|Year : 2021 | Volume
| Issue : 4 | Page : 346-348
Lipoatrophic linear morphea in a 5-year-old girl: A novel variant?
Vibhu Mendiratta1, Mansi Bansal1, Shivangi Rana1, Kiran Agarwal2
1 Department of Dermatology, Lady Hardinge Medical College, New Delhi, India
2 Department of Pathology, Lady Hardinge Medical College, New Delhi, India
|Date of Submission||07-May-2020|
|Date of Decision||22-May-2020|
|Date of Acceptance||30-Mar-2021|
|Date of Web Publication||01-Oct-2021|
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Source of Support: None, Conflict of Interest: None
Introduction: Morphea profunda or subcutaneous morphea is a variant of morphea in which inflammation and sclerosis found in deep dermis, subcutaneous tissue, fascia or muscle. Case report: We are report an atypical case of subcutaneous morphea in five year-old girl, presenting with atrophy of subcutaneous fat over the left lower limb in a liner pattern, closely mimicking linear lipoatrophy. Discussion: Early intervention in such cases can halt progression of the disease.
Keywords: Scleroderma localized, subcutaneous fat, child, female, lipodystrophy
|How to cite this article:|
Mendiratta V, Bansal M, Rana S, Agarwal K. Lipoatrophic linear morphea in a 5-year-old girl: A novel variant?. Indian J Paediatr Dermatol 2021;22:346-8
|How to cite this URL:|
Mendiratta V, Bansal M, Rana S, Agarwal K. Lipoatrophic linear morphea in a 5-year-old girl: A novel variant?. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Nov 28];22:346-8. Available from: https://www.ijpd.in/text.asp?2021/22/4/346/327458
| Introduction|| |
Morphea encompasses a group of conditions characterized by varying degrees of sclerosis, fibrosis, and atrophy in the skin and subcutaneous tissue. Morphea profunda is a variant with inflammation and sclerosis found in the deep dermis, subcutaneous tissue, fascia, or muscle.
We report an unusual presentation of morphea, which mimicked localized lipoatrophy in a linear pattern.
| Case Report|| |
A five-year-old girl presented with asymptomatic hollowing of soft tissue which began over the posterior aspect of left ankle that progressed to involve the calf, thigh, and buttock. There was a history of single intramuscular injection in left buttock for fever (nature unknown) 1 month prior, however, no lesion appeared at the site of injection. There was no history of muscle weakness or joint pain or any other systemic involvement.
Examination revealed multiple foci of lipoatrophy with prominent cutaneous veins over posterior aspect of left ankle, extending to calf, knee, and the left thigh with extension to lateral aspect of left thigh, with the presence of normal skip areas over the middle thigh. Another circular localized lipoatrophy was noted measuring 1 cm × 1 cm, with mild hyperpigmentation and tethering of skin over the left buttock [Figure 1]a, [Figure 1]b, [Figure 1]c.
|Figure 1: (a-c) Linear lipoatrophy on left lower limb with guttering of superficial veins and skip areas in between|
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There was shortening of the left lower limb and reduced girth of the left calf by 1 cm. Muscle power and gait were normal.
All laboratory investigations, including hematologic and biochemical tests, were within normal limits. Antinuclear and anticentromere antibody levels were negative. Complement levels, muscle enzymes, and hormone profile were normal. Borrelia serology was non-reactive.
Magnetic resonance imaging (MRI) of left knee showed loss of subcutaneous fat with normal underlying muscle, fascia, and bone. The thickness of subcutaneous fat as seen on ultrasound at upper part of legs at the time of presentation was 1.13 cm (left) versus 2.7 cm (right).
The deep incisional biopsy from left thigh showed epidermis with orthokeratosis and focal basal cell vacuolization. Superficial dermis showed mild perivascular lymphocytic inflammatory infiltrate. Lower dermis showed homogenization of collagen and pulling up of appendages. There was a loss of subcutaneous fat [Figure 2] and [Figure 3]. Based on clinical appearance and histopathology, a final diagnosis of linear lipoatrophic morphea was made, and the patient was started on intravenous methylprednisolone pulses, oral methotrexate, and hydroxychloroquine sulfate.
|Figure 2: H and E, ×40: Showing loss of subcutaneous fat. Inset, H and E, ×400: homogenization of collagen|
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|Figure 3: H and E × 40: Showing homogenization of collagen with loss of subcutaneous fat|
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After receiving 12 pulses of methylprednisolone and daily doses of hydroxychloroquine and weekly methotrexate (7.5 mg), there was halt in progression of disease with improvement in limb girth. After 12 months of treatment, thickness was 1.74 cm (left) versus 2.9 cm (right) as compared to the previous values.
| Discussion|| |
Morphea comprises a group of related conditions characterized by increased collagen deposition in an autoimmune setting. There is an early inflammatory phase followed by sclerotic and then atrophic phase. It is largely confined to skin, subcutaneous tissues and can extend to involve the underlying fat, fascia, muscle, bone, and joints. The etiology of morphea is unknown, however, infection with Borrelia, genetic susceptibility, trauma, and drugs including bromocriptine and L-tryptophan may trigger the disease onset. The pathogenesis of morphea involves three major components: vascular changes, activated T-cells, and activation of fibroblasts. Even though there is no increased mortality, significant morbidity can occur as a result of facial and limb asymmetry, flexion contractures, extracutaneous manifestations, and psychological disability. Early initiation of treatment during the active inflammatory stage is the most crucial step in the management.
Morphea profunda is a variant with minimal or no cutaneous changes. Inflammation and sclerosis are found in deep dermis, subcutaneous tissue, fascia, or muscle. Most of the patients present with a single fibrotic plaque located over the back, shoulder, and neck or paraspinal area involving the skin and deeper tissues. Plaques are poorly circumscribed and symmetrical, without skin changes, mimicking lipodystrophy with skin bound down to deeper tissues.
Clinically morphea profunda can mimic acquired lipoatrophy and eosinophilic fasciitis, which can be differentiated on histology. Various atypical presentations of morphea profunda have been reported in the literature. In a case report by Khelifa et al., there were multiple asymptomatic atrophic lesions in the left arm, at the site of previous intramuscular vaccination with involvement of deltoid muscle. Kobayashi et al. reported a solitary sclerotic plaque of morphea profunda on the back of a five-year-old girl. Giri et al. described a case with multiple noninflammatory atrophic lesions without significant skin induration, pigmentation, and texture change, with histology suggestive of morphea profunda. Voermans et al. reported linear scleroderma of the right arm with progressive muscle weakness and atrophy with loss of function. Blaszczyk et al. reported three cases of primary atrophic scleroderma presenting in linear and band-like pattern with involvement of muscles.
In our case, the patient presented with sudden onset loss of subcutaneous fat over the left lower limb in a linear pattern. Due to the absence of any skin changes, lipoatrophy was considered as a differential diagnosis. However, the presence of homogenization of collagen in the deep dermis confirmed the diagnosis of subcutaneous morphea. Linear pattern of morphea is a well-known entity which presents with epidermal changes in the form of flattening of rete ridges and acanthosis. These epidermal changes were not seen in our patient. Blaszczyk et al. had used a new term “primary atrophic profound linear scleroderma” for such presentation, which is differentiated from subcutaneous morphea by a distinct band-like distribution, primary atrophies, and less pronounced disabling deformities.
All the previously reported cases of linear scleroderma with lipoatrophy also showed involvement of underlying muscle on histopathology and electromyography (EMG). EMG could not be performed in our case, however, MRI showed no involvement of underlying muscle. Muscle involvement leads to limitation of movements, disabilities, and joint contractures. Early detection and treatment can prevent complications.
In our area of practice (North India), Borrelial infections are not very frequently seen, incidence of Borrelia infection is not that common. Hence, we did not start the patient on procaine penicillin. We started her on methylprednisolone as biopsy showed perivascular lymphocytic infiltrate.
Clinical improvement observed in our patient suggests that early management of such patients can halt progression of disease and can further improve the prognosis.
We are reporting this case of morphea for its unusual clinical presentation in a linear pattern and favorable response to immunosuppressive therapy.
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.
We thank the parents of the girl described for allowing us to share her details.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Azad J, Dawn G, Shaffrali FC, Holmes SC, Barnetson RJ, Forsyth A. Does solitary morphoea profunda progress? Clin Exp Dermatol 2004;29:25-7.
Khelifa E, Masouyé I, Chavaz P, Hauser H, Grillet JP, Borradori L. Primary atrophic solitary morphea profunda. Dermatology 2008;217:207-10.
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.
Giri S, Paudel U, Jha A, Gurung D, Parajuli S. Morphoea profunda presenting with atrophic skin lesions in a 26 year old female: A case report. Clin Med Insights Case Rep 2012;5:57-62.
Voermans NC, Pillen S, de Jong EM, Creemers MC, Lammens M, van Alfen N. Morphea profundal presenting as a neuromuscular mimic. J Clin Neuromuscular Dis 2008;9:407-14.
Blaszczyk M, Krysicka-Janiger K, Jabłońska S. Primary atrophic profound linear scleroderma. Report of three cases. Dermatology 2000;200:63-6.
[Figure 1], [Figure 2], [Figure 3]