of two cases and review of the literature: a case report. SAGE Open Med Case Rep. 2020;8:2050313x20979206. doi:10.1177/2050313X20979206
- Jin H, Zhang G, Zhou Y, et al. Old lines tell new tales: Blaschko linear lupus erythematosus. Autoimmun Rev. 2016;15:291-306. doi:10.1016/j.autrev.2015.11.014
- Yu S, Yu H-S. A patient with subacute cutaneous lupus erythematosus along Blaschko lines: implications for the role of keratinocytes in lupus erythematosus. Dermatologica Sinica. 2016;34:144-147. doi:10.1016/j.dsi.2015.12.002
- Kouzak SS, Mendes MST, Costa IMC. Cutaneous mosaicisms: concepts, patterns and classifications. An Bras Dermatol. 2013;88:507-517. doi:10.1590/abd1806-4841.20132015
- Liu W, Vano-Galvan S, Liu J-W, et al. Pigmented linear discoid lupus erythematosus following the lines of Blaschko: a retrospective study of a Chinese series. Indian J Dermatol Venereol Leprol. 2020;86:359-365. doi:10.4103/ijdvl.IJDVL_341_19
- O’Brien JC, Chong BF. Not just skin deep: systemic disease involvement in patients with cutaneous lupus. J Invest Dermatol Symp Proc. 2017;18:S69-S74. doi:10.1016/j.jisp.2016.09.001
- Curtiss P, Walker AM, Chong BF. A systematic review of the progression of cutaneous lupus to systemic lupus erythematosus. Front Immunol. 2022:13:866319. doi:10.3389/fimmu.2022.866319
- Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27:391-404. doi:10.1016/j.berh.2013.07.008
- Milosavljevic K, Fibeger E, Virata AR. A case of linear cutaneous lupus erythematosus in a 55-year-old woman. Am J Case Rep. 2020;21:E921495. doi:10.12659/AJCR.921495
To the Editor:
Chronic cutaneous lupus erythematosus (CCLE) is an inflammatory condition with myriad cutaneous manifestations. Most forms of CCLE have the potential to progress to systemic lupus erythematosus (SLE).1
Blaschkolinear lupus erythematosus (BLE) is an exceedingly rare subtype of cutaneous lupus erythematosus that usually manifests during childhood as linear plaques along the lines of Blaschko.2,3 Under normal conditions, Blaschko lines are not noticeable; they correspond to the direction of ectodermal cell migration during cutaneous embryogenesis.4,5 The embryonic cells travel ventrolaterally, forming a V-shaped pattern on the back, an S-shaped pattern on the trunk, and an hourglass-shaped pattern on the face with several perpendicular intersections near the mouth and nose.6 During their migration, the cells are susceptible to somatic mutations and clonal expansion, resulting in a monoclonal population of genetically heterogenous cells. This phenomenon is known as somatic mosaicism and may lead to an increased susceptibility to an array of congenital and inflammatory dermatoses, such as cutaneous lupus erythematosus.4 Blaschkolinear entities tend to manifest in a unilateral distribution following exposure to a certain environmental trigger, such as trauma, viral illness, or UV radiation, although a trigger is not always present.7 We report a case of BLE manifesting on the head and neck in an adult patient.
A 46-year-old man presented with a pruritic rash of 3 months’ duration on the right cheek that extended inferiorly to the right upper chest. He had a medical history of well-controlled psoriasis, and he denied any antecedent trauma, fevers, chills, arthralgia, or night sweats. There had been no improvement with mometasone ointment 0.1% applied daily for 2 months as prescribed by his primary care provider. Physical examination revealed indurated, red-brown, atrophic plaques in a blaschkolinear distribution around the nose, right upper jaw, right side of the neck, and right upper chest (Figure, A).
Histopathology of punch biopsies from the right jaw and right upper chest showed an atrophic epidermis with scattered dyskeratotic keratinocytes and vacuolar alteration of the basal cell layer. A superficial and deep perivascular and periadnexal lymphocytic infiltrate was observed in both biopsies. Staining with Verhoeff-van Gieson elastin and periodic acid–Schiff highlighted prominent basement membrane thickening and loss of elastic fibers in the superficial dermis. These findings favored a diagnosis of CCLE, and the clinical blaschkolinear distribution of the rash led to our specific diagnosis of BLE. Laboratory workup for SLE including a complete blood cell count; urine analysis; and testing for liver and kidney function, antinuclearantibodies, complement levels, and erythrocyte sedimentation rate revealed no abnormalities.
The patient started hydroxychloroquine 200 mg twice daily and methotrexate 25 mg weekly along with strict photoprotection measures, including wearing photoprotective clothing and avoiding sunlight during the most intense hours of the day.
Linear lichen planus is an important differential diagnosis to consider in patients with a blaschkolinear eruption.7 Although the clinical manifestations of BLE and linear lichen planus are similar, they differ histopathologically. One study found that only 33.3% of patients (6/18) who clinically presented with blaschkolinear eruptions were correctly diagnosed before histologic examination.7 Visualization of the adnexa as well as the superficial and deep vascular plexuses is paramount in distinguishing between linear lichen planus and BLE; linear lichen planus does not have perivascular and periadnexal infiltration, while BLE does. Thus, in our experience, a punch biopsy—rather than a shave biopsy—should be performed to access the deeper layers of the skin.
Because these 2 entities have noteworthy differences in their management, prognosis, and long-term follow-up, accurate diagnosis is critical. To start, BLE is treated with the use of photoprotection, whereas linear lichen planus is commonly treated with phototherapy. Given the potential for forms of CCLE to progress to SLE, serial monitoring is indicated in patients with BLE. As the risk for progression to SLE is highest in the first 3 years after diagnosis, a review of systems and laboratory testing should occur every 2 to 3 months in the first year after diagnosis (sooner if the disease presentation is more severe).9 Also, treatment with hydroxychloroquine likely delays transformation to SLE and is important in the early management of BLE.10 On the other hand, linear lichen planus tends to self-resolve without progression to systemic involvement, warranting limited follow-up.9
Blaschkolinear lupus erythematosus typically manifests in childhood, but it also can be seen in adults, such as in our patient. Adult-onset BLE is rare but may be underrecognized or underreported in the literature.11 However, dermatologists should consider it in the differential diagnosis for any patient with a blaschkolinear eruption, as establishing the correct diagnosis is key to ensuring prompt and effective treatment for this rare inflammatory condition.
To the Editor:
Chronic cutaneous lupus erythematosus (CCLE) is an inflammatory condition with myriad cutaneous manifestations. Most forms of CCLE have the potential to progress to systemic lupus erythematosus (SLE).1
Blaschkolinear lupus erythematosus (BLE) is an exceedingly rare subtype of cutaneous lupus erythematosus that usually manifests during childhood as linear plaques along the lines of Blaschko.2,3 Under normal conditions, Blaschko lines are not noticeable; they correspond to the direction of ectodermal cell migration during cutaneous embryogenesis.4,5 The embryonic cells travel ventrolaterally, forming a V-shaped pattern on the back, an S-shaped pattern on the trunk, and an hourglass-shaped pattern on the face with several perpendicular intersections near the mouth and nose.6 During their migration, the cells are susceptible to somatic mutations and clonal expansion, resulting in a monoclonal population of genetically heterogenous cells. This phenomenon is known as somatic mosaicism and may lead to an increased susceptibility to an array of congenital and inflammatory dermatoses, such as cutaneous lupus erythematosus.4 Blaschkolinear entities tend to manifest in a unilateral distribution following exposure to a certain environmental trigger, such as trauma, viral illness, or UV radiation, although a trigger is not always present.7 We report a case of BLE manifesting on the head and neck in an adult patient.
A 46-year-old man presented with a pruritic rash of 3 months’ duration on the right cheek that extended inferiorly to the right upper chest. He had a medical history of well-controlled psoriasis, and he denied any antecedent trauma, fevers, chills, arthralgia, or night sweats. There had been no improvement with mometasone ointment 0.1% applied daily for 2 months as prescribed by his primary care provider. Physical examination revealed indurated, red-brown, atrophic plaques in a blaschkolinear distribution around the nose, right upper jaw, right side of the neck, and right upper chest (Figure, A).
Histopathology of punch biopsies from the right jaw and right upper chest showed an atrophic epidermis with scattered dyskeratotic keratinocytes and vacuolar alteration of the basal cell layer. A superficial and deep perivascular and periadnexal lymphocytic infiltrate was observed in both biopsies. Staining with Verhoeff-van Gieson elastin and periodic acid–Schiff highlighted prominent basement membrane thickening and loss of elastic fibers in the superficial dermis. These findings favored a diagnosis of CCLE, and the clinical blaschkolinear distribution of the rash led to our specific diagnosis of BLE. Laboratory workup for SLE including a complete blood cell count; urine analysis; and testing for liver and kidney function, antinuclearantibodies, complement levels, and erythrocyte sedimentation rate revealed no abnormalities.
The patient started hydroxychloroquine 200 mg twice daily and methotrexate 25 mg weekly along with strict photoprotection measures, including wearing photoprotective clothing and avoiding sunlight during the most intense hours of the day.
Linear lichen planus is an important differential diagnosis to consider in patients with a blaschkolinear eruption.7 Although the clinical manifestations of BLE and linear lichen planus are similar, they differ histopathologically. One study found that only 33.3% of patients (6/18) who clinically presented with blaschkolinear eruptions were correctly diagnosed before histologic examination.7 Visualization of the adnexa as well as the superficial and deep vascular plexuses is paramount in distinguishing between linear lichen planus and BLE; linear lichen planus does not have perivascular and periadnexal infiltration, while BLE does. Thus, in our experience, a punch biopsy—rather than a shave biopsy—should be performed to access the deeper layers of the skin.
Because these 2 entities have noteworthy differences in their management, prognosis, and long-term follow-up, accurate diagnosis is critical. To start, BLE is treated with the use of photoprotection, whereas linear lichen planus is commonly treated with phototherapy. Given the potential for forms of CCLE to progress to SLE, serial monitoring is indicated in patients with BLE. As the risk for progression to SLE is highest in the first 3 years after diagnosis, a review of systems and laboratory testing should occur every 2 to 3 months in the first year after diagnosis (sooner if the disease presentation is more severe).9 Also, treatment with hydroxychloroquine likely delays transformation to SLE and is important in the early management of BLE.10 On the other hand, linear lichen planus tends to self-resolve without progression to systemic involvement, warranting limited follow-up.9
Blaschkolinear lupus erythematosus typically manifests in childhood, but it also can be seen in adults, such as in our patient. Adult-onset BLE is rare but may be underrecognized or underreported in the literature.11 However, dermatologists should consider it in the differential diagnosis for any patient with a blaschkolinear eruption, as establishing the correct diagnosis is key to ensuring prompt and effective treatment for this rare inflammatory condition.
- Grönhagen CM, Fored CM, Granath F, et al. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol. 2011;164:1335-1341. doi:10.1111/j.1365-2133.2011.10272.x
- Requena C, Torrelo A, de Prada I, et al. Linear childhood cutaneous lupus erythematosus following Blaschko lines. J Eur Acad Dermatol Venereol. 2002;16:618-620. doi:10.1046/j.1468-3083.2002.00588.x
- Lim D, Hatami A, Kokta V, et al. Linear cutaneous lupus erythematosus in children-report of two cases and review of the literature: a case report. SAGE Open Med Case Rep. 2020;8:2050313x20979206. doi:10.1177/2050313X20979206
- Jin H, Zhang G, Zhou Y, et al. Old lines tell new tales: Blaschko linear lupus erythematosus. Autoimmun Rev. 2016;15:291-306. doi:10.1016/j.autrev.2015.11.014
- Yu S, Yu H-S. A patient with subacute cutaneous lupus erythematosus along Blaschko lines: implications for the role of keratinocytes in lupus erythematosus. Dermatologica Sinica. 2016;34:144-147. doi:10.1016/j.dsi.2015.12.002
- Kouzak SS, Mendes MST, Costa IMC. Cutaneous mosaicisms: concepts, patterns and classifications. An Bras Dermatol. 2013;88:507-517. doi:10.1590/abd1806-4841.20132015
- Liu W, Vano-Galvan S, Liu J-W, et al. Pigmented linear discoid lupus erythematosus following the lines of Blaschko: a retrospective study of a Chinese series. Indian J Dermatol Venereol Leprol. 2020;86:359-365. doi:10.4103/ijdvl.IJDVL_341_19
- O’Brien JC, Chong BF. Not just skin deep: systemic disease involvement in patients with cutaneous lupus. J Invest Dermatol Symp Proc. 2017;18:S69-S74. doi:10.1016/j.jisp.2016.09.001
- Curtiss P, Walker AM, Chong BF. A systematic review of the progression of cutaneous lupus to systemic lupus erythematosus. Front Immunol. 2022:13:866319. doi:10.3389/fimmu.2022.866319
- Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27:391-404. doi:10.1016/j.berh.2013.07.008
- Milosavljevic K, Fibeger E, Virata AR. A case of linear cutaneous lupus erythematosus in a 55-year-old woman. Am J Case Rep. 2020;21:E921495. doi:10.12659/AJCR.921495
- Grönhagen CM, Fored CM, Granath F, et al. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol. 2011;164:1335-1341. doi:10.1111/j.1365-2133.2011.10272.x
- Requena C, Torrelo A, de Prada I, et al. Linear childhood cutaneous lupus erythematosus following Blaschko lines. J Eur Acad Dermatol Venereol. 2002;16:618-620. doi:10.1046/j.1468-3083.2002.00588.x
- Lim D, Hatami A, Kokta V, et al. Linear cutaneous lupus erythematosus in children-report of two cases and review of the literature: a case report. SAGE Open Med Case Rep. 2020;8:2050313x20979206. doi:10.1177/2050313X20979206
- Jin H, Zhang G, Zhou Y, et al. Old lines tell new tales: Blaschko linear lupus erythematosus. Autoimmun Rev. 2016;15:291-306. doi:10.1016/j.autrev.2015.11.014
- Yu S, Yu H-S. A patient with subacute cutaneous lupus erythematosus along Blaschko lines: implications for the role of keratinocytes in lupus erythematosus. Dermatologica Sinica. 2016;34:144-147. doi:10.1016/j.dsi.2015.12.002
- Kouzak SS, Mendes MST, Costa IMC. Cutaneous mosaicisms: concepts, patterns and classifications. An Bras Dermatol. 2013;88:507-517. doi:10.1590/abd1806-4841.20132015
- Liu W, Vano-Galvan S, Liu J-W, et al. Pigmented linear discoid lupus erythematosus following the lines of Blaschko: a retrospective study of a Chinese series. Indian J Dermatol Venereol Leprol. 2020;86:359-365. doi:10.4103/ijdvl.IJDVL_341_19
- O’Brien JC, Chong BF. Not just skin deep: systemic disease involvement in patients with cutaneous lupus. J Invest Dermatol Symp Proc. 2017;18:S69-S74. doi:10.1016/j.jisp.2016.09.001
- Curtiss P, Walker AM, Chong BF. A systematic review of the progression of cutaneous lupus to systemic lupus erythematosus. Front Immunol. 2022:13:866319. doi:10.3389/fimmu.2022.866319
- Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27:391-404. doi:10.1016/j.berh.2013.07.008
- Milosavljevic K, Fibeger E, Virata AR. A case of linear cutaneous lupus erythematosus in a 55-year-old woman. Am J Case Rep. 2020;21:E921495. doi:10.12659/AJCR.921495
Practice Points
- Blaschkolinear lupus erythematosus (BLE), an exceedingly rare subtype of chronic cutaneous lupus erythematosus, usually presents during childhood as linear plaques along the lines of Blaschko.
- It is important to consider linear lichen planus in patients with a blaschkolinear eruption, as the clinical manifestations are similar but there are differences in histopathology, management, prognosis, and long-term follow-up.
- Serial monitoring is indicated in patients with BLE given the potential for progression to systemic lupus erythematosus, which may be delayed with early use of hydroxychloroquine.
Verruciform Plaques Within a Tattoo of an HIV-Positive Patient
The Diagnosis: Lichenoid Reaction With Pseudoepitheliomatous Hyperplasia
A shave biopsy of the left ankle and a punch biopsy of the left medial calf were performed and sent for histologic examination and acid-fast stain. Bacterial, fungal, and mycobacterial tissue cultures also were sent for testing. The findings from direct examination were negative, and tissue cultures exhibited no growth. The shave and punch biopsies and histology revealed pseudoepitheliomatous hyperplasia (PEH) with keratinocyte necrosis, satellitosis, and areas of acute folliculitis (Figure 1). A lichenoid hypersensitivity mixed infiltrate that included histiocytes admixed with anthracotic and red-orange pigment, lymphocytes, plasma cells, neutrophils, and rare eosinophils was noted in the dermis. Given these clinical and histopathologic findings, the patient was diagnosed with red-pigment tattoo lichenoid reaction with PEH.
Tattoo-related inflammatory reactions can manifest clinically as allergic contact dermatitis, photodermatitis, infection, malignancy, foreign body granulomas, and delayed hypersensitivity reactions with myriad associated histopathologic patterns including spongiotic, psoriasiform, granulomatous, and lichenoid (as seen in our patient). Lichenoid tattoo reactions are the most common histopathologic variants of delayed hypersensitivity seen, mostly with cinnabar or red dye.1 However, there is a paucity of cases in the literature of PEH following tattooing with red dye. Interestingly, lichenoid tissue reaction accompanies PEH in all reported cases.2
Pseudoepitheliomatous hyperplasia can mimic squamous cell carcinoma and keratoacanthoma (KA) both clinically and histologically. All 3 conditions may exhibit epithelial hyperplasia with prominent dilated hyperplastic infundibula. In a case series of 11 presumed KAs within tattoos, Fraga and Prossick2 reported 82% (9/11) of the lesions were located strictly in areas with red pigment, and many were associated with a lichenoid tissue reaction. Kazlouskaya and Junkins-Hopkins3 previously described cases of KAs in tattoos that may represent PEH.
When treating lesions with this histologic appearance, consider the clinical and histologic overlap between KAs and PEH. Our patient was managed with clobetasol ointment 0.05% under occlusion followed by intralesional triamcinolone acetonide with notable improvement of the verrucous plaques on the left lateral malleolus (Figure 2). He also noted near resolution of the papules on the pretibial shin and complete resolution of all associated pruritus and burning. Calcineurin inhibitors, photochemotherapy, CO2 laser, excimer laser, and surgical removal with interval grafting also were considered.
It is important to recognize PEH in the differential of eruptions occurring within tattoos to avoid unnecessary invasive surgical procedures such as complete surgical excision of a KA to avoid malignant transformation.
- Mortimer NJ, Chave TA, Johnston GA. Red tattoo reactions. Clin Exp Dermatol. 2003;28:508-510.
- Fraga GR, Prossick TA. Tattoo-associated keratoacanthomas: a series of 8 patients with 11 keratoacanthomas. J Cutan Pathol. 2010;37:85-90.
- Kazlouskaya V, Junkins-Hopkins JM. Pseudoepitheliomatous hyperplasia in a red pigment tattoo: a separate entity or hypertrophic lichen planus-like reaction? J Clin Aesthet Dermatol. 2015;8:48-52.
The Diagnosis: Lichenoid Reaction With Pseudoepitheliomatous Hyperplasia
A shave biopsy of the left ankle and a punch biopsy of the left medial calf were performed and sent for histologic examination and acid-fast stain. Bacterial, fungal, and mycobacterial tissue cultures also were sent for testing. The findings from direct examination were negative, and tissue cultures exhibited no growth. The shave and punch biopsies and histology revealed pseudoepitheliomatous hyperplasia (PEH) with keratinocyte necrosis, satellitosis, and areas of acute folliculitis (Figure 1). A lichenoid hypersensitivity mixed infiltrate that included histiocytes admixed with anthracotic and red-orange pigment, lymphocytes, plasma cells, neutrophils, and rare eosinophils was noted in the dermis. Given these clinical and histopathologic findings, the patient was diagnosed with red-pigment tattoo lichenoid reaction with PEH.
Tattoo-related inflammatory reactions can manifest clinically as allergic contact dermatitis, photodermatitis, infection, malignancy, foreign body granulomas, and delayed hypersensitivity reactions with myriad associated histopathologic patterns including spongiotic, psoriasiform, granulomatous, and lichenoid (as seen in our patient). Lichenoid tattoo reactions are the most common histopathologic variants of delayed hypersensitivity seen, mostly with cinnabar or red dye.1 However, there is a paucity of cases in the literature of PEH following tattooing with red dye. Interestingly, lichenoid tissue reaction accompanies PEH in all reported cases.2
Pseudoepitheliomatous hyperplasia can mimic squamous cell carcinoma and keratoacanthoma (KA) both clinically and histologically. All 3 conditions may exhibit epithelial hyperplasia with prominent dilated hyperplastic infundibula. In a case series of 11 presumed KAs within tattoos, Fraga and Prossick2 reported 82% (9/11) of the lesions were located strictly in areas with red pigment, and many were associated with a lichenoid tissue reaction. Kazlouskaya and Junkins-Hopkins3 previously described cases of KAs in tattoos that may represent PEH.
When treating lesions with this histologic appearance, consider the clinical and histologic overlap between KAs and PEH. Our patient was managed with clobetasol ointment 0.05% under occlusion followed by intralesional triamcinolone acetonide with notable improvement of the verrucous plaques on the left lateral malleolus (Figure 2). He also noted near resolution of the papules on the pretibial shin and complete resolution of all associated pruritus and burning. Calcineurin inhibitors, photochemotherapy, CO2 laser, excimer laser, and surgical removal with interval grafting also were considered.
It is important to recognize PEH in the differential of eruptions occurring within tattoos to avoid unnecessary invasive surgical procedures such as complete surgical excision of a KA to avoid malignant transformation.
The Diagnosis: Lichenoid Reaction With Pseudoepitheliomatous Hyperplasia
A shave biopsy of the left ankle and a punch biopsy of the left medial calf were performed and sent for histologic examination and acid-fast stain. Bacterial, fungal, and mycobacterial tissue cultures also were sent for testing. The findings from direct examination were negative, and tissue cultures exhibited no growth. The shave and punch biopsies and histology revealed pseudoepitheliomatous hyperplasia (PEH) with keratinocyte necrosis, satellitosis, and areas of acute folliculitis (Figure 1). A lichenoid hypersensitivity mixed infiltrate that included histiocytes admixed with anthracotic and red-orange pigment, lymphocytes, plasma cells, neutrophils, and rare eosinophils was noted in the dermis. Given these clinical and histopathologic findings, the patient was diagnosed with red-pigment tattoo lichenoid reaction with PEH.
Tattoo-related inflammatory reactions can manifest clinically as allergic contact dermatitis, photodermatitis, infection, malignancy, foreign body granulomas, and delayed hypersensitivity reactions with myriad associated histopathologic patterns including spongiotic, psoriasiform, granulomatous, and lichenoid (as seen in our patient). Lichenoid tattoo reactions are the most common histopathologic variants of delayed hypersensitivity seen, mostly with cinnabar or red dye.1 However, there is a paucity of cases in the literature of PEH following tattooing with red dye. Interestingly, lichenoid tissue reaction accompanies PEH in all reported cases.2
Pseudoepitheliomatous hyperplasia can mimic squamous cell carcinoma and keratoacanthoma (KA) both clinically and histologically. All 3 conditions may exhibit epithelial hyperplasia with prominent dilated hyperplastic infundibula. In a case series of 11 presumed KAs within tattoos, Fraga and Prossick2 reported 82% (9/11) of the lesions were located strictly in areas with red pigment, and many were associated with a lichenoid tissue reaction. Kazlouskaya and Junkins-Hopkins3 previously described cases of KAs in tattoos that may represent PEH.
When treating lesions with this histologic appearance, consider the clinical and histologic overlap between KAs and PEH. Our patient was managed with clobetasol ointment 0.05% under occlusion followed by intralesional triamcinolone acetonide with notable improvement of the verrucous plaques on the left lateral malleolus (Figure 2). He also noted near resolution of the papules on the pretibial shin and complete resolution of all associated pruritus and burning. Calcineurin inhibitors, photochemotherapy, CO2 laser, excimer laser, and surgical removal with interval grafting also were considered.
It is important to recognize PEH in the differential of eruptions occurring within tattoos to avoid unnecessary invasive surgical procedures such as complete surgical excision of a KA to avoid malignant transformation.
- Mortimer NJ, Chave TA, Johnston GA. Red tattoo reactions. Clin Exp Dermatol. 2003;28:508-510.
- Fraga GR, Prossick TA. Tattoo-associated keratoacanthomas: a series of 8 patients with 11 keratoacanthomas. J Cutan Pathol. 2010;37:85-90.
- Kazlouskaya V, Junkins-Hopkins JM. Pseudoepitheliomatous hyperplasia in a red pigment tattoo: a separate entity or hypertrophic lichen planus-like reaction? J Clin Aesthet Dermatol. 2015;8:48-52.
- Mortimer NJ, Chave TA, Johnston GA. Red tattoo reactions. Clin Exp Dermatol. 2003;28:508-510.
- Fraga GR, Prossick TA. Tattoo-associated keratoacanthomas: a series of 8 patients with 11 keratoacanthomas. J Cutan Pathol. 2010;37:85-90.
- Kazlouskaya V, Junkins-Hopkins JM. Pseudoepitheliomatous hyperplasia in a red pigment tattoo: a separate entity or hypertrophic lichen planus-like reaction? J Clin Aesthet Dermatol. 2015;8:48-52.
A 40-year-old man with a medical history of human immunodeficiency virus infection managed with highly active antiretroviral therapy (CD4 count, 888 cells/mm3 and an undetectable viral load), psoriasis, and recurrent condyloma acuminatum presented with exophytic, annular, hyperkeratotic, verrucous plaques on the left lateral malleolus with multiple erythematous hyperkeratotic papules on the pretibial shin of the left leg of 6 months' duration. These plaques and papules were localized to areas where red dye was used in a tattoo the patient had received 2 years prior to presentation. There was no associated fluctuance or drainage. The patient reported paroxysmal pruritus and burning pain.
