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LETTER TO EDITOR
Year : 2015  |  Volume : 16  |  Issue : 2  |  Page : 113-114

Generalized morphea in an infant with favorable outcome to topical steroid


1 Department of Pediatrics, Midnapur Medical College, Pashim Medinipur, West Bengal, India
2 Department of Physiology, R. G. Kar Medical College, Kolkata, West Bengal, India
3 Department of Pediatrics, Medical College, Kolkata, West Bengal, India
4 Department of Dermatology, Medical College, Kolkata, West Bengal, India

Date of Web Publication9-Apr-2015

Correspondence Address:
Pranab Kumar Dey
Govt. Housing Estate, Block B, Flat 6, 82 Belgachia Road, Kolkata 700 037, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.152123

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How to cite this article:
Dey PK, Banerjee J, Datta K, Bandyopadhyay D. Generalized morphea in an infant with favorable outcome to topical steroid. Indian J Paediatr Dermatol 2015;16:113-4

How to cite this URL:
Dey PK, Banerjee J, Datta K, Bandyopadhyay D. Generalized morphea in an infant with favorable outcome to topical steroid. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Sep 19];16:113-4. Available from: http://www.ijpd.in/text.asp?2015/16/2/113/152123

Sir,

A 10-month-old male infant presented to us with a thickened circular, depressed skin lesion on his upper back and three similar lesions over the scalp and forehead. The lesions had progressed in size from small spots to larger lesions that felt hard and became discolored since the age of 6 weeks. The child had no history of physical trauma, bites or history of inflammation lesion over those sites. He was delivered normally after full-term pregnancy of a nonconsanguinous marriage. He had no history of delayed developmental milestones. There was no family history of similar lesions. There were no apparent symptoms of pain or pruritus at any time. On examination, the largest lesion on the upper trunk was a plaque [Figure 1]. There were three other plaques with the same morphology (approximately 6 cm, 4 cm and 3 cm in diameter) circular over occipital region of the scalp, right temporal region and left supraorbital region respectively. No other skin, underlying muscles, extracutaneous manifestations and developmental dysmorphological features were present. Hemoglobin, white blood cell, platelets, erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, antinuclear antibody and rheumatoid arthritis factor were within normal limits. Magnetic resonance imaging of brain and spine showed normal study. Histopathology of a lesional biopsy from interscapular area were described in [Figure 2]. A diagnosis of generalized morphea was made. He was treated with twice daily topical betamethasone dipropionate (0.05%). Evolution was favorable after 1-year of treatment, with obvious improvement of skin lesions.
Figure 1: A plaque type, approximately 10 cm in diameter, circular, indurated and scar-like lesion over the interscapular region

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Figure 2: Histopathology showed orthokeratotic epidermis and perivascular lymphocytic infiltrates and sclerosis of collagen in the papillary dermis (H and E, ×40)

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In a large, multicenter, international study of juvenile localized scleroderma of 750 children, generalized morphea were present in 7% of patients and the mean age at disease onset was 8.7 years (range, 0.6-16). The mean time between the first manifestation of the disease and diagnosis was 1.7 years (median 1-year, range, 0-16.7 years). [1] It is rare during infancy. The diagnostic criteria of generalized morphea consists of (1) four or more plaques become larger than 3 cm and merge, (2) involvement of two or more anatomical areas of the body out of seven areas (head and neck, left or right upper limb or lower limb, anterior or posterior trunk). [2] The diagnosis of morphea is primarily clinical, sometimes supplemented by skin biopsy. Histopathology findings of morphea vary according to the stage of the disease. In the initial inflammatory phase, collagen bundles in reticular dermis are thickened and there is an interstitial and perivascular lymphocytic inflammatory infiltrate. It can also affect the subcutaneous adipose tissue, and replace it with thickened trabeculars of new formed collagen. In the late sclerotic phase, the inflammatory infiltrate is reduced. Reticular dermis shows thickened collagen, arranged in bundles, and a mild infiltrate consisting mostly of eosinophils. In papillary dermis, normal fibers may be replaced by uniform collagen. [3] Histopathological examination in our case was consistent with morphea, in the initial inflammatory phase. The treatment of generalized morphea is challenging and unlike localized morphea, lesions of generalized morphea show less frequent spontaneous involution. High-potency topical glucocorticoids may be applied locally, with their effect augmented by intralesional injections of triamcinolone. A number of the systemic agents may also help generalized disease including systemic steroids, penicillamine, antimalarials, low-dose methotrexate, sulfasalazine, salazopyrin and ciclosporin, ultraviolet light A phototherapy and photochemotherapy. [4] Significant improvement was seen in our patient received only potent topical steroid, may be the lesions were in the initial inflammatory phase, although spontaneous improvement cannot be excluded.

 
  References Top

1.
Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2006;45:614-20.  Back to cited text no. 1
    
2.
Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol 2006;18:606-13.  Back to cited text no. 2
    
3.
Jaworsky C. Connective tissue diseases. In: Elder DE, Elenitsas R, Murphy GF, Johnson BL Jr, editors. Lever's Histopathology of the Skin. 9 th ed. Philadelphia: Lippincott Raven; 2004. p. 310-5.  Back to cited text no. 3
    
4.
Goodfield MJ, Jones SK, Veale DJ. The connective tissue diseases. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7 th ed. Oxford: Blackwell Science; 2004. p. 56.81-3.  Back to cited text no. 4
    


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