Advertisment Novartis
Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1044  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
CORRESPONDENCES
Year : 2017  |  Volume : 62  |  Issue : 2  |  Page : 214-215
Linear atrophoderma of moulin over face: An exceedingly rare entity


Department of Dermatology, R.G. Kar Medical College, Kolkata, West Bengal, India

Date of Web Publication9-Mar-2017

Correspondence Address:
Ivoreen Darung
Department of Dermatology, R.G. Kar Medical College, Kolkata, West Bengal
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_469_16

Rights and Permissions



How to cite this article:
Darung I, Rudra O, Samanta A, Agarwal M, Ghosh A. Linear atrophoderma of moulin over face: An exceedingly rare entity. Indian J Dermatol 2017;62:214-5

How to cite this URL:
Darung I, Rudra O, Samanta A, Agarwal M, Ghosh A. Linear atrophoderma of moulin over face: An exceedingly rare entity. Indian J Dermatol [serial online] 2017 [cited 2017 Mar 29];62:214-5. Available from: http://www.e-ijd.org/text.asp?2017/62/2/214/201763


Sir,

A 16-year-old girl presented to us with asymptomatic hyperpigmented lesions over the left side of her chin since the past 6 months. According to the patient, it started as a small black discoloration of the skin which gradually increased in size. There was no history of preceding trauma, redness or tightness of the skin, associated systemic complaints, or family history of similar illness. Cutaneous examination revealed three broad unilateral linear hyperpigmented atrophic lesions, with depressed margins along Blaschko's lines without any sign of inflammation or induration [Figure 1]a and [Figure 1]b. The surface of the atrophic lesions was wrinkled. A 4 mm punch biopsy was taken from the margin of the lesion. Histopathologic examination (HPE) with hematoxylin and eosin staining showed epidermal atrophy along with dense melanin deposition along the basal layer with apparently normal subcutaneous tissue [Figure 2]. Sparse perivascular and periappendageal lymphocytic infiltrate with slight thickening of collagen bundles was present in the dermis. There was no evidence of sclerosis or atrophy of the appendages [Figure 3]. The difference with the normal epidermis could be seen in the HPE [Figure 4]. Verhoeff–van Gieson stain showed normal elastic tissue [Figure 5]. On the basis of the clinical and histopathologic findings, we diagnosed the case as linear atrophoderma of moulin (LAM).
Figure 1: (a and b) Linear hyperpigmented atrophic plaque, with depressed margins along Blaschko's lines

Click here to view
Figure 2: Epidermal atrophy along with dense melanin deposition along the basal layer with apparently normal sub cutaneous tissue (H and E, ×40)

Click here to view
Figure 3: Sparse perivascular and periappendageal lymphocytic infiltrate with slight thickening of collagen bundles in the dermis with no evidence of sclerosis or atrophy of the appendages (highlighted with arrows) (H and E, ×100)

Click here to view
Figure 4: High power view showing the difference between normal and atrophic skin (an arrow highlighting the junction) (H and E, ×400)

Click here to view
Figure 5: Verhoeff–van Gieson stain showed normal elastic tissue (×100)

Click here to view


LAM is a rare dermatosis characterized by a hyperpigmented atrophoderma that follows Blaschko's lines with onset usually during childhood and adolescence.[1] Postzygotic mutation in lamin A gene has been postulated as a possibility for developing this disorder.[2] The trunk and limbs are usually involved without any preceding inflammation or subsequent induration and sclerosis.[3] Presentation over the face, as was seen in our case, is rarely reported in literature. The clinical atrophy is reported to be induced by a reduction of subcutaneous tissue but not dermal tissue as observed by ultrasound imaging.[4] However, subcutaneous tissue is not routinely mentioned to be reduced according to other reports and text.[1],[2] LAM is a self-limited disease. Progression of the lesions usually stops within a few months without any pattern of remission.[1] Hematoxylin and eosin staining usually show normal or atrophic epidermis, along with hyperpigmentation of the basal layer. The collagen bundles in the mid and deep dermis may be edematous or slightly homogenized in appearance. Although elastic fibers are usually normal, occasionally some clumping and loss of fibers may be seen in the deep dermis. Adnexal structures are usually preserved. Mild perivascular infiltrate is seen in the upper dermis and somewhat heavier in the deep dermis consisting of lymphocytes and a few macrophages and rarely, plasma cells. Some superficial vessels may be mildly dilated. There may be a few melanophages in the superficial dermis.[2]

The differential diagnosis includes atrophoderma of Pasini and Pierini, linear scleroderma, and porokeratosis. Clinically, the preceding inflammation, sclerosis, and induration that accompany linear scleroderma are usually absent in LAM. Histopathology of the former reveals thickened and closely packed collagen bundles with atrophic eccrine glands, hair follicles, and periappendageal fat. Atrophoderma of Pasini and Pierini clinically and histologically resembles LAM except that it does not follow Blaschko's lines.[5] Porokeratosis was excluded clinically and histopathologically by the absence of keratotic ridge with central groove and cornoid lamella, respectively.[2]

Effective treatment with methotrexate 20 mg/week for a duration of 6 months has been reported.[6] Due to esthetic nature of the disorder, use of self-tanning cream has been advised.[4] The partial improvement was reported with topical calcipotriol, intravenous penicillin together with topical PUVA, oral Potaba (potassium para-aminobenzoate), high-dose Vitamin E (400 IU/day), and topical clobetasol propionate.[1] Since none of these medications are uniformly effective for this rare dermatosis, especially over face, we started topical tretinoin (0.05%) cream empirically once at night. If we do not see any appreciable improvement after 3 months, we would consider starting methotrexate. We report this case of LAM because of paucity of its clinical and histopathological description in the literature, especially from India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
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;5:11-4.  Back to cited text no. 1
    
2.
Weedon D, editor. Viral diseases. In: Weedon's Skin Pathology. 3rd ed. Edinburgh, UK: Churchill Livingstone Elsevier; 2010.  Back to cited text no. 2
    
3.
Cecchi R, Bartoli L, Brunetti L, Pavesi M. Linear atrophoderma of Moulin localized to the neck. Dermatol Online J 2008;14:12.  Back to cited text no. 3
    
4.
Villani AP, Amini-Adlé M, Wagschal D, Balme B, Thomas L. Linear atrophoderma of moulin: Report of 4 cases and 20th anniversary case review. Dermatology 2013;227:5-9.  Back to cited text no. 4
    
5.
Zahedi Niaki O, Sissons W, Nguyen VH, Zargham R, Jafarian F. Linear atrophoderma of moulin: An underrecognized entity. Pediatr Rheumatol Online J 2015;13:39.  Back to cited text no. 5
    
6.
Zaouak A, Ghorbel HH, Benmously-Mlika R, Koubaa W, Badri T, Fenniche S, et al. A case of linear atrophoderma of moulin successfully treated with methotrexate. Dermatol Ther 2014;27:153-5.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
    Article in PDF (2,478 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed227    
    Printed0    
    Emailed0    
    PDF Downloaded9    
    Comments [Add]    

Recommend this journal

Epiduo