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CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 4  |  Page : 333-335

Muscle weakness in a 4-year-old girl: A rare presentation of deep morphea


Department of Dermatology, St John's Medical College Hospital, Bengaluru, Karnataka, India

Date of Submission06-Apr-2020
Date of Decision07-May-2020
Date of Acceptance01-Jun-2021
Date of Web Publication01-Oct-2021

Correspondence Address:
Sumedha Ballal
Department of Dermatology, St. John's Medical College Hospital, Sarjapur Road, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_55_20

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  Abstract 


Introduction: Morphea is a rare fibrosing disorder of the skin and underlying tissues. Deep morphea involves the deep dermis, subcutis, fascia, muscle, and bone. The above structures may be involved independently or in combination. Case report: We describe a case of deep morphea presenting as muscle weakness, independent of skin lesions, in a child with generalized morphea. Discussion: Muscle weakness, even in the absence of overlying or progressive skin sclerosis, can be deep morphea, especially when segmental and associated with atrophy.

Keywords: Muscle weakness, scleroderma localized, atrophy, collagen, child


How to cite this article:
Augustine M, Ballal S. Muscle weakness in a 4-year-old girl: A rare presentation of deep morphea. Indian J Paediatr Dermatol 2021;22:333-5

How to cite this URL:
Augustine M, Ballal S. Muscle weakness in a 4-year-old girl: A rare presentation of deep morphea. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Dec 6];22:333-5. Available from: https://www.ijpd.in/text.asp?2021/22/4/333/327454




  Introduction Top


Morphea (localized scleroderma [LS]) is a rare fibrosing disorder of the skin and underlying tissues. Pediatric morphea has an incidence of 2.7 per 100,000 children per year; more prevalent in females, with a mean age of onset between between six and nine years.[1]

According to the Pediatric Rheumatology European Society (PReS), there are five subtypes of morphea: linear morphea (LM), circumscribed morphea (CM), mixed morphea (MM), generalized morphea (GM), and pansclerotic morphea (PM); LM is the most frequent subtype, followed by PM, MM, GM, and DM.[2]


  Case Report Top


A four-year-old girl presented to a neurologist with weakness of the left shoulder of eight months duration. There was no history of any systemic medication, recent illness, intramuscular injections, or trauma. The patient had received immunizations appropriate for age, and there was no history of recent immunization. Examination revealed atrophy of the shoulder muscles with a muscle power of 2/5. Differential diagnoses considered were focal myopathies and brachial plexus injury. The patient had a history of hyperpigmented patches on the body for 2 years, which were not progressive at the time of the muscle weakness or after that. She was treated with topical Vitamin D3 analogs for the same. Dermatological examination showed multiple atrophic, indurated, hyperpigmented plaques over the trunk [Figure 1], neck [Figure 2], and limbs with linear lesions over the left forearm and left foot [Figure 3]. The skin overlying the affected muscles was, however, normal. The general physical examination was noncontributory. Nerve conduction studies were normal. Magnetic resonance imaging (MRI) did not reveal any cord pathology. However, MRI of the muscles showed fatty atrophy of the supraspinatus, infraspinatus, and posterior part of deltoid and distal fibers of biceps on the left side. Biopsy of the patches revealed dense homogenized bands of collagen in the dermis with perivascular and periadnexal lymphocytic infiltrate, consistent with morphea [Figure 4]. Routine investigations showed a hemoglobin of 12.9 g/dl, total leukocyte count of 7.29 × 103/μl (50% neutrophils, 44% lymphocytes, 4% eosinophils, 2% monocytes), platelet count of 365 × 103/μl, normocytic normochromic peripheral smear with relative neutrophilia, and an elevated erythrocyte sedimentation rate of 43 mm/h. A mild elevation of aspartate aminotransferase (49 U/L) with normal alanine aminotransferase (23 U/L) and alkaline phosphatase (203 U/L) was noted in liver function tests. Serum lactate dehydrogenase was mildly elevated (286 U/L) but serum creatinine kinase was normal (147 U/L). Antinuclear antibodies (ANAs) were detected, in a homogenous pattern. Moderate positivity to anti-double-stranded DNA (ds DNA) and anti-histone (AHA) antibodies was noted. With the skin lesions of morphea and the MRI findings of muscle atrophy in the absence of nerve pathology, a diagnosis of mixed morphea was made. The child was given a course of steroids (1 mg/kg body weight, for three months). She was also started on hydroxychloroquine and methotrexate (10 mg/week), along with physiotherapy. After year, the skin lesions have softened, and the muscle power has improved from 2/5 to 4/5.
Figure 1: Hyperpigmented indurated atrophic plaques over the trunk

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Figure 2: Hyperpigmented indurated atrophic plaques over the neck

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Figure 3: Linear hyperpigmented atrophic plaque over the dorsum of the left foot

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Figure 4: Histopathology demonstrating homogenization of collagen in the dermis along with a perivascular and periadnexal lymphocytic infiltrate, consistent with morphea [Hematoxylin and Eosin x40]

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  Discussion Top


Clinical forms of morphea according to the PReS classification include LM, CM, MM, GM, and PM.[2]

Our patient is a case of MM with linear and circumscribed lesions, also fulfilling the generalized category criteria.

In morphea, muscle involvement can precede skin manifestations or/and may occur at sites unrelated to the skin lesions. Böckle et al. reported an adult presenting with weakness of the extensors of the left upper limb, followed by linear induration of the right hand and forearm, three years later, suggestive of LM.[3] On the contrary, Voermans et al. reported a young adult who continued to have progressive muscle weakness three years after the skin disease became inactive, which suggests that disease activity of the skin may not correspond to that of the muscles.[4]

In our case, the skin disease was nonprogressive at the onset of muscle weakness, and the skin overlying the affected muscles was clinically normal.

MRI of the muscles showed fatty atrophy of the muscle bellies of the left shoulder girdle, consistent with deep morphea. Given the age of the patient, invasive tests such as electromyography and muscle biopsy were deferred. MRI offers the advantages of being noninvasive and readily repeatable. MRI shows a wide range of muscle abnormalities in morphea, including fascial thickening around muscle groups, muscle inflammation, muscle atrophy, and fat infiltration.[5] These changes may be seen even at sites distant from the skin lesions.[5] MRI has detected musculoskeletal involvement in morphea, even when clinically not suspected or apparent. A recent study by Abbas et al., on comparison of MRI and clinical assessment using modified localized scleroderma skin severity index (mLOSSI) in deep morphea, showed that MRI revealed clinically occult muscle involvement, and mLOSSI scores did not correspond to features of activity on MRI.[6] This highlights the inaccuracy of classifying lesions as inactive, using the mLOSSI alone, particularly in deep morphea.[1] Muscle biopsy, in addition to being invasive, also has a risk of sampling error. Electromyography can be used to detect spontaneous activity to evaluate the effect of treatment. Ultrasound and MRI are useful in monitoring disease progression and selecting optimal site for biopsy.[7]

These patients may have personal or family history of autoimmune diseases and multiple autoantibodies, especially ANA, AHA, and anti-single-stranded antibody (ss-DNA). ANA is associated with early disease and extracutaneous involvement, whereas higher titers of AHA and ss-DNA correlate with severe disease.[8]

Our patient had active disease (progressive deep tissue involvement; documented by MRI), and moderately high levels of AHA and ds DNA, all of which warrant systemic treatment.[8],[9],[10] Early systemic treatment may prevent end-stage myopathy and contractures.

Muscle weakness, even in the absence of overlying or progressive skin sclerosis, can be seen in deep morphea, especially when segmental and associated with atrophy. This can easily mimic several neuromuscular disorders. MRI of the muscles is a reliable noninvasive test and should be considered if there is no evidence of a neurological cause.

Acknowledgment

We would like to acknowledge Dr. Thomas Mathew, Professor and Head, Department of Neurology, St John's Medical College Hospital, Bangalore.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
George R, George A, Kumar TS. Update on management of morphea (localized scleroderma) in children. Indian Dermatol Online J 2020;11:135-45.  Back to cited text no. 1
  [Full text]  
2.
Aranegui B, Jiménez-Reyes J. Morphea in childhood: An update. Actas Dermosifiliogr (Engl Ed) 2018;109:312-22.  Back to cited text no. 2
    
3.
Böckle BC, Willeit J, Freund M, Sepp NT. Unexplained muscle atrophy as the unique preceding symptom of bilateral linear morphea. J Neurol Neurosurg Psychiatry 2009;80:310-11.  Back to cited text no. 3
    
4.
Voermans NC, Pillen S, de Jong EM, Creemers MC, Lammens M, van Alfen N. Morphea profunda presenting as a neuromuscular mimic. J Clin Neuromuscul Dis 2008;9:407-14.  Back to cited text no. 4
    
5.
Eutsler EP, Horton DB, Epelman M, Finkel T, Averill LW. Musculoskeletal MRI findings of juvenile localized scleroderma. Pediatr Radiol 2017;47:442-9.  Back to cited text no. 5
    
6.
Abbas LF, O'Brien JC, Goldman S, Pezeshk P, Chalian M, Chhabra A, et al. A cross-sectional comparison of magnetic resonance imaging findings and clinical assessment in patients with morphea. JAMA Dermatol 2020;156:590-2.  Back to cited text no. 6
    
7.
Schanz S, Fierlbeck G, Ulmer A, Schmalzing M, Kümmerle-Deschner J, Claussen CD, et al. Localized scleroderma: MR findings and clinical features. Radiology 2011;260:817-24.  Back to cited text no. 7
    
8.
Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, et al. Development of consensus treatment plans for juvenile localized scleroderma: A roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken) 2012;64:1175-85.  Back to cited text no. 8
    
9.
Zulian F, Culpo R, Sperotto F, Anton J, Avcin T, Baildam EM, et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis 2019;78:1019-24.  Back to cited text no. 9
    
10.
Constantin T, Foeldvari I, Pain CE, Pálinkás A, Höger P, Moll M, et al. Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr 2018;177:961-77.  Back to cited text no. 10
    


    Figures

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



 

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