Keywords: linear; atrophoderma; Moulin; linear scleroderma
Citation: Patsatsi A, Kyriakou A, Chaidemenos G, Sotiriadis D. Linear atrophoderma of Moulin: a case report and review of the literature. Dermatol Pract Conc. 2013;3(1):3. http://dx.doi.org/10.5826/dpc.0301a03.
History: Received: June 14, 2012. Accepted: November 25, 2012. Published: January 31, 2013.
Copyright: ©2013 Patsatsi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Competing interests: The authors have no conflicts of interest to disclose.
Corresponding Author: Aikaterini Patsatsi, M.D., Plagiari PO Box 461, 57500 Thessaloniki, Greece. Tel. +306944500285; Fax. +302310991583. Email: email@example.com
Linear atrophoderma of Moulin is a rare, acquired, linear dermatosis. We present a 17-year-old girl with multiple asymptomatic brownish atrophic plaques in a zosteriform distribution on the left side of the trunk. Clinical presentation and dermatopathology was compatible with the diagnosis of linear atrophoderma. Twenty years after its initial description by Moulin, there are yet a limited number of case reports and unanswered questions regarding this entity.
A 17-year-old girl presented with a six-month history of multiple asymptomatic brownish atrophic plaques in a zosteriform distribution on the left side of her trunk (Figure 1). There was no family history of a similar skin disease. Laboratory studies were unremarkable for any chronic or autoimmune disorder. Biopsy of a lesion showed a normal epidermis with increased pigmentation of the basal layer and a broad dermis with thickened collagen fibers and diminished periadnexal and subcutaneous fat tissue (Figures 2, 3, 4).
Figure 1. ((A & B) Atrophic plaques on the left side of the trunk. [Copyright: ©2013 Patsatsi et al.]
Figure 2. Normal epidermis and broad dermis. [Copyright: ©2013 Patsatsi et al.]
Figure 3. Normal epidermis with hyperpigmented basal layer. [Copyright: ©2013 Patsatsi et al.]
Figure 4. Thickened collagen fibers in the dermis. [Copyright: ©2013 Patsatsi et al.]
Clinical and dermatopathologic findings were compatible with atrophoderma of Moulin. The use of topical steroids and tacrolimus as a second-line regimen did not result in any improvement of lesions.
Linear atrophoderma is a rare, acquired, linear dermatosis. It is named after Moulin, who, in 1992, reported on five patients with pigmented and more or less atrophic bands along Blaschko’s lines .
The age of onset in the first described cases ranged from 6 to 20 years. Lesions were unilateral, forming a recumbent “S” pattern, and the intensity of pigmentation and atrophy was variable. They remained stable throughout an observation period of 2 to 30 years. Of the skin biopsies performed on three patients, there was only irregular and moderate hyperpigmentation of the basal layer. In the dermis, there was no distinct pigment incontinence, no inflammation or alteration of connective tissue texture, and the clinical impression of skin atrophy was attributed to atrophy of the subcutaneous tissue .
Up to now there have been 30 reported cases of linear atrophoderma of Moulin [2-22]. In 2005, Ang et al mentioned that many cases of linear dermatoses were grouped under the umbrella of linear atrophoderma . In Table 1, 30 reported cases that resemble the initial description of linear atrophoderma of Moulin are listed.
Table 1. Reported cases of linear atrophoderma of Moulin [Copyright: ©2013 Patsatsi et al.]
Now, twenty years after the initial description of this entity, there are still some unresolved issues. The differential diagnosis of zosteriform or linear scleroderma is not clear clinically nor dermatopathologically. It may well be that linear atrophoderma of Moulin and zosteriform or linear scleroderma belong to the spectrum of a single disease.
With the growing literature it has been demonstrated that age of onset is not limited to childhood or adolescence. The disease may also present later in life. Lesions are not always unilateral. They may be bilateral but in a linear distribution along Blaschko’s lines (Table 1). Distribution mainly along the Blaschko lines reflects mosaicism.
Dermatopathologic findings of linear atrophoderma of Moulin vary. The epidermis is normal in the majority of cases. However, in two reports there was vacuolar degeneration of the basement membrane [2,7]. Few reports suggest an inflammatory early stage is suggested. The question of a transient inflammatory early stage that is no longer present by the time of clinical evaluation and biopsy remains.
Although the alteration of the connective tissue is not described in the original paper, most of the reviewed papers describe an increase of collagen. Unaltered, fragmented or decreased collagen fibers have been also reported in isolated cases, as well as edema of the dermis (Table 1). Another issue that has not been addressed is the cause of the clinical presentation of atrophic plaques. Does the loss of subcutaneous fat result in atrophy?
In conclusion, even now, 20 years after the first description of atrophoderma of Moulin this rare disease is puzzling. It seems to occur at any age. It is characterized clinically by the presence of atrophic patches distributed along Blaschko lines and dermatopathologically by a normal epidermis with a hyperpigmented basal layer, a dermis with thickened collagen fibers and loss of subcutaneous fat. Etiology and pathogenesis of this disease remains still unclear.
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