Predominant Telangiectatic Erythema in Linear Atrophoderma of Moulin: Novel Variant or Separate Entity?

Utikal, Jochen; Keil, Darinka; Klemke, Claus‐Detlev; Bayerl, Christiane; Goerdt, Sergij

doi:10.1159/000073096

Cited by 24 publications

(33 citation statements)

References 31 publications

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“…Telangiectasia is a prominent skin manifestation of scleroderma and related diseases [9]. Intriguingly, ‘CREST syndrome’, or limited cutaneous SSc (lcSSc), was first described as a syndrome with multiple telangiectasia mimicking HHT [10].…”

Section: Introductionmentioning

confidence: 99%

A Clue for Telangiectasis in Systemic Sclerosis: Elevated Serum Soluble Endoglin Levels in Patients with the Limited Cutaneous Form of the Disease

et al. 2006

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Background: The transforming growth factor-β (TGF-β) system plays a critical role both in systemic sclerosis (SSc) and hereditary hemorrhagic telangiectasia (HHT). Endoglin, known as a gene responsible for HHT, is a TGF-β receptor preferentially expressed on endothelial cells. The role of endoglin in SSc is potentially intriguing since limited cutaneous SSc (lcSSc) and HHT share several symptoms, including telangiectasia. Objective: To determine serum levels of soluble endoglin (sEndoglin) and clinical associations in patients with SSc. Methods: Serum sEndoglin levels were examined by ELISA in 70 patients with SSc, 20 patients with systemic lupus erythematosus and 20 healthy individuals. Results: Serum sEndoglin levels were significantly elevated in patients with lcSSc compared with diffuse cutaneous SSc and systemic lupus erythematosus patients as well as normal controls. Patients with elevated sEndoglin levels had telangiectasia more frequently than those with normal sEndoglin levels. Furthermore, pulmonary artery pressure was positively correlated with sEndoglin levels in patients with lcSSc. Conclusion: Abnormal expression/function of endoglin may be linked to lcSSc-specific manifestations.

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Section: Introductionmentioning

confidence: 99%

A Clue for Telangiectasis in Systemic Sclerosis: Elevated Serum Soluble Endoglin Levels in Patients with the Limited Cutaneous Form of the Disease

et al. 2006

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“…After the 5 first cases of linear atrophoderma reported by Moulin et al [1], 26 additional cases were described ([2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23], summarized in table 1). We include in our review of the literature a case of unilateral idiopathic atrophoderma described as a Pasini and Pierini entity but with lesions arranged along Blaschko's lines [24].…”

Section: Methodsmentioning

confidence: 99%

“…Lesions were mainly unilateral (87.5%). Four bilateral cases have been reported [12,19]. A specific ‘S' pattern was described for cases with localization on the trunk.…”

Section: Methodsmentioning

confidence: 99%

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Linear Atrophoderma of Moulin: Report of 4 Cases and 20th Anniversary Case Review

Villani

Amini‐Adlé²,

Wagschal³

et al. 2013

Dermatology

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Background: Linear atrophoderma of Moulin (LAM) is a rare clinical entity which was first described by Moulin et al. in 1992. The diagnosis is clinical, characterized by acquired unilateral hyperpigmented, depressed band-like skin lesions following Blaschko's lines. The disease typically affects children or adolescents and has a good prognosis without evidence of long-term progression. To date, the pathophysiology is unclear. Different authors hypothesize that the disease is secondary to a mosaic manifestation as a result of a post-zygotic mutational event. Observations: Four patients (2 men, 2 women) had a history of unilateral band-like skin lesions located on the lower legs (50%) or the trunk (50%). Physical examination showed atrophic and hyperpigmented skin lesions along Blaschko's lines, which appeared during childhood in 3 cases and at the age of 20 in the last case. Lesions had progressed rapidly but seemed to have stabilized so far except for 1 case who presented spontaneous improvement. Histopathological examination revealed a normal epidermis with a hyperpigmented basal layer and a perivascular lymphocytic infiltrate in the dermis. Conclusion: LAM is a rare disease with 32 reported cases and remains an exclusion diagnosis. Since the problem is mainly esthetic, treatments should not be too aggressive.

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“…The etiology of this condition remains unclear. It is thought to be caused by a somatic mutation resulting in a geno-and phenotypic mosaicism such as other dermatosis following Blaschko's lines (9). Several therapeutic approaches such as penicilins, topical steroids, heparin and oral potassium aminobenzoate have been used for linear atrophoderma of Moulin with no effective results (10).…”

Section: A B Cmentioning

confidence: 99%

Niño de 6 años de edad con manchas hiperpigmentadas en el brazo derecho y pecho

Husein‐ElAhmed¹,

Ruiz-Molina²,

Cívico-Amat³

et al. 2014

biomedica

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A 6 year-old male patient was admitted to the dermatology department with an eruption on the right arm, chest and left leg. No clinical symptoms such as pain, pruritus or previous inflammation were present. His mother reported that the lesion had developed four years ago as small-sized hyperpigmented macules which enlarged in a short period of time as band-like hyperpigmentation, and then remained unchanged. His past medical history was unremarkable and there were no similar cases in the family.On physical examination, band-like hyperpigmented patches on the right arm were observed. A similar lesion was located on the right side. When the right shoulder was on horizontal adduction, lesions of chest and arm concurred in a large brown ring-like patch. Lesions on left leg showed the similar bandlike distribution with a darker brown color (figure 1). There were no signs of induration or sclerosis.Laboratory investigations included complete blood count, liver and kidney function tests, erythrocyte sedimentation rate, and antinuclear antibody test, which were negative or within the normal range.A skin biopsy was performed. Histological examination showed a normal epidermis outlined by a hyperpigmented basal layer. In the papillary dermis, vessels were surrounded by mild lymphocytic infiltrate and melanin-laden macrophages were present (figure 2).

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Predominant Telangiectatic Erythema in Linear Atrophoderma of Moulin: Novel Variant or Separate Entity?

Cited by 24 publications

References 31 publications

A Clue for Telangiectasis in Systemic Sclerosis: Elevated Serum Soluble Endoglin Levels in Patients with the Limited Cutaneous Form of the Disease

A Clue for Telangiectasis in Systemic Sclerosis: Elevated Serum Soluble Endoglin Levels in Patients with the Limited Cutaneous Form of the Disease

Linear Atrophoderma of Moulin: Report of 4 Cases and 20th Anniversary Case Review

Niño de 6 años de edad con manchas hiperpigmentadas en el brazo derecho y pecho

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