(Last Updated On: May 4, 2019)

Authors: Carmelie Marisse A. Villespin, MD, Lunardi Bintanjoyo, MD, Gracia B. Teodosio,MD, FPDS, Mary Ann P. Aguado, MD, FPDS

Abstract

Introduction: There are very few reported incidences of juvenile dermatomyositis in the Philippine setting.

Case Summary: This is a case of a 9-year-old female from Batangas City, who came in with a 3-year history of multiple non-tender, non-pruritic erythematous papules which started on the dorsal aspect of the metacarpophalangeal (MCP) and interphalangeal joints of the hands, with some progressing into plaques on the face and extremities. No other associated symptoms such as fever, cough, colds or weakness were noted. Three months prior to consult, there was persistence of the above-mentioned lesions with body weakness described as difficulty getting out of bed and climbing stairs. Consult with a dermatologist and rheumatologist was done.

A skin punch biopsy showed hyperkeratosis of the stratum corneum. There was focal vacuolar alteration of the basal cell layer with thickening of the basement membrane zone. The papillary dermis showed pigment-laden macrophages, a calcified nodule, fibrosis, and a sparse perivascular inflammatory infiltrate of lymphocytes. There was also thickening of the basement membrane zone on Periodic acid-Schiff stain. Both clinical and histopathological findings point to Juvenile Dermatomyositis.

Conclusion: Juvenile Dermatomyositis requires prompt diagnosis for proper treatment and prognostication. This entails extensive diagnostic procedures such as skin punch biopsy, muscle enzymes such as CK-MB and CK- MM, and blood tests. Co-management with a pediatric rheumatologist is highly advised for initiation and regulation of oral corticosteroids as well as vitamin supplementation.  

Citation

Villespin CM, Bintanjoyo L, Teodosio G, Aguado MA. It’s a bumpy ride: A report of a 9–year-old Filipino female with dermatomyositis. J Phil Dermatol Soc. 2018; 27(1): 68-70

 

Keywords

Juvenile Dermatomyositis, Connective tissue, rheumatology

1. Dalakas M. and Hohlfeld M. Polymyositis and Dermatomyositis. Lancet. 2003; 362:971-982

2. Klein, R., Teal V., Taylor L., Troxel B. and Werth V. Number, Characteristics and Classification of Patients with Dermatomyositis seen by dermatology and rheumatology departments at a large tertiary medical center. J Am Acad Dermatoly.2007;57:937-43.

3. Peloro T. Miller O. Hahn T. Newman E.: Juvenile Dermatomyositis: A retrospective review of a 30 year experience. J Am Acad Dermatoly. 2001; 45:28-34.

4. Feldman B. Rider L. Reed A. Pachman L. Juvenile Dermatomyositis and other idiopathic inflammatory myopathies in childhood. Lancet 2008; 371: 2201–12.

5. Fisler R. Liang M. Fuhlbrigge R. Yalcinday A. Sundel R. Aggressive Management of Juvenile Dermatomyositis results in improved outcome and decreased incidence in calcinosis. Am Acad Dermatol. 2002;47:505-11.

6. Mebazaa A, Boussen H, Nouira R, Rokbani L, Ben Osman-Dhahri A, Bouaouina N, et al. Dermatomyositis and Malignancy in Tunisia: a multicenter retrospective study of 20 cases. J Am Acad Dermatoly. 2003; 48:530-534.

7. Gerami P, Walling H.W, Lewis J, Doughty L, Sontheimer R.D. A systematic review of juvenile onset clinically amyopathic dermatomyositis. British journal of Dermatology. 2007; 157: 637-644.

8. Lorizzo L, Jorizzo J. The treatment and prognosis of dermatomyositis: an updated review. J AM Acad Dermatolol 2008; 59: 99-112.

9. Lowdry C, Pilkington C. Juvenile Dermatomoysitis: extramuscular manifestations and their management. Curr Opin Rheumatol 2009; 21: 575-580

10. Robinson A, palmer-Thierry M, Gibson K, Rabinovich C. Disease Activity, Proteinuria, and Vitamin D Status in Children with Systemic Lupus Erythematosus and Juvenile Dermatomyositis. J Pediatr. 2012 Feb; 160 (2):297-302.

11. Tzaribachev N, Well C, Schnedel J, Horger M. Whole body MRI: a helpful diagnostic tool for juvenile dermatomyositis case report and review of the literature. Rheumatol Int. 2009; 29: 1511-1514.

12. McCann L, Juggins A, Maillard S, Wedderburn L, Davidson J, Pilkington C. The juvenile dermatomyositis national registry and repository (UK and Ireland)- clinical characteristics of children recruited within the first year. Rheumatol. 2006; 45:1255-

More Articles

A randomized, double-blind, comparative study on the safety and efficacy of virgin coconut (Cocos nucifera l.) oil against 1% hydrocortisone lotion as an anti-inflammatory and antipruritic preparation for mosquito reactions

  Authors: Uy, Veronica S, MD; Gracia B. Teodosio, MD, FPDS; Ma. Teresita G. Gabriel, MD, FPDS; Mary Catherine T. Galang, MD; Mohammad Yoga A. Waskito, MD; Johannes F. Dayrit, MD, FPDS     Abstract Background: Virgin coconut oil (VCO) has been reported...

read more