Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.     

Pancreatic panniculitis in active systemic lupus erythematosus

This report documents the case of a 64‐year‐old African‐American female with new end‐stage renal disease (ESRD), diagnosed with systemic lupus erythematosus (SLE) on renal biopsy and serologies including a positive ANA (>1:2560), positive anti‐Sm antibodies, low titer anti‐RNP antibodies, high titer anti‐Ro antibodies, anti‐dsDNA antibodies, lupus anticoagulant, and hypocomplementemia. She was also noted to have tender nodules on the bilateral shins. Excisional biopsy of one of the nodules showed marked fat necrosis with “ghost cells” and patchy basophilic granular debris consistent with pancreatic panniculitis. Further examination for pancreatic pathology showed an elevated lipase of 585 U/L (reference range 8‐78 U/L) and amylase of 214 U/L (reference range 25‐125 U/L). However, computed tomography imaging showed no evidence of pancreatitis or pancreatic tumors. This is very similar to another case recently reported in the literature. Similarities of these two cases (African‐American females with lupus nephritis on dialysis) may represent a particular subset of SLE patients at increased risk for pancreatic panniculitis.     

Linear morphea with overlying lichen sclerosus and calcinosis cutis associated with juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is associated with many distinguishing features including cutaneous calcinosis, vasculitis, and ulcerated lesions. In this case, we describe an unusual presentation in a 12-year-old girl who had muscle weakness along with linear morphea over the right upper and lower extremities with overlying lichen sclerosus and calcinosis cutis. Of interest, these initial cutaneous manifestations occurred years before onset of myositis.     

Dermatomyositis panniculitis: A case report

Dermatomyositis‐related panniculitis is a rare cutaneous manifestation of dermatomyositis. There are few reported cases in the medical literature. We present the case of a 60‐year‐old woman with a 2‐year history of dermatomyositis and recent biopsy‐confirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.     

Deep morphea

Deep morphea encompasses a variety of clinical entities in which inflammation and sclerosis are found in the deep dermis, panniculus, fascia, or superficial muscle. Morphea profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are included in this group, but overlapping of the extent and depth of cutaneous involvement in these various conditions precludes their distinction on the sole basis of clinical or even histologic examination. Furthermore, the limits between morphea profunda and generalized morphea, which usually are classified outside this group, are not clear. Histologically, all these disorders show similar inflammatory and sclerotic findings, the primary difference being the depth of these changes. Associated clinical findings, including arthralgias, arthritis, contractures, or carpal tunnel syndrome, are frequent. Although visceral complications are uncommon, pulmonary, esophageal, and even cardiac abnormalities have been reported. Eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate may be present with disease activity. Laboratory studies may demonstrate autoantibody production. Treatment is nonstandardized but UVA irradiation and antiinflammatory or immunosuppressive drugs (mainly antimalarial agents and corticosteroids) may be beneficial.     

Facets of lupus erythematosus: panniculitis responding to thalidomide

Lupus erythematosus profundus or lupus panniculitis is a rare clinical variant of lupus erythematosus, which involves the deep dermis and subcutaneous fat. Diagnosis may be difficult in cases with isolated involvement. Further manifestations of lupus erythematosus may thus be essential for diagnosis, which depends on the clinical picture, histopathology and a positive lesional lupus band test. We report a severe, mutilating case of lupus panniculitis, which responded well to thalidomide.     

Three cases of bullous morphea: histopathologic findings with implications regarding pathogenesis

Bullous morphea is a rare variant and is not frequently reported. We present three cases of bullous morphea. Although lymphangiectases have been suggested as the most likely mechanism for the development of the bullae in cases of morphea, none of the cases presented with lymphangiectases. To the contrary, all of our cases showed hemorrhagic content in the bullae, which suggests local trauma as a mechanism involved in bulla formation.     

Palindromic morphea: multiple recurrence of morphea lesions in a case of systemic sclerosis.

We report an unusual scleroderma case. A 45-year-old woman had recurrent morphea lesions over 10 times in 6 years. She had had preceding inactive systemic scleroderma. New morphea lesions developed cyclically on various portions of her body and improved within 2 years. Interestingly, new lesions have developed on once involved skin as well as uninvolved skin. No exacerbation of systemic scleroderma was induced by outbreaks of new morphea lesions. We could not find a similar case in the literature and named it "Palindromic morphea" because of its unique clinical course. D-penicillamine treatment had a limited effect. Minimal oral prednisolone (5 mg/day) completely suppressed the multiple recurrence of the morphea lesions and enhanced improvement of the sclerosis.     

Perineural inflammation in morphea (localized scleroderma): systematic characterization of a poorly recognized but potentially useful histopathological feature

The association between morphea and perineural inflammation has been reported sporadically but never studied systematically. To assess the prevalence and nature of perineural inflammation in various clinicopathologic stages of morphea and a cohort of other inflammatory dermatoses, 80 morphea and 36 control skin biopsies were studied using hematoxylin/eosin and S100 stains. Perineural inflammation was semiquantitatively analyzed (scored), which along with the pattern (concentric vs. marginal) and cellular composition was compared in the two groups. Perineural inflammation was identified in 84% and 61% of morphea and control cases, respectively. Examination of only routinely stained sections could still detect this feature in 58% of morphea and 33% of control biopsies. Mean perineural inflammation score in morphea (0.65) was significantly higher than in the control group (0.23) (p < 0.0001) and the inflammation tended to show a concentric pattern with plasma cell neurotropism. Intraneural inflammation was limited to four morphea cases. Although perineural inflammation is common in morphea, it is not unusual to find this feature in other inflammatory conditions. Nevertheless, perineural inflammation can serve as an important diagnostic adjunct in difficult cases of morphea if one considers its greater intensity, predominantly concentric pattern and the tendency to show plasma cell neurotropism.     

Presenting characteristics and progression of pediatric-onset morphea: Interim analysis of a prospective registry

Morphea is a rare fibrosing disorder with a highly variable disease course, which can complicate management. Here, we present a prospective cohort study describing the current treatments used in the management of pediatric-onset morphea and assessing responses to systemic and topical therapies. Most patients demonstrated inactive disease by 1 year, regardless of treatment, though recurrences were common in our cohort overall (39%). Our results support the need for continuous monitoring of all children with morphea following the completion of treatment, including topical treatment, due to high rates of disease relapse.     

Adiponectin expression is decreased in the involved skin and sera of diffuse cutaneous scleroderma patients

In this study, we determined the adiponectin expression in the serum and lesional skin of patients with scleroderma (SSc). Serum adiponectin concentrations were measured in 32 patients with SSc, 10 patients with SLE, 12 patients with dermatomyositis patients and 13 healthy subjects with specific enzyme-linked immunosorbent assays. Adiponectin mRNA was determined in skin tissues of five patients with diffuse cutaneous SSc (dcSSc), seven patients with limited cutaneous SSc (lcSSc) and seven healthy subjects with real-time polymerase chain reaction. There was a significant reduction in serum adiponectin levels in patients with dcSSc. SSc patients with decreased serum adiponectin levels had higher total skin thickness score and higher incidence of pulmonary fibrosis. Adiponectin mRNA levels in skin tissues from patients with dcSSc were also reduced. Serum adiponectin levels may be a useful biomarker for fibrotic condition in patients with SSc. Clarifying the role of adiponectin in collagen diseases may lead to further understanding of the pathogenesis and new therapeutic approach.     

Discordance of pediatric morphea treatment by pediatric dermatologists

Background/Objectives

Studies describing treatment efficacy in pediatric morphea are lacking. Subspecialists have reached no consensus on how to optimally treat pediatric morphea. The objective of the current study was to describe the most common treatment practices of pediatric dermatologists worldwide who care for children with morphea.

Methods

A survey regarding topical treatment practices of pediatric morphea, with representative case‐based scenarios, was distributed to pediatric dermatologists and results were tallied.

Results

The survey response rate was 13.4%, with 110 respondents in the final analysis. The majority of respondents agreed on red violaceous rim (99%), increased local warmth (75%), raised borders (69%), and dermal thickening (64%) as signs of disease activity. Respondents had less agreement on sclerotic lesions (41%), scaling (43%), dyspigmentation (19%), and atrophy (13%) as signs of disease activity. Ninety‐two percent of respondents used primary therapy or monotherapy with topical medications, including 45% in linear morphea of the limbs and 37% in linear morphea of the head or neck. High‐potency topical corticosteroids were most commonly used (80%), although respondents did not agree on specific regimens. Sixteen different treatment regimens were selected as first‐line therapy for one case scenario of active disease.

Conclusion

The survey found large variation in how pediatric dermatologists treat pediatric morphea. Consensus treatment guidelines developed by pediatric dermatologists and pediatric rheumatologists are urgently needed regarding the efficacy of therapies for pediatric morphea. Prospective studies of treatment efficacy in pediatric morphea are urgently needed as well.     

以脂膜炎为皮肤表现的皮肌炎1例

报告1例以脂膜炎为皮肤表现的皮肌炎。患者男,20岁。14岁起四肢近端肌力下降,伴面部红斑。1年后大腿出现疼痛性结节,伴吞吐咽困难。组织病理学检查示小叶性脂膜炎伴淋巴细胞,浆细胞浸润。经参剂量糖皮质激素及环磷酰胺、静脉滴性丙种球蛋白治疗后肌炎及脂膜炎病情均缓解,提示脂腊炎可能是炎症性肌病的一个皮肤特征。     

Orthopedic complications of linear morphea: Implications for early interdisciplinary care

Linear morphea of the limb primarily affects children, and extracutaneous manifestations are common. Orthopedic surgeons are often essential in the care of patients with linear morphea, yet there are few reports outlining specific orthopedic complications in this population. We sought to improve the understanding of orthopedic complications in linear morphea of the limb. Between 1999 and 2014, 51 children were evaluated for linear morphea of an extremity. Twenty‐six (51%) had documented orthopedic manifestations. Outcome measures included limb length discrepancy, angular malalignment, limb atrophy, and orthopedic surgical intervention. Joint contractures were most common, affecting 88% of patients, followed by limb atrophy, angular deformity, and limb length discrepancy; 14% required surgical intervention. Despite the use of systemic immunosuppressive therapy in many patients, approximately half of patients with linear morphea of an extremity have orthopedic disease. Early orthopedist involvement is crucial to improve limb alignment and preserve function.     

Effectiveness of LPG® treatment in morphea

BACKGROUND: The LPG technique, also known as Endermology treatment, is a noninvasive technique consisting of a tissue mobilization process in which a skin fold is created between two rollers, stretching the underlying tissue and mobilizing the fold. The LPG technique is very effective in treating scars. Because the lesions of morphea or circumscribed scleroderma are similar to atrophic scars, it seemed reasonable to treat them with a method proven helpful for scars. MATERIALS AND METHODS: We treated 17 lesions of 10 patients (four males and six females) with the diagnosis of morphea ranging in age from 17 to 78 years (mean age 55 years) and investigated and documented the evolution of their lesions and changes in their quality of life. RESULTS: In all patients there was a large improvement in the clinical appearance of the lesions, the induration and the pain. Elasticity was particularly increased, not only based on clinical findings but also as documented with objective assessment. The acceptability of the treatment was good and the patients reported an improved quality of life. CONCLUSION: The LPG technique (Endermology) is an adjunctive treatment for morphea. It cannot eliminate the disease but can relieve the pain, soften the skin and improve the quality of life for these patients.     

Connective tissue panniculitis in a child with vitiligo and Hashimoto's thyroiditis

SUMMARY A 9-year-old girl presented with a 6-month history of inflamed tender nodules in the pretibial area. These eventually healed leaving depressed areas of atrophy and loss of subcutaneous tissue. Histology showed a predominantly lymphocytic lobular panniculitis, consistent with connective tissue panniculitis. Investigations revealed an elevated thyroid stimulating hormone, elevated thyroid antiperoxidase antibody and a weakly positive antinuclear antibody (titre 1 in 40). She was commenced on hydroxychloroquine 300 mg daily, which resulted in resolution of the panniculitis. She developed focal vitiligo on the thighs. This gradually improved with 0.1% mometasone furoate ointment. The hydroxychloroquine dose was tapered to 200 mg daily after 12 months, then to 100 mg daily after 18 months therapy. Her thyroid autoantibody levels continued to rise and the hydroxychloroquine was increased again to 300 mg daily. She became borderline hypothyroid. Hashimoto's thyroiditis was diagnosed. Thyroxine was instituted with a resultant improvement in her thyroid blood tests. The lipoatrophy has not developed further during 2-year follow up.     

A case of cytophagic histiocytic panniculitis with sicca symptoms and lupus nephritis.

A 48-year-old Japanese woman presented with many subcutaneous nodules. The skin was purplish in color and tender; the nodules were scattered over the entire surface. Histological findings of biopsy specimens from the nodules indicated septal panniculitis comprised of histiocyte and/or macrophage infiltrates, often with erythro- and/or leukophagocytosis. Phagocytic cells were OKM1 (CD11b), MT1 (CD43), LeuM3 (CD14), and histiocyte antigen positive, indicating the presence of histiocytes and/or macrophages. The patient had sicca symptoms, positive homogenous, speckled pattern ANA (x320), and diffuse proliferative lupus nephritis.