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  Table of Contents  
Year : 2020  |  Volume : 11  |  Issue : 3  |  Page : 451-452  

Unilateral limb atrophy: Is it a forme fruste localized scleroderma?

1 Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission17-Jul-2019
Date of Decision01-Sep-2019
Date of Acceptance05-Sep-2019
Date of Web Publication10-May-2020

Correspondence Address:
Pandiarajan Vignesh
Allergy Immunology Unit, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_349_19

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How to cite this article:
Bhattarai D, Vignesh P, Guleria S, Sinha A, Singhal M. Unilateral limb atrophy: Is it a forme fruste localized scleroderma?. Indian Dermatol Online J 2020;11:451-2

How to cite this URL:
Bhattarai D, Vignesh P, Guleria S, Sinha A, Singhal M. Unilateral limb atrophy: Is it a forme fruste localized scleroderma?. Indian Dermatol Online J [serial online] 2020 [cited 2021 Oct 22];11:451-2. Available from: https://www.idoj.in/text.asp?2020/11/3/451/276576

Dear Editor,

Unilateral limb atrophy can be an unusual clinical presentation of localized scleroderma in children.[1],[2],[3] We report a case with slowly progressive atrophy of the upper limb with normal neurological findings.

   Case History Top

A 12-year-old boy presented with 4 months history of progressive atrophy of the arm with a paucity of hair in the left upper limb. He also reported fixed flexion deformity of the left little finger leading to inability to extend the finger. There was no muscular or bony pain, muscle fasciculations, skin pigmentation, distal tapering of fingers, or nail changes. For the last 2 months, he had difficulty in picking up heavy objects and had an abducted and pronated posture of the left upper arm. There was mild swelling on dorsal aspect of left wrist. There was no history of trauma, joint pain, joint swelling, ulceration, sensory abnormalities, autonomic disturbances, or any evidence of sclerosis. There was no restriction of movement in the joints. His gait was normal. In addition, family history was noncontributory.

On physical examination, there was an obvious muscle wasting of the entire left upper limb [Figure 1]a and [Figure 1]b and restriction of movement at the wrist and elbow joints. The left little finger was deformed [Figure 1]c. Radiograph of left hand showed periosteal reaction with trabeculations over the distal end of proximal phalanx of the little finger [Figure 1]d. There was weakness of small muscles of the hand without signs of upper or lower motor neuron involvement. Hair density in left forearm was noted to be less as compared to the right side [Figure 1]b. There were no nail changes [Figure 1]c. The neurological examination was unremarkable.
Figure 1: Photographs of (a) both forearms and hands (atrophied left forearm); (b) forearms showing marked atrophy and paucity of hairs on the left side; (c) deformed left little finger; (d) radiograph of left hand showing periosteal reaction with trabeculations over the distal end of proximal phalanx

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Hemogram revealed hemoglobin 110 g/L, total leucocytes 6.3 × 109/L (N50L32M12E6), platelets 343 × 109/L and erythrocyte sedimentation rate 10 mm in the first hour. Electrolytes, renal and liver function tests, muscle enzymes, and lipid profile were normal. Inflammatory markers were not elevated. The nerve conduction velocity test showed normal results. Nail-fold capillaroscopy showed normal findings.

Serum immunoglobulin (Ig) and IgG subclass levels were normal. Antinuclear antibodies, antidouble stranded-deoxyribonucleic acid antibodies, and immunoblot tests were negative.

A chest X-ray did not reveal any evidence of the cervical rib. Computed tomography angiography (CTA) of the upper limb was normal. The X-ray of forearm and wrist revealed periosteal reaction with trabeculations seen all over the distal end of the proximal phalanx and proximal end of the middle phalanx. Ultrasound examination of left limb was normal. Magnetic resonance imaging (MRI) of the forearm, neck, brachial plexus, and pectoral girdle was normal. MRI of the hand showed periosteal reaction and trabeculations over distal end of proximal phalanx of the little finger on the left side.

The case was discussed with international scleroderma experts (through personal email communications). A clinical possibility of deep morphea was thought of due to significant difference in the circumference and bulk of the left limb without any neurological deficit. The paucity of the hairs further strengthened the proposition. However, there were no obvious skin changes. A possibility of progressive monomelic hemiatrophy as a pointer of evolving linear scleroderma was considered wherein skin changes of scleroderma could occur at a later stage.[4] Kobayashi et al. had described one child with solitary morphea profunda in a young girl.[5] Bockle et al. reported a similar case of unilateral atrophy in a patient with localized scleroderma.[3] During early phase of illness, deep morphea may remain asymptomatic without involving internal organs.[4],[6] Sometimes, these patients present with isolated facial or limb hemiatrophy. Blaszczyk et al. reported few such cases with primary atrophic profound linear scleroderma.[6] Our patient had no signs of sclerosis. Deep morphea may not be accompanied by preceding signs of inflammation, sclerosis, or discoloration during early phase.[4] A deep subcutaneous biopsy has been planned during the follow up. This case is highlighted to ignite a discussion whether such subtle atrophic manifestation is the forme fruste of localized scleroderma.

Declaration of patient consent

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

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   References Top

Al-Ghawi E, Al-Harbi T, Al-Sarawi A, Binfalah M. Monomelic amyotrophy with proximal upper limb involvement: A case report. J Med Case Rep 2016;10:54.  Back to cited text no. 1
Yoo SD, Kim HS, Yun DH, Kim DH, Chon J, Lee SA, et al. Monomelic amyotrophy (hirayama disease) with upper motor neuron signs: A case report. Ann Rehabil Med 2015;39:122-7.  Back to cited text no. 2
Bockle BC, Willeit J, Freund M, Sepp NT. Unexplained muscle atrophy as the unique preceding symptom of bilateral linear morphea. J Neurol Neurosurg Psychiatry 2009;80:310-1.  Back to cited text no. 3
Careta MF, Romiti R. Localized scleroderma: Clinical spectrum and therapeutic update. An Bras Dermatol 2015;90:62-73.  Back to cited text no. 4
Kobayashi KA, Lui H, Prendiville JS. Solitary morphea profunda in a 5-year-old girl: Case report and review of the literature. Pediatr Dermatol 1991;8:292-5.  Back to cited text no. 5
Blaszczyk M, Krysicka-Janiger K, Jabłońska S. Primary atrophic profound linear scleroderma. Report of three cases. Dermatology (Basel) 2000;200:63-6.  Back to cited text no. 6


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