Address
Jaipur
Email
editor@e-ijcd.in
Indian Journal Of Clinical Dermatology

CASE REPORT

Year: 2019 I Volume: 2 I Issue: 1 I Page: 26-27

Generalized Morphea In A Child – A Rare Entity

Dr. Sonal Jain1 , Dr. Liza Mohapatra1 , Dr. Prasenjeet Mohanty1 , Dr.Jiss Elizabeth Sebastian1

1 Department of Skin & VD, SCB Medical College and Hospital. Cuttack

Corresponding Author:
Dr. Sonal Jain
15, Ashok Colony, Malviya Nagar, Jaipur 302017, Rajasthan
Email : sonalreachesout@gmail.com

How to cite this article:
Jain S, Mohapatra L, Mohanty P, Sebastian E.Generalized Morphea In A Child – A Rare Entity. JDA Indian Journal of Clinical Dermatology 2019;2:26-27.

Abstract:

Generalized morphea is a rare presentation, if present in children. We present a 5-year old female child who developed tautness of skin since 3 months.

Key words: generalized morphea, children, rare

Introduction:

The term “Morphea” encompasses a group of related conditions characterized by varying degrees of sclerosis, fibrosis and atrophy in skin and subcutaneous tissue, sometimes extending deeply into muscle, bone and brain.[1] Anti-nuclear antibody(ANA) positivity is common but the specific autoantibodies seen in systemic sclerosis are rarely present.[1] Various type of morphea are – Limited morphea, Generalized morphea , Linear morphea & Mixed type. Generalized morphea is a rare condition, it occurs in the cases of dissesminated sclerosis with no systemic involvement. It occurs mainly in adults.It begins in the trunk and causes contractures and deformities in limbs. It differentiates from systemic sclerosis because it does not accompany Raynaud’s phenomenon and doesn’t show capillaroscopic changes.[2] The disease is extremely uncommon in children.[3]

Case report:

A 5-year female child presented with gradual onset and progressive tautness and tightness of skin since 3 months, which was accompanied by limitation of movement of hands,elbow,ankle and knee joints. Patient also complained of pain over fingers and toes without any change in colour.There was no history of regurgitation of food and difficulty in breathing.There was no history of trauma,bites or history of inflammation over those sites On examination,multiple large shiny atrophic plaques of size ranging from 3 cm to 20 cm were present over bilateral upper limb and lower limb involving the joints [Figure 1 and 2]. Contracture of small joints of hand was present, limiting the movement of hand [Figure 3]

Figure 1,2,3: Figure 1,2, 3 : 1. Large plaques of morphea over right upper limb. 2. Large plaques of morphea over both legs. 3. Contracture of small joints of hands

Click here to view

.Matt telangiectesia was seen over lips with telangiectesia over bilateral cheeks(without any history of any topical application)[Figure 4 and 5].

Figure 4, 5 : 4. Telanigectesia over face. 5. Matt telangiectesia over lips

Click here to view

Other dermatological examination was unremarkable. Based on history and clinical examination, a differential diagnosis of generalized morphea and CREST syndrome was considered.Complete blood count,ESR thyroid profile, Anti-nuclear antibody(ANA), Extractable nuclear antibody(ENA) profile and upper GI endoscopy were all within normal limit.Histopathology revealed dermal stroma with packed collagen bundles,eccrine glands with replacement of periadnexal fat by collagen and moderate lymphocytes, plasma cells and histiocytes around vessels[Figure 6 and 7].

Figure 6: Epidermal atrophy and dermal stroma with thick collagen bundles. (H & E Scanner View)

Click here to view
Figure 7: Dermal stroma with thick collagen bundles, atrophy of pilosebaceous units with hypertrophied piloarrector muscle, presence of eccrine sweat glands in the dermis with replacement of periadnexal fat by collagen and moderate lymphocytes, plasma cells and histiocytes around blood vessels. (H & E 10×)

Click here to view

Based on clinicohistological correlation,a diagnosis of generalized morphea was made.Patient was started with oral prednisolone (10 mg),topical calcipotriol and physiotherapy with an improvement of around 40%.

Discussion:

When scleroderma of any type affects children, it is called Juvenile Scleroderma.[4] They constitute about 10% of patients suffering from scleroderma.[5] Generalized morphea is rare in infants. The diagnostic criteria of generalized morphea consists of – 1. 4 or more plaques become larger than 3 cm and merge, 2.Involvement of 2 or more anatomical areas[Head and neck, left or right upper limb or lower limb, anterior and posterior trunk].[6] The diagnosis of morphea is primarily clinical.

We excluded the differential of CREST syndrome by means of history and negative Anti-nuclear antibody and Extractable nuclear antibody profile

In a large multicentre, international study of Juvenile Localized Scleroderma of 750 children, generalized morphea was present in 7% of patients. It is rare during childhood.[7]

In 1976,Ansell et al(8) described 2 cases (10-year girl and 8-year boy) of generalized morphea with fixed flexion deformities of joints at the site of morphea lesions, similar to our case.

In 2003,Brar BK et al(3) reported a 9-year girl with generalized morphea, following measles vaccination.

In 2015,PK Dey et al(9) described a 10-month male infant with generalized morphea and reported its response to topical steroids.

The above cases lacked presence of telangiectesia over lips and face which was present in our case and drifted us to rule out CREST syndrome.

Only a handful cases have been reported from the Indian subcontinent,hence this case is being reported.

References:

1. Ajili F, Garbouj W, Boussetta N, Laabidi J, Abdelhafidh NB, Louzir B, Othmani S. Werner Syndrome: A new case report. Our Dermatol Online. 2013 Oct 1;4:490-2.

2. Sert M, Fakioglu K, Tetiker T. Review of Two Siblings with Werner’s Syndrome: A Case Report. Case reports in medicine. 2009;2009.

3. Khan GA, Azfar NA, Malik LM, Sammar T, Jahangir M. Werner syndrome: A case report and review of literature. Journal of Pakistan Association of Dermatology. 2016 Dec 22;21(4):304-8.

4. Muftuoglu M, Oshima J, von Kobbe C, Cheng WH, Leistritz DF, Bohr VA. The clinical characteristics of Werner syndrome: molecular and biochemical diagnosis. Human genetics. 2008 Nov 1;124(4):369-77.

5. Irvine AD, Mellerio JE. Syndromes with Premature Ageing. Rook’s Textbook of Dermatology, Ninth Edition. 2016 Jul 15:1-9.

230 comments on “Generalized Morphea In A Child – A Rare Entity”

  9. Very nice post. I just stumbled upon your blog and wanted to say that I’ve really enjoyed browsing your blog posts. In any case I’ll be subscribing to your feed and I hope you write again soon!

  10. I have read your article carefully and I agree with you very much. This has provided a great help for my thesis writing, and I will seriously improve it. However, I don’t know much about a certain place. Can you help me?

  16. Avoid fancy mascaras. Within the past few years, cosmetic companies have marketed mascaras with “extension fibers” that will build on top of your natural eyelashes to make them look longer. While this may be the effect you’re looking for, those fibers can flake off and get into your eyes. Besides that, the ingredients used to create the fibrous look can irritate the eyes. Keep it simple by using mascaras with limited ingredients and a hypoallergenic formula. What it is: ILIA’s Limitless Lash Mascara is an award-winning clean mascara that goes above and beyond the expectations of any natural mascara. Buildable and flake-resistant, Limitless Lash Mascara lengthens, lifts, and separates with just the right amount of volume.
    https://neurofeedbackalliance.org/community/profile/yettajuo2065060/
    Seriously why! My life has not been the same ever since you took this product out of my life . The epic eyeliner is HORRENDOUS!!!!!!! I don’t understand why you guys would discontinue the most loved eyeliner out there from so many people ! Customer Reviews Trace your upper lash line with the thin, precision brush tip to define your eyes with matte color, or extend the line past your outer corners to create a winged eyeliner look. Pair it with mascara (we recommend On The Rise Volume Liftscara) for eyes that really pop. RRP: CA$14.00 Because this richly pigmented eyeliner is a pencil, you’ll have plenty of control during application, but the formula isn’t dry like other pencils. This one is nice and creamy—perfect for creating a soft, smokey lash line, says Fred Connors, owner of FRED. salon.

  17. All AIs and Nolvadex is justr slowing or halt new further growth but once its there as perm tissue its there dr reddy finasteride buy Silodosin has greater affinity for the О± 1A adrenergic receptor than for the О± 1B adrenergic receptor, minimizing the propensity for blood pressure related adverse effects mediated by О± 1B blockade 135

  21. Howdy just wanted to give you a brief heads up and let you know a few of the pictures aren’t loading correctly. I’m not sure why but I think its a linking issue. I’ve tried it in two different browsers and both show the same outcome.

  85. Hebat blog ini! 🌟 Saya suka banget bagaimana penulisannya memberikan pemahaman yang baik dalam topik yang dibahas. 👌 Artikelnya menyenangkan dan informatif sekaligus. 📖 Tiap artikel membuat saya lebih penasaran untuk melihat artikel lainnya. Teruskan karya hebat

  105. Win big and win often at our Mexican online casino. With generous payouts and thrilling tournaments, the sky’s the limit when you play with us. casi te da mas.

  120. Join the elite ranks of winners at our Mexican casino site. With exclusive rewards and VIP privileges, you’ll be treated like royalty every time you play. caliente mx te da mucho.

  124. Play like a pro at our Mexican online casino. With expert tips and strategies, you’ll maximize your chances of hitting the big time. stre la clave para una buena vida.

  134. KahveOyun.com – Mobil ve bilgisayar üzerinden okey odalarında oynayarak zaman geçireceğiniz ve okey oynarken sohbet edebilme fırsatını ücretsiz sunmaktadır.

  144. Hi there to all, for the reason that I am genuinely keen of reading this website’s post to be updated on a regular basis. It carries pleasant stuff.

  158. Bu konu hakkında bilgi vermeniz çok güzel. Genellikle türkçe içerikler az oluyor fakat böyle güzel içerikler görmek ve okumak çok zevkli.

  169. Sitenizin tasarımı da içerikleriniz de harika, özellikle içerikleri adım adım görsellerle desteklemeniz çok başarılı emeğinize sağlık.

  179. This article really resonated with me. I’ve been struggling with the same issue for a while now, and it’s refreshing to see someone else articulate it so well

  186. Yerden ısıtma, geleneksel radyatörlerin aksine daha temiz ve sessiz bir ısıtma yöntemi sunar. Ayrıca, zeminin yüzeyini ısıtarak toz ve alerjen birikimini azaltabilir, böylece daha sağlıklı bir iç mekan ortamı oluşturabilir. Yerden ısıtma sistemleri, modern bina tasarımlarında ve yenileme projelerinde giderek daha popüler hale gelmektedir. Yerden Isıtma Yerden Isıtma

  199. I also run a site and if you want to ever serious in a little bit of guest writing for me if possible feel free to let me know, im always appear for people to test out my site

  205. This is really interesting, You’re a very skilled blogger. I’ve joined your feed and look forward to seeking more of your magnificent post. Also, I’ve shared your site in my social networks!

Leave a Reply

Your email address will not be published. Required fields are marked *