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Challenges in the Diagnosis and Treatment of Disabling Pansclerotic Morphea of Childhood: Case-Based Review

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Challenges in the Diagnosis and Treatment of Disabling Pansclerotic Morphea of Childhood: Case-Based Review Rheumatology International (2019) 39:933–941 Rheumatology https://doi.org/10.1007/s00296-019-04269-w INTERNATIONAL CASE BASED REVIEW Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review Han Jie Soh1 · Courtney Samuel1 · Victoria Heaton1 · William Douglas Renton1 · Angela Cox1 · Jane Munro1,2 Received: 10 January 2019 / Accepted: 28 February 2019 / Published online: 5 March 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Disabling pansclerotic morphea of childhood (DPMC) is a rare subtype of juvenile localized scleroderma (JLS) characterized by pansclerosis mainly affecting children under the age of 14. This aggressive disease has a poor prognosis due to the rapid progression of deep musculoskeletal atrophy resulting in cutaneous ulceration and severe joint contractures. We describe the challenges in treating a previously well 5-year-old male who has refractory symptoms of DPMC. Over the 29 months, since his initial presentation, we trialed over ten therapies. There was subjective improvement with prednisolone and mycophe- nolate mofetil (MMF). However, other therapies including biologics and tyrosine kinase inhibitors (TKI) were ineffective. The patient has been referred for hematopoietic stem cell transplant given ongoing disease progression. We conducted a literature search focusing on English articles with keywords including DPMC. Publications with limited information or describing cases aged 20 and above were excluded. Thirty-seven case reports were identified and the reported treatments were evaluated. Methotrexate and corticosteroids have been the most commonly utilized. MMF has been anecdotally effective. Biologics, TKI, and Janus kinase inhibitors lack evidence in DPMC, but have had demonstrated efficacy in similar patholo- gies including systemic sclerosis, and, thus, have been used for DPMC. Phototherapy has been documented to be reducing skin thickness and stiffness of plaques. Eventually, most children require multi-modal and high-dose immunosuppressive therapies to reduce the inflammation inflicted by the disease. Long-term antibiotics and nutritional support are important in the ongoing care of these patients. Keywords Disabling pansclerotic morphea of childhood · Pediatric · Children · Treatment Introduction * Han Jie Soh Disabling pansclerotic morphea of childhood (DPMC) [email protected] is a rare subtype of juvenile localized scleroderma (JLS) Courtney Samuel characterized by the rapid progression of deep cutaneous [email protected] fibrosis or pansclerosis that involves the subcutaneous Victoria Heaton adipose tissue and, occasionally, the fascia, muscles, and [email protected] bone [1, 2]. It was detailed in 1980 [2] and mainly affects William Douglas Renton children under the age of 14 [3]. Initially, the extremi- [email protected] ties would be involved and the disease would spread to Angela Cox the trunk, face, and scalp, while distal areas are spared [email protected] [2]. DPMC is aggressive and prognosis is usually poor Jane Munro [1]. Complications include severe joint contractures, limb [email protected] discrepancy, articular ankyloses [4, 5], and cutaneous ulceration which often results in septicemia [6]. Apart 1 Paediatric Rheumatology Unit, The Royal Children’s from marked functional and psychological impacts, neo- Hospital, Parkville, VIC, Australia plasia, cardiomyopathy, and restrictive pulmonary disease 2 Arthritis and Rheumatology, Murdoch Children’s Research have also been reported [1, 4, 7–9]. The etiology is still Institute, Parkville, VIC, Australia Vol.:(0123456789)1 3 934 Rheumatology International (2019) 39:933–941 unknown, although vascular damage, increased collagen Case presentation synthesis, and deposition along with altered B- and T-cell production and function may contribute to the cause [5]. A 5-year-old Caucasian male, who was previously well, Laboratory findings are non-specific. The most consist- presented with a 6-month history of generalized pain in ent findings from the previous studies were hypergam- most of his joints, morning stiffness, weight loss, and maglobulinemia and peripheral eosinophilia [2, 4, 10], fatigue. He had been developmentally appropriate, was although hypogammaglobulinemia has also been reported up-to-date with vaccinations, and had no significant fam- [11]. Cytokines including interleukin-6 (IL-6) have been ily history. On examination, he had generalized tender- suggested to play an integral role [12]. Antinuclear anti- ness and had proximal lower limb and truncal weakness. body (ANA) positivity has been reported in some cases His skin over his proximal interphalangeal and metacar- [1, 13]. The values for cryoglobulins, rheumatoid factor, pophalangeal joints was tight and shiny. Mild desquama- and complements are generally within normal ranges [2]. tion and subtle patches of hypopigmentation and hyper- Histological findings have shown inflammatory round cell pigmentation were noticed around his shoulders. Both his infiltrates to various depths, but the blood vessels are usu- knees and wrists had active synovitis. He had mild gen- ally unaffected [2]. Due to the pansclerotic characteristics eralized lymphadenopathy and mild hepatosplenomegaly. of DPMC, an excisional biopsy is recommended over the The initial laboratory investigations are summarized in usual punch or incisional biopsy to evaluate the degree of Table 2. His chest X-ray and electrocardiogram were both inflammation [2]. normal. Bone marrow analysis showed normal tri-lineage Optimal treatment for DPMC is unknown and numerous hematopoiesis with some reactive eosinophils and plasma treatments have been trialed in small numbers of patients cells. The initial magnetic resonance imaging (MRI) of his with the mixed results. Methotrexate (MTX) [14], which is pelvis and lower limbs showed the evidence of extensive the first line for JLS [14–16], is frequently used for DPMC. edema confined to the subcutaneous tissues, joint effusions Corticosteroids [4, 5, 17], mycophenolate mofetil (MMF) throughout his joints, and bilateral inguinal lymphadenopa- [12, 18], and tocilizumab [12] have also been used with thy. There was no evidence of myositis on MRI; however, some success [19]. Other therapies such as phosphodies- Mi-2-alpha myositis-specific antibodies were weakly posi- terase inhibitors, endothelin receptor antagonists, vitamin tive on immunofluorescence (but became negative on sub- D analogues, antimalarials, D-penicillamine, colchicine, sequent testing). Muscle enzymes activities were within and topical psoralen with ultraviolet (UV) light treatment normal range. Single-nucleotide polymorphism (SNP) have been trailed with limited benefit [1, 4, 6, 13, 20–22]. array revealed a single long continuous stretch of homozy- Autologous stem cell transplantation appears to slow the gosity (5.9 Mb) detected on chromosome 7 of unknown progress of new lesions, but is not curative [23]. Most treat- significance. ments described have varied efficacies. We aim to describe Our patient was commenced on regular naproxen, pred- the challenges in treating a refractory patient with DPMC nisolone (1 mg/kg/day), and MTX (15 mg/m2/week) for a and to present a focused review of the literature of the thera- presumed mixed connective tissue disease with the fea- peutic options that have been previously trialed. tures of juvenile idiopathic arthritis, dermatomyositis, and localized scleroderma. Three months after the initial presentation, his synovitis had resolved and his strength improved on these therapies. Search strategy However, he still had progressive skin changes culminating in dyspigmentation and further sclerosis. He also started to We performed a literature search from January 1980 to develop lipodystrophy of the lower limbs. Monthly intrave- June 2018 in PubMed, MEDLINE, EMBASE, Scopus, and nous immunoglobulin (IVIG) (1 g/kg/month) and monthly Web of Science with keywords ‘paediatric/pediatric’, ‘disa- methylprednisolone pulses (30 mg/kg/month) were added bling pansclerotic morphea of childhood’, ‘treatment’, and to the existing treatments. At 7 months, hydroxychloro- ‘management’. Due to the various nomenclatures that have quine (200 mg/dose 3 days a week) was added due to his been previously used to describe DPMC, we streamlined cutaneous changes and incomplete response. our search to focus on articles that include DPMC as key- Due to progressing disease, a skin punch biopsy was words. We included original articles, letters to the editor, and performed 11 months into treatment. This showed hyper- case reports published in English. Publications containing keratosis with epidermal atrophy and diffuse dermal scle- abstracts with limited information, comments, or involving rosis consistent with scleroderma. A follow-up MRI of his case presentations of aged 20 and above were excluded. We whole body showed resolution of the previous diffuse sub- identified 37 case reports and summarized the findings in cutaneous edema and synovitis, although small effusions Table 1. 1 3 Rheumatology International (2019) 39:933–941 935 Table 1 Reported cases of disabling pansclerotic morphea of childhood First author Year Number of Gender Age (years) Treatment Outcome References patients (n) Diaz-Perez 1980 14 10 F; 4 M 1–14 Corticosteroids, cyclophosphamide, 2 (D); 9 (PD); 2 (PR) [2] D-penicillamine, anti-malarial agents Scharfetter-Kochanek 1995 1 1 F 8 PUVA PR [20] Gruss 1997 1 1 M 16 UVA PR [38] Corticosteroids,
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