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Erythema dyschromicum perstans and lichen planus: Are they related?
Erythema dyschromicum perstans and lichen planus: Are they related? Robert S, Berger, MD,* Thomas J. Hayes, MD, and Steven L. Dixon, M D Lackland Air Force Base, Texas A 53-year-old woman initially had lichen planus primarily on her extremities. Approximately 1 year later, lesions consistent with erythema dyschromicum perstans were observed. Both diseases cleared with griseofulvin therapy but returned after discontinuation of the drug. Retreatment with griseofulvin again resulted in clearing. (J Ara ACAD DEaMATOL 1989;21:438-42.)
Lichen planus is a well-described entity of unknown cause and various presentations. ~ Some authors include erythema dyschromicum perstans as a variant of lichen planus because of the many clinical and histopathologic similarities between the diseases, z3 We describe a patient with erythema dyschromicum perstans preceded by lichen planus and present arguments to support that erythema dyschromicum perstans and lichen planus are variants of the same disease. CASE REPORT
A 53-year-old black woman came to Wilford Hall USAF Medical Center with lichen planus on the neck and arms. Despite topical fluorinated steroid therapy, the eruptions gradually spread to involve the extremities and trunk. The oral mucosa also was involved, but the scalp and naris were not. Examination of a biopsy specimen of a violaceous papule revealed the typical findings of lichen planus (Fig. 1). Prednisone therapy was started (initial dose 60 mg, tapered to 20 mg during 3 weeks); lesions resolved, leaving residual hyperpigmentafion. After several months, the lesions recurred, primarily on the extremities and later becoming widespread. In addition to the lichen planus, there were large annular From Wilford Hall USAF Medical Center. The opinions expressed herein are those of the authors and do not purport to express the views of the Department of the Air Force or any other Department or Agency of the United States Government, Reprint requests: Robert S, Berger, MAJ, MC, USAF, Department of Dermatology, 509th Strategic Hospital, Pease AFB, NH 03801. *Presently stationed at 509th Strategic Hospital, Pease AFB.
438
plaques with an erythematous raised border and central "ashy" bluish gray discoloration on the upper aspect of the back (Fig. 2). A diagnosis of erythema dyschromicure perstans was considered. Examination of a biopsy specimen from the active edge (Fig. 3) demonstrated compact orthokeratosis, focal hypergranulosis, attenuation of the rete ridge pattern, extensive vacuolar alteration, and a superficial perivascular and interstitial infiltrate composed of lymphocytes, histiocytes, and melanophages. Examination of the specimen from the center (Fig. 4) demonstrated an atrophic, flattened epidermis; mild focal vacuolar alteration; and a sparse infiltrate composed of scattered lymphocytes and numerous melanophages in the papillary dermis. These findings were consistent with erythema dyschromicum perstans. Griseofulvin therapy (250 mg twice a day) was begun, with resolution of the active borders of the erythema dyschromicum perstans lesions and the lichen planus after 1 month. The dosage was reduced to 125 mg twice a day. After 3 more months of therapy, the lichen planus had resolved completely, with some residual hyperpigmentation. The erythema dyschromicum perstans lesions remained hyperpigmented with no active borders. Because of the excellent response, the patient discontinued the medication, but within 3 weeks Iesions recurred on her neck and anterior aspect of her chest. Griseofulvin therapy (250 mg twice a day) was resumed, with resolution. Topical fluorinated steroids also were used intermittently. DISCUSSION The most consistent feature of erythema dyschromicum perstans is a bluish gray pigmentation. The size and distribution of lesions vary and can involve a large portion of the skin. The lesions can
Volume 21 Number 2, Part 2 August 1989
Erythema dyschromicum perstans and lichen planus 439
Fig. 1. Photomicrograph of biopsy specimen of violaceous papule shows compact orthokeratosis, wedge-shaped hypergranulosis, "sawtooth" rete ridge pattern, vacuolar alteration of dermoepidermal interface, and lichenoid lymphohistiocytic infiltrate.
Fig. 2. Annular plaque with erythematous raised border and central ashy, bluish gray discoloration on upper aspect of back.
have an indistinct, sharply demarcated, or erythematous border, which is sometimes raised. 36 They are usually asymptomatic, although some can be mildly pruritic. 6,7 The palms, soles, scalp, nails, and mucous membranes are not involved.3,5'7~9This clinical presentation can be similar to that with the atrophic or actinic variants of lichen planus. Some authors prefer lichen planus pigmentosus3 as the descriptive term for erythema dyschromicum perstans.
Findings on light microscopic, immunofluorescence, and electron microscopic examinations of lesions of erythema dyschromicum perstans are consistent with a lichenoid reaction pattern. 4"5'8'9 Light microscopic findings of erythema dyschromicum perstans can be divided into two phases: early and late. In the early phase there is a lichenoid infiltrate of varying degree, composed of mononuclear ceils and vacuolar alteration of the basal cells? ~ As the lesion matures (late phase), the
440
Berger et al.
Journal of the American Academy of Dermatology
Fig. 3. Photomicrograph of biopsy specimen of active edge shows compact orthokeratosis, focal hypergranulosis, attenuation of rete ridge pattern, extensive vacuolar attenuation, and superficial perivascular and interstitial infiltrate composed of lymphocytes, histiocytes, and melanophages.
Fig. 4. Photomicrograph of biopsy specimen from center of lesion shows epidermal atrophy with loss of rete ridge pattern, mild focal vacuolar alteration, and sparse infiltrate composed of scattered lymphocytes and numerous melanophages in papillary dermis. infiltrate diminishes, leaving only vacuolar alteration of the basal cells.~~ 11 In time the vacuolar changes also disappear. It There is a considerable amount of pigment incontinence, which results in the discoloration and may be the only microscopic finding in old lesions. There is a tendency toward a loss of the fete ridge pattern as the lesion matures:. ~0 Civatte bodies have been detected in
some lesions, 5 as have spongiosis and spongiotic microvesicle formation, papillary dermal, edema, and exocytosis (in active lesions): ,7.12 There are many similarities in the microscopic findings of erythema dyschromicum perstans and lichen planus. The superficial mononuelear lichenoid infiltrate with vacuolar alteration is the main feature of both) ~ ~3 Pigment incontinence and the
Volume 21 Number 2, Part 2 August 1989
Erythema dyschromicum perstans and lichen planus 441
resulting melanophages are commonly seen with both, as are Civatte bodies. Some of the lichen planus variants, such as lichen planus actinicus and lichen planus atrophicus, have an even stronger histologic resemblance to erythema dyschromicum perstans. Direct immunofluorescence studies of the active border of erythema dyschromicum perstans have demonstrated IgM, IgG, and C4 staining of Civatte bodies. S,7 Civatte bodies also stain with IgM and C4 in lichen planus), 14 Fibrinogen has been localized at the dermoepidermal junction in active lesions of erythema dyschromicum perstans, 5 whereas the inactive (ashy-colored) areas only demonstrated a weak fibrinogen pattern? Fibrinogen is deposited at the basement membrane zone of lichen planus as well. s, 14 Electron microscopic findings of erythema dyschromicum perstans and lichen planus demonstrate Civatte bodies, interruption of the basement membrane, and widening of intracellular spaces in the basal cell layer. 5,9.zs Although the basal lamina is interrupted in both, it is reportedly to be reduplicated only in lichen planus. 9, t5 The desmosomes and hemidesmosomes are broken down and their number reduced in lichen planus, 15whereas in erythema dyschromicum perstans the desmosomes appear normal but retreat to one cell after cell separation? There are intranuclear bodies in lichen planus ~S and perinuclear vacuoles in erythema dyschromicum perstans. 9 Although there have been numerous reports of lichen planus and erythema dyschromicum perstans, there have been few reports of the two diseases together. Naidorf and Cohen 2 reported a case of erythema dyschromicum perstans preceding lichen planus, whereas Bhutani et al. 3 described 11 of 40 patients with erythema dyschromicum perstans with coincidental lichen planus. In the latter report, lichen planus preceded, coincided with, or followed the onset of erythema dyschromicum perstans. Classic bullous and maculopapular variants of lichen planus were described. Pinkus I~ described a patient in whom ashy patches developed after erythematous scaly papules. (He does not comment as to whether this is lichen planus.) In other reports lichen planus actinicus (or subtropicum), and lichen pigmentosus, have been mentioned as variants of lichen planus that closely
resemble erythema dyschromicum perstans, z 3.5,Jo Ramirez, 4 in reviewing 139 cases of erythema dyschromicum perstans, does not mention an associated lichen planus. Although there is a great degree of similarity between erythema dyschromicum perstans and lichen planus, there are also significant clinical and ultrastructural differences. Why is erythema dyschromicum perstans described primarily in patients from South America and India rather than from Europe and the United States? Is erythema dyschromicum perstans a variant of lichen planus that occurs primarily in darker skin? There is a strong argument for considering erythema dyschromicum perstans as a variant of lichen planus. In our case, lichen planus and erythema dyschromicum perstans may have developed coincidentatly. However, it seems more reasonable to consider both types of lesions as variants of lichen planus. The term lichen planus pigmentosus, as offered by Bhutani et al., 3 seems more reasonable than the descriptive term erythema dyschromicum perstans until such time as the two can be separated unequivocally. In summary, we present a case of erythema dyschromicum perstans and lichen planus in the same patient, as well as clinical and ultrastructural similarities of both entities. We believe our patient manifested a spectrum of lichen planus, initially presenting with typical lichen planus and then developing classic lesions of erythema dyschromicum perstans. We prefer the term lichen planus pigmentosus, rather than erythema dyschromicum
perstans. REFERENCES 1. Arndt KA. Lichen planus. In: Fitzpatrick TB, Eiscn AZ, Wolff K, Freedberg [M, Austen KF, eds. Dermatology in general medicine. 3rd ed. New York: McGraw-Hill, 1987:967-73. 2. Naidorf KF, Cohen SR. Erythema dyscbromicurn perstans and lichen planus. Arch Dermatol 1982;118: 683-5. 3. Bhutani LK, Bedi TR, Pandhi RK, Nayak NC. Lichen planus pigmentosus. Dermatologica 1974;149:43-50. 4. Ramirez CO. The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis 1967;3:244-7. 5. Tschen JA, Tschen EA, McGavran MH. Erythema dyschromicum perstans. J AM AcAr~ DEr~MA'rot. 1980;2:295-302. 6. Convit J, Kerdel-Vegas F, Rodriguez G. Erythema dys-
442
7. 8. 9. 10.
Berger et al. chromicum perstans: a hitherto undescribed skin disease. J Invest Dermatol 1967;36:457-62. Knox JM, Dodge BG, Freeman RG. Erytherna dyschromicum perstans. Arch Dermatol 1968;97:262-72. Kark EC, Litt JZ. Ashy dermatosis: a variant of lichen planus? Cuffs 1980;25:631-3. Sorer NA, Wand C, Freeman RG. Ultrastructural pathology of erythema dyschromicum perstans. J Invest Dermatol 1969;52:155-62. Pinkus H. Lichenoid tissue reactions. Arch Dermatol 1973;107:840-6.
Journal of the American Academy of Dermatology 11. Ackerman AB, eel. Histologic diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger, 1978:197-8. 12. Lever WF, Schaumburg-Lever G, eds. Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983:138. 13. Stevenson JR, Miura M. Erythema dyschromicam perstans (ashy dermatosis). Arch Dermatol 1966;94:196-9. 14. Baart de la Faille-Kuyper EH, Baart de la Faille H, An immunofluorescence study of lichen planus. Br J Dermatol 1974;90:365-71. 15. Johnson FR, Fry L. Ultrastructural observations on lichen planus. Arch Dermatol 1967;95:596-607.
Lichen planus is a well-described entity of unknown cause and various presentations. ~ Some authors include erythema dyschromicum perstans as a variant of lichen planus because of the many clinical and histopathologic similarities between the diseases, z3 We describe a patient with erythema dyschromicum perstans preceded by lichen planus and present arguments to support that erythema dyschromicum perstans and lichen planus are variants of the same disease. CASE REPORT
A 53-year-old black woman came to Wilford Hall USAF Medical Center with lichen planus on the neck and arms. Despite topical fluorinated steroid therapy, the eruptions gradually spread to involve the extremities and trunk. The oral mucosa also was involved, but the scalp and naris were not. Examination of a biopsy specimen of a violaceous papule revealed the typical findings of lichen planus (Fig. 1). Prednisone therapy was started (initial dose 60 mg, tapered to 20 mg during 3 weeks); lesions resolved, leaving residual hyperpigmentafion. After several months, the lesions recurred, primarily on the extremities and later becoming widespread. In addition to the lichen planus, there were large annular From Wilford Hall USAF Medical Center. The opinions expressed herein are those of the authors and do not purport to express the views of the Department of the Air Force or any other Department or Agency of the United States Government, Reprint requests: Robert S, Berger, MAJ, MC, USAF, Department of Dermatology, 509th Strategic Hospital, Pease AFB, NH 03801. *Presently stationed at 509th Strategic Hospital, Pease AFB.
438
plaques with an erythematous raised border and central "ashy" bluish gray discoloration on the upper aspect of the back (Fig. 2). A diagnosis of erythema dyschromicure perstans was considered. Examination of a biopsy specimen from the active edge (Fig. 3) demonstrated compact orthokeratosis, focal hypergranulosis, attenuation of the rete ridge pattern, extensive vacuolar alteration, and a superficial perivascular and interstitial infiltrate composed of lymphocytes, histiocytes, and melanophages. Examination of the specimen from the center (Fig. 4) demonstrated an atrophic, flattened epidermis; mild focal vacuolar alteration; and a sparse infiltrate composed of scattered lymphocytes and numerous melanophages in the papillary dermis. These findings were consistent with erythema dyschromicum perstans. Griseofulvin therapy (250 mg twice a day) was begun, with resolution of the active borders of the erythema dyschromicum perstans lesions and the lichen planus after 1 month. The dosage was reduced to 125 mg twice a day. After 3 more months of therapy, the lichen planus had resolved completely, with some residual hyperpigmentation. The erythema dyschromicum perstans lesions remained hyperpigmented with no active borders. Because of the excellent response, the patient discontinued the medication, but within 3 weeks Iesions recurred on her neck and anterior aspect of her chest. Griseofulvin therapy (250 mg twice a day) was resumed, with resolution. Topical fluorinated steroids also were used intermittently. DISCUSSION The most consistent feature of erythema dyschromicum perstans is a bluish gray pigmentation. The size and distribution of lesions vary and can involve a large portion of the skin. The lesions can
Volume 21 Number 2, Part 2 August 1989
Erythema dyschromicum perstans and lichen planus 439
Fig. 1. Photomicrograph of biopsy specimen of violaceous papule shows compact orthokeratosis, wedge-shaped hypergranulosis, "sawtooth" rete ridge pattern, vacuolar alteration of dermoepidermal interface, and lichenoid lymphohistiocytic infiltrate.
Fig. 2. Annular plaque with erythematous raised border and central ashy, bluish gray discoloration on upper aspect of back.
have an indistinct, sharply demarcated, or erythematous border, which is sometimes raised. 36 They are usually asymptomatic, although some can be mildly pruritic. 6,7 The palms, soles, scalp, nails, and mucous membranes are not involved.3,5'7~9This clinical presentation can be similar to that with the atrophic or actinic variants of lichen planus. Some authors prefer lichen planus pigmentosus3 as the descriptive term for erythema dyschromicum perstans.
Findings on light microscopic, immunofluorescence, and electron microscopic examinations of lesions of erythema dyschromicum perstans are consistent with a lichenoid reaction pattern. 4"5'8'9 Light microscopic findings of erythema dyschromicum perstans can be divided into two phases: early and late. In the early phase there is a lichenoid infiltrate of varying degree, composed of mononuclear ceils and vacuolar alteration of the basal cells? ~ As the lesion matures (late phase), the
440
Berger et al.
Journal of the American Academy of Dermatology
Fig. 3. Photomicrograph of biopsy specimen of active edge shows compact orthokeratosis, focal hypergranulosis, attenuation of rete ridge pattern, extensive vacuolar attenuation, and superficial perivascular and interstitial infiltrate composed of lymphocytes, histiocytes, and melanophages.
Fig. 4. Photomicrograph of biopsy specimen from center of lesion shows epidermal atrophy with loss of rete ridge pattern, mild focal vacuolar alteration, and sparse infiltrate composed of scattered lymphocytes and numerous melanophages in papillary dermis. infiltrate diminishes, leaving only vacuolar alteration of the basal cells.~~ 11 In time the vacuolar changes also disappear. It There is a considerable amount of pigment incontinence, which results in the discoloration and may be the only microscopic finding in old lesions. There is a tendency toward a loss of the fete ridge pattern as the lesion matures:. ~0 Civatte bodies have been detected in
some lesions, 5 as have spongiosis and spongiotic microvesicle formation, papillary dermal, edema, and exocytosis (in active lesions): ,7.12 There are many similarities in the microscopic findings of erythema dyschromicum perstans and lichen planus. The superficial mononuelear lichenoid infiltrate with vacuolar alteration is the main feature of both) ~ ~3 Pigment incontinence and the
Volume 21 Number 2, Part 2 August 1989
Erythema dyschromicum perstans and lichen planus 441
resulting melanophages are commonly seen with both, as are Civatte bodies. Some of the lichen planus variants, such as lichen planus actinicus and lichen planus atrophicus, have an even stronger histologic resemblance to erythema dyschromicum perstans. Direct immunofluorescence studies of the active border of erythema dyschromicum perstans have demonstrated IgM, IgG, and C4 staining of Civatte bodies. S,7 Civatte bodies also stain with IgM and C4 in lichen planus), 14 Fibrinogen has been localized at the dermoepidermal junction in active lesions of erythema dyschromicum perstans, 5 whereas the inactive (ashy-colored) areas only demonstrated a weak fibrinogen pattern? Fibrinogen is deposited at the basement membrane zone of lichen planus as well. s, 14 Electron microscopic findings of erythema dyschromicum perstans and lichen planus demonstrate Civatte bodies, interruption of the basement membrane, and widening of intracellular spaces in the basal cell layer. 5,9.zs Although the basal lamina is interrupted in both, it is reportedly to be reduplicated only in lichen planus. 9, t5 The desmosomes and hemidesmosomes are broken down and their number reduced in lichen planus, 15whereas in erythema dyschromicum perstans the desmosomes appear normal but retreat to one cell after cell separation? There are intranuclear bodies in lichen planus ~S and perinuclear vacuoles in erythema dyschromicum perstans. 9 Although there have been numerous reports of lichen planus and erythema dyschromicum perstans, there have been few reports of the two diseases together. Naidorf and Cohen 2 reported a case of erythema dyschromicum perstans preceding lichen planus, whereas Bhutani et al. 3 described 11 of 40 patients with erythema dyschromicum perstans with coincidental lichen planus. In the latter report, lichen planus preceded, coincided with, or followed the onset of erythema dyschromicum perstans. Classic bullous and maculopapular variants of lichen planus were described. Pinkus I~ described a patient in whom ashy patches developed after erythematous scaly papules. (He does not comment as to whether this is lichen planus.) In other reports lichen planus actinicus (or subtropicum), and lichen pigmentosus, have been mentioned as variants of lichen planus that closely
resemble erythema dyschromicum perstans, z 3.5,Jo Ramirez, 4 in reviewing 139 cases of erythema dyschromicum perstans, does not mention an associated lichen planus. Although there is a great degree of similarity between erythema dyschromicum perstans and lichen planus, there are also significant clinical and ultrastructural differences. Why is erythema dyschromicum perstans described primarily in patients from South America and India rather than from Europe and the United States? Is erythema dyschromicum perstans a variant of lichen planus that occurs primarily in darker skin? There is a strong argument for considering erythema dyschromicum perstans as a variant of lichen planus. In our case, lichen planus and erythema dyschromicum perstans may have developed coincidentatly. However, it seems more reasonable to consider both types of lesions as variants of lichen planus. The term lichen planus pigmentosus, as offered by Bhutani et al., 3 seems more reasonable than the descriptive term erythema dyschromicum perstans until such time as the two can be separated unequivocally. In summary, we present a case of erythema dyschromicum perstans and lichen planus in the same patient, as well as clinical and ultrastructural similarities of both entities. We believe our patient manifested a spectrum of lichen planus, initially presenting with typical lichen planus and then developing classic lesions of erythema dyschromicum perstans. We prefer the term lichen planus pigmentosus, rather than erythema dyschromicum
perstans. REFERENCES 1. Arndt KA. Lichen planus. In: Fitzpatrick TB, Eiscn AZ, Wolff K, Freedberg [M, Austen KF, eds. Dermatology in general medicine. 3rd ed. New York: McGraw-Hill, 1987:967-73. 2. Naidorf KF, Cohen SR. Erythema dyscbromicurn perstans and lichen planus. Arch Dermatol 1982;118: 683-5. 3. Bhutani LK, Bedi TR, Pandhi RK, Nayak NC. Lichen planus pigmentosus. Dermatologica 1974;149:43-50. 4. Ramirez CO. The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis 1967;3:244-7. 5. Tschen JA, Tschen EA, McGavran MH. Erythema dyschromicum perstans. J AM AcAr~ DEr~MA'rot. 1980;2:295-302. 6. Convit J, Kerdel-Vegas F, Rodriguez G. Erythema dys-
442
7. 8. 9. 10.
Berger et al. chromicum perstans: a hitherto undescribed skin disease. J Invest Dermatol 1967;36:457-62. Knox JM, Dodge BG, Freeman RG. Erytherna dyschromicum perstans. Arch Dermatol 1968;97:262-72. Kark EC, Litt JZ. Ashy dermatosis: a variant of lichen planus? Cuffs 1980;25:631-3. Sorer NA, Wand C, Freeman RG. Ultrastructural pathology of erythema dyschromicum perstans. J Invest Dermatol 1969;52:155-62. Pinkus H. Lichenoid tissue reactions. Arch Dermatol 1973;107:840-6.
Journal of the American Academy of Dermatology 11. Ackerman AB, eel. Histologic diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger, 1978:197-8. 12. Lever WF, Schaumburg-Lever G, eds. Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983:138. 13. Stevenson JR, Miura M. Erythema dyschromicam perstans (ashy dermatosis). Arch Dermatol 1966;94:196-9. 14. Baart de la Faille-Kuyper EH, Baart de la Faille H, An immunofluorescence study of lichen planus. Br J Dermatol 1974;90:365-71. 15. Johnson FR, Fry L. Ultrastructural observations on lichen planus. Arch Dermatol 1967;95:596-607.