Dermatologic Therapy, Vol. ••, 2013, ••–•• Printed in the United States · All rights reserved
© 2013 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE A case of linear atrophoderma of Moulin successfully treated with methotrexate Anissa Zaouak*, Houda Hammami Ghorbel*, Rym Benmously-Mlika*, Wafa Koubaa†, Talel Badri*, Samy Fenniche* & Insaf Mokhtar* *Dermatology Department and †Anatomopathology Department, Habib Thameur Hospital, Montfleury, Tunis, Tunisia
ABSTRACT: Linear atrophoderma of Moulin is an acquired rare and self-limited skin condition. It is characterized by atrophic bandlike skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. Usually it begins in childhood or adolescence and there is no evidence of any long term progression. We describe a case of a 21-year-old woman with clinical and histological features of linear atrophoderma of Moulin. The patient was successfully treated with methotrexate 20 mg/week during 6 months with an improvement of skin pigmentation and atrophy. Approximately, 30 cases of linear atrophoderma of Moulin were described in the literature. There is not a proven effective treatment of this dermatosis. High dose penicillin, topical corticosteroids, heparin, and oral potassium aminobenzoate have been used but found to be uneffective. To our knowledge, this is the first case of extensive linear atrophoderma of Moulin treated with methotrexate. KEYWORDS: linear atrophoderma, methotrexate, treatment
Linear atrophoderma of Moulin (LAM) is an acquired rare and self-limited skin condition. It is characterized by atrophic band-like skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. Usually it begins in childhood or adolescence and there is no evidence of any long-term progression (1,2). No previous reports have discussed the treatment of LAM. This case is reported to discuss the use of methotrexate as an effective treatment of this rare skin condition.
Address correspondence and reprint requests to: Houda Hammami Ghorbel, MD, Dermatology Department, Habib Thameur Hospital, Ali Ben Ayed Street, N°8, 1008, Montfleury, Tunis, Tunisia, or email: [email protected].
A 21-year-old woman had a 10-year history of asymptomatic linear hyperpigmented atrophic lesions in a band-like arrangement on the right side of her body. On physical examination, the patient exhibited wide brown linear skin lesions localized on the right side of her abdomen (FIG. 1a), her back, a linear S-shaped hyperpigmented band on her right arm (FIG. 1c) and right thigh and leg. The plaques had no induration or sclerosis. There were some erythematous areas on hyperpigmented plaques. The patient was otherwise healthy. No family history of similar disorder was reported. Blood tests including cell blood count, liver and kidney functions, antinuclear antibodies and anti–DNA antibodies, anticentromere antibodies, protein profile, and erythrocyte sedimentation rate were all within normal ranges. A
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Zaouak et al.
a
c
b
d
e
FIG. 1 (a,b) Before treatment: Hyperpigmented linear atrophic lesions in a band-like arrangement on the right side of her body along the lines of Blaschko. (c) Presence of an irregular and moderate hyperpigmentation of the basal layer of the epidermis with normal collagen budles and elastic fibers (hematoxylin and eosin [HE] × 10). (d) Perivascular lymphocytic infiltrate in the dermis (HE × 40). (e) Left picture, before treatment: atrophic pigmented plaque on the back; right picture: improvement in skin coloration and texture 6 months after methotrexate therapy.
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Atrophoderma of Moulin responding to methotrexate
skin biopsy specimen was taken from her arm and histopathologic examination showed hyperpigmentation of the basal layer of the epidermis with a perivascular lymphocytic infiltrate in the dermis without any other pathological feature (FIG. 1d). The diagnosis of LAM was made. The patient was then treated with methotrexate 20 mg/week during 6 months with an improvement of skin pigmentation and atrophy (FIG. 1e). LAM was first described by Moulin et al. in 1992 (3). Approximately, 30 cases of LAM were described in the literature (4). The diagnostic criteria include onset during childhood or adolescence, development of hyperpigmented, slightly atrophic unilateral lesions following Blaschko lines on the trunk or limbs, absence of prior inflammation or subsequent scleroderma, a stable nonprogressive clinical course without a pattern of remission and histological findings showing hyperpigmentation of the basal epidermis (1). Our patient responds to these diagnostic criteria. A preceding inflammatory phase was noted clinically and histologically in a few reported cases (5). In our patient, we noted the coexistence of pigmented and erythematous areas on linear distributed skin lesions of LAM. Our data support the hypothesis that LAM had a history of inflammatory phase, which ultimately evolved into hyperpigmentation with atrophy (5). Differential diagnosis of this rare skin condition includes mainly idiopathic atrophoderma of Pasini and Pierini and linear scleroderma. In addition, other pigmentary dermatoses that follow Blaschko’s lines such as incontinentia pigmenti, lichen striatus, nevoid hypermelanosis, or focal dermal hypoplasia (2) may be considered. Histopathologically, it is characterized by an irregular and moderate hyperpigmentation of the lower part of the epidermis with few perivascular lymphocytes in the dermis as in our patient. The collagen bundles and the elastic fibers are unchanged (2,6). Alteration of the collagen component, should lead to the diagnosis of atrophoderma of Pasini and Pierini. The clinical atrophy seems to be induced by a reduction of the subcutaneous tissue (7). Its origin and pathogenesis remain unknown. It may reflect mosaïcism caused by a postzygotic mutation that occurred at an early developmental stage (2,5). There is not a proven effective treatment of LAM. High-dose penicillin (2), topical corticosteroids, heparin, and oral potassium aminobenzoate have been used, but found to be ineffective (8). A partial
response to topical calcipotriol was recently reported (9). Our patient was treated with methotrexate with a remarkable progressive regression of the hyperpigmented patches especially on her trunk and her right forearm. There is ongoing uncertainty about the nosological relationships between LAM and other diseases, such as atrophoderma of Pasini and Pierini or linear scleroderma. The response to methotrexate, which is an effective therapeutic option for morphea in our case, may suggest that these three entities are a spectrum of common disease. To our knowledge, this is the first case of extensive LAM treated with methotrexate. Hence, methotrexate seems to be beneficial for patients with LAM as it improves the clinical manifestations of the disease especially hyperpigmentation. Besides, it is well tolerated with rare occurrence of significant adverse events. More controlled studies are required to emphasize that methotrexate could be a safe and effective treatment of this rare skin condition with has mainly a cosmetic blemish.
References 1. Moulin G, Hill MP, Guillaud V, Barrut D, Chevallier J, Thomas L. Bandes pigmentées atrophiques acquises suivant les lignes de Blaschko. Ann Dermatol Venereol 1992: 119: 729– 736. 2. Danarti R, Bittar M, Happle R, Konig A. Linear atrophoderma of Moulin: postulation of mosaicism for a predisposing gene. J Am Acad Dermatol 2003: 49: 492–498. 3. Moulin G, Hill MP, Guillaud V, Barrut D, Chevallier J, Thomas L. Bandes pigmente’es atrophiques acquises suivant les lignes de Blaschko. Ann Dermatol Venereol 1992: 119: 729– 736. 4. Patsatsi A, Kyriakou A, Chaidemenos G, Sotiriadis D. Linear atrophoderma of Moulin: a case report and review of the literature. Dermatol Pract Concept 2013: 3: 7–11. 5. Browne C, Fisher BK. Atrophoderma of Moulin with preceding inflammation. Int J Dermatol 2000: 39: 850–852. 6. Rompel R, Mischake AL, Langner C, Happle R. Linear atrophoderma of Moulin. Eur J Dermatol 2000: 10: 611–613. 7. Norisugi O, Makino T, Hara H, Matsui K, Furuichi M, Shimizu T. Evaluation of skin atrophy associated with linear atrophoderma of Moulin by ultrasound imaging. J Am Acad Dermatol 2011: 65: 232–233. 8. Demirci GT, Altunay IK, Mertoglu E, Kucukunal A, Sakiz D. Linear atrophoderma of Moulin on the neck. J Dermatol 2011: 3: 17–49. 9. Wongkietkachorn K, Intarasupht J, Srisuttiyakorn C, Aunhachoke K, Nakakes A, Niumpradit N. Linear atrophoderma of moulin: a case report and review of the literature. Case Rep Dermatol 2013: 5: 11–14.
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© 2013 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE A case of linear atrophoderma of Moulin successfully treated with methotrexate Anissa Zaouak*, Houda Hammami Ghorbel*, Rym Benmously-Mlika*, Wafa Koubaa†, Talel Badri*, Samy Fenniche* & Insaf Mokhtar* *Dermatology Department and †Anatomopathology Department, Habib Thameur Hospital, Montfleury, Tunis, Tunisia
ABSTRACT: Linear atrophoderma of Moulin is an acquired rare and self-limited skin condition. It is characterized by atrophic bandlike skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. Usually it begins in childhood or adolescence and there is no evidence of any long term progression. We describe a case of a 21-year-old woman with clinical and histological features of linear atrophoderma of Moulin. The patient was successfully treated with methotrexate 20 mg/week during 6 months with an improvement of skin pigmentation and atrophy. Approximately, 30 cases of linear atrophoderma of Moulin were described in the literature. There is not a proven effective treatment of this dermatosis. High dose penicillin, topical corticosteroids, heparin, and oral potassium aminobenzoate have been used but found to be uneffective. To our knowledge, this is the first case of extensive linear atrophoderma of Moulin treated with methotrexate. KEYWORDS: linear atrophoderma, methotrexate, treatment
Linear atrophoderma of Moulin (LAM) is an acquired rare and self-limited skin condition. It is characterized by atrophic band-like skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. Usually it begins in childhood or adolescence and there is no evidence of any long-term progression (1,2). No previous reports have discussed the treatment of LAM. This case is reported to discuss the use of methotrexate as an effective treatment of this rare skin condition.
Address correspondence and reprint requests to: Houda Hammami Ghorbel, MD, Dermatology Department, Habib Thameur Hospital, Ali Ben Ayed Street, N°8, 1008, Montfleury, Tunis, Tunisia, or email: [email protected].
A 21-year-old woman had a 10-year history of asymptomatic linear hyperpigmented atrophic lesions in a band-like arrangement on the right side of her body. On physical examination, the patient exhibited wide brown linear skin lesions localized on the right side of her abdomen (FIG. 1a), her back, a linear S-shaped hyperpigmented band on her right arm (FIG. 1c) and right thigh and leg. The plaques had no induration or sclerosis. There were some erythematous areas on hyperpigmented plaques. The patient was otherwise healthy. No family history of similar disorder was reported. Blood tests including cell blood count, liver and kidney functions, antinuclear antibodies and anti–DNA antibodies, anticentromere antibodies, protein profile, and erythrocyte sedimentation rate were all within normal ranges. A
1
Zaouak et al.
a
c
b
d
e
FIG. 1 (a,b) Before treatment: Hyperpigmented linear atrophic lesions in a band-like arrangement on the right side of her body along the lines of Blaschko. (c) Presence of an irregular and moderate hyperpigmentation of the basal layer of the epidermis with normal collagen budles and elastic fibers (hematoxylin and eosin [HE] × 10). (d) Perivascular lymphocytic infiltrate in the dermis (HE × 40). (e) Left picture, before treatment: atrophic pigmented plaque on the back; right picture: improvement in skin coloration and texture 6 months after methotrexate therapy.
2
Atrophoderma of Moulin responding to methotrexate
skin biopsy specimen was taken from her arm and histopathologic examination showed hyperpigmentation of the basal layer of the epidermis with a perivascular lymphocytic infiltrate in the dermis without any other pathological feature (FIG. 1d). The diagnosis of LAM was made. The patient was then treated with methotrexate 20 mg/week during 6 months with an improvement of skin pigmentation and atrophy (FIG. 1e). LAM was first described by Moulin et al. in 1992 (3). Approximately, 30 cases of LAM were described in the literature (4). The diagnostic criteria include onset during childhood or adolescence, development of hyperpigmented, slightly atrophic unilateral lesions following Blaschko lines on the trunk or limbs, absence of prior inflammation or subsequent scleroderma, a stable nonprogressive clinical course without a pattern of remission and histological findings showing hyperpigmentation of the basal epidermis (1). Our patient responds to these diagnostic criteria. A preceding inflammatory phase was noted clinically and histologically in a few reported cases (5). In our patient, we noted the coexistence of pigmented and erythematous areas on linear distributed skin lesions of LAM. Our data support the hypothesis that LAM had a history of inflammatory phase, which ultimately evolved into hyperpigmentation with atrophy (5). Differential diagnosis of this rare skin condition includes mainly idiopathic atrophoderma of Pasini and Pierini and linear scleroderma. In addition, other pigmentary dermatoses that follow Blaschko’s lines such as incontinentia pigmenti, lichen striatus, nevoid hypermelanosis, or focal dermal hypoplasia (2) may be considered. Histopathologically, it is characterized by an irregular and moderate hyperpigmentation of the lower part of the epidermis with few perivascular lymphocytes in the dermis as in our patient. The collagen bundles and the elastic fibers are unchanged (2,6). Alteration of the collagen component, should lead to the diagnosis of atrophoderma of Pasini and Pierini. The clinical atrophy seems to be induced by a reduction of the subcutaneous tissue (7). Its origin and pathogenesis remain unknown. It may reflect mosaïcism caused by a postzygotic mutation that occurred at an early developmental stage (2,5). There is not a proven effective treatment of LAM. High-dose penicillin (2), topical corticosteroids, heparin, and oral potassium aminobenzoate have been used, but found to be ineffective (8). A partial
response to topical calcipotriol was recently reported (9). Our patient was treated with methotrexate with a remarkable progressive regression of the hyperpigmented patches especially on her trunk and her right forearm. There is ongoing uncertainty about the nosological relationships between LAM and other diseases, such as atrophoderma of Pasini and Pierini or linear scleroderma. The response to methotrexate, which is an effective therapeutic option for morphea in our case, may suggest that these three entities are a spectrum of common disease. To our knowledge, this is the first case of extensive LAM treated with methotrexate. Hence, methotrexate seems to be beneficial for patients with LAM as it improves the clinical manifestations of the disease especially hyperpigmentation. Besides, it is well tolerated with rare occurrence of significant adverse events. More controlled studies are required to emphasize that methotrexate could be a safe and effective treatment of this rare skin condition with has mainly a cosmetic blemish.
References 1. Moulin G, Hill MP, Guillaud V, Barrut D, Chevallier J, Thomas L. Bandes pigmentées atrophiques acquises suivant les lignes de Blaschko. Ann Dermatol Venereol 1992: 119: 729– 736. 2. Danarti R, Bittar M, Happle R, Konig A. Linear atrophoderma of Moulin: postulation of mosaicism for a predisposing gene. J Am Acad Dermatol 2003: 49: 492–498. 3. Moulin G, Hill MP, Guillaud V, Barrut D, Chevallier J, Thomas L. Bandes pigmente’es atrophiques acquises suivant les lignes de Blaschko. Ann Dermatol Venereol 1992: 119: 729– 736. 4. Patsatsi A, Kyriakou A, Chaidemenos G, Sotiriadis D. Linear atrophoderma of Moulin: a case report and review of the literature. Dermatol Pract Concept 2013: 3: 7–11. 5. Browne C, Fisher BK. Atrophoderma of Moulin with preceding inflammation. Int J Dermatol 2000: 39: 850–852. 6. Rompel R, Mischake AL, Langner C, Happle R. Linear atrophoderma of Moulin. Eur J Dermatol 2000: 10: 611–613. 7. Norisugi O, Makino T, Hara H, Matsui K, Furuichi M, Shimizu T. Evaluation of skin atrophy associated with linear atrophoderma of Moulin by ultrasound imaging. J Am Acad Dermatol 2011: 65: 232–233. 8. Demirci GT, Altunay IK, Mertoglu E, Kucukunal A, Sakiz D. Linear atrophoderma of Moulin on the neck. J Dermatol 2011: 3: 17–49. 9. Wongkietkachorn K, Intarasupht J, Srisuttiyakorn C, Aunhachoke K, Nakakes A, Niumpradit N. Linear atrophoderma of moulin: a case report and review of the literature. Case Rep Dermatol 2013: 5: 11–14.
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