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Australasian Journal of Dermatology (2015) 56, 224–226
doi: 10.1111/ajd.12172
BRIEF REPORT
Dermatomyositis panniculitis: A case report Melissa Carroll,1 Nick Mellick2 and Godfrey Wagner3 1
Department of Dermatology, Royal Brisbane and Women’s Hospital, 2Zenith Specialist Pathology, and 3 Queensland Institute of Dermatology, Brisbane, Queensland, Australia
ABSTRACT 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 biopsyconfirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.
Dermatomyositis-related panniculitis (DP) manifests as red subcutaneous, firm and tender plaques, usually on the arms, buttocks, thighs and abdomen. They may calcify or form sinuses. DP is commonly described in textbooks yet there are few reports in the medical literature. There are no guidelines on its best management. This report describes our management with prednisone, cyclophosphamide and i.v. immunoglobulin (Ig).
Key words: dermatomyositis, panniculitis.
INTRODUCTION Dermatomyositis is a connective tissue disorder affecting skin, muscle and blood vessels. Classical cutaneous features may include violaceous erythematous flat papules over the interphalangeal and metocarpophalangeal joints (Gottron’s papules), macular violaceous erythema over extensor surfaces of the hands, elbows, knees and ankles (Gottron’s sign), heliotrope rash and photodistributed violaceous erythema of arms, upper back, posterior shoulders and neck (shawl sign) together with dilated nail fold capillaries, ragged cuticles and hyperkeratosis of the ulnar thumb and radial fingers (mechanic’s hands). Other clinical features may include proximal myopathy, dysphagia, dysphonia, interstitial lung disease, respiratory failure, cardiomyopathy, myocarditis and conduction disorders.
Correspondence: Dr Melissa Carroll, Department of Dermatology, Royal Brisbane and Women’s Hospital, Butterfield Street, Herston QLD 4006, Australia. Email: [email protected] Melissa Carroll, MBBS. Nick Mellick, FRCPA. Godfrey Wagner, FACD. Conflict of interest: none. Submitted 4 July 2013; accepted 25 February 2014. © 2014 The Australasian College of Dermatologists
CASE REPORT A 60-year-old woman presented with a 3-week history of hot, indurated, tender plaques on her thighs and buttocks (Fig. 1). The plaques were exquisitely painful on sitting and lying. Two years previously she had been diagnosed with dermatomyositis with typical skin changes, proximal muscle weakness, elevated creatine kinase and confirmed myositis on magnetic resonance imaging and electromyography. Her current treatment was prednisone 10 mg twice daily. Adjuvant treatments had been ceased due to their adverse effects. Two months previously she commenced monthly i.v. Ig infusions 2 g/kg for a dermatomyositis flare. Three weeks previously cyclophosphamide 50 mg daily was added. This was ceased on presentation in case it had triggered the panniculitis. A deep incisional biopsy was performed on the lesional skin. The epidermis was mildly spongiotic with focal areas of lichenoid lymphocytic inflammation at the dermoepidermal junction and clusters of Civatte bodies (Fig. 2). The dermis had a superficial and deep perivascular and interstitial infiltrate of lymphocytes and histiocytes. Small numbers of neutrophils were present. Features in the subcutaneous tissue included the necrosis of fat lobules, scattered neutrophilic infiltrates and basophilic alteration resembling a saponification-like process. There were extensive regions of calcification (Fig. 3). A fresh tissue culture was not performed as an infective process was
Abbreviations: DP Ig
dermatomyositis-related panniculitis immunoglobulin
Dermatomyositis panniculitis
225
a
Figure 2 Mildly spongiotic epidermis, superficial and deep perivascular infiltrate, clusters of Civatte bodies.
b
treatment was continued. A clinical improvement was noted within 10 days with decreased heat and tenderness; however the plaques remained significantly firm to touch. The patient was discharged 2 weeks later on cyclophosphamide 50 mg alternating with 100 mg. Six months later the prednisone was weaned to 7.5 mg twice daily with cyclophosphamide 50 mg twice daily. The plaques were still firm to palpation and uncomfortable for the patient.
DISCUSSION
Figure 1 Exquisitely tender, indurated, hot plaques on the thighs showing (a) side view and (b) front view. Similar plaques were present on the buttocks.
DP was first published in medical literature in 1924.1 A literature review revealed only 23 adult cases worldwide since then and six juvenile cases, including one from Australia.2–8 No adult cases have been reported in Australia. Due to limited reports treatment guidelines have not been established. In nearly 50% of reported cases prednisone was the initial treatment and in 30% of cases it was the only systemic treatment. Responses ranged from a complete resolution at 6 weeks to relapse during the weaning process. Other cases were treated with i.v. methylprednisolone or adjuvant azathioprine, methotrexate, i.v. Ig, hydroxychloroquine or cyclosporin.
CONCLUSION considered unlikely. A diagnosis was made of lobular panniculitis occurring in a setting of dermatomyositis. Prednisone was increased to 25 mg twice daily and weaned to 10 mg twice daily over 10 days after the recommencement of cyclophosphamide 50 mg daily. I.v. Ig
DP is a rare manifestation of dermatomyositis. We describe an effective treatment of it with prednisone, cyclophosphamide and i.v. Ig in an acute presentation. Further reports may assist in improving the recognition and treatment of this condition. © 2014 The Australasian College of Dermatologists
226
a
M Carroll et al.
b
Figure 3 (a) Necrosis of fat lobules in the subcutis with degenerated neutrophils, apoptotic nuclei and calcium deposition and (b) high-power view.
REFERENCES 1.
2.
3.
4.
Weber FP, Gray AM. Chronic relapsing polydermatomyositis with predominant involvement of the subcutaneous fat. Br. J. Dermatol. 1924; 36: 544–60. Solans R, Cortes J, Selva A et al. Panniculitis: a cutaneous manifestation of dermatomyositis. J. Am. Acad. Dermatol. 2002; 46: S148–150. Suwa A, Hirakata M, Hama N et al. An adult case of dermatomyositis complicated with cecum perforation and panniculitis. Nihon Rinsho Meneki Gakkai Kaishi 1997; 20: 60–6. Laraki R, Genestie C, Wechsler J et al. Juvenile dermatomyositis and panniculitis-type subcutaneous T-cell
© 2014 The Australasian College of Dermatologists
5.
6.
7.
8.
lymphoma. A case report. Rev. Med. Interne 2001; 22: 978–83. Basanovic´ J, Brasanac D, Zivanovic´ D et al. Rare forms of panniculitis: subcutaneous sarcoidosis and panniculitis associated with dermatomyositis. Vojnosanit.Pregl. 2004; 61: 211–5. Abdul-Wahab A, Holden CA, Harland C et al. Calcific panniculitis in adult-onset dermatomyositis. Clin. Exp. Dermatol. 2009; 34: e854–856. Arias M, Hernández MI, Cunha LG et al. Panniculitis in a patient with dermatomyositis. An. Bras. Dermatol. 2011; 86: 146–8. Girouard SD, Velez NF, Penson RT et al. Panniculitis associated with dermatomyositis and recurrent ovarian cancer. Arch. Dermatol. 2012; 148: 740–4.
Australasian Journal of Dermatology (2015) 56, 224–226
doi: 10.1111/ajd.12172
BRIEF REPORT
Dermatomyositis panniculitis: A case report Melissa Carroll,1 Nick Mellick2 and Godfrey Wagner3 1
Department of Dermatology, Royal Brisbane and Women’s Hospital, 2Zenith Specialist Pathology, and 3 Queensland Institute of Dermatology, Brisbane, Queensland, Australia
ABSTRACT 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 biopsyconfirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.
Dermatomyositis-related panniculitis (DP) manifests as red subcutaneous, firm and tender plaques, usually on the arms, buttocks, thighs and abdomen. They may calcify or form sinuses. DP is commonly described in textbooks yet there are few reports in the medical literature. There are no guidelines on its best management. This report describes our management with prednisone, cyclophosphamide and i.v. immunoglobulin (Ig).
Key words: dermatomyositis, panniculitis.
INTRODUCTION Dermatomyositis is a connective tissue disorder affecting skin, muscle and blood vessels. Classical cutaneous features may include violaceous erythematous flat papules over the interphalangeal and metocarpophalangeal joints (Gottron’s papules), macular violaceous erythema over extensor surfaces of the hands, elbows, knees and ankles (Gottron’s sign), heliotrope rash and photodistributed violaceous erythema of arms, upper back, posterior shoulders and neck (shawl sign) together with dilated nail fold capillaries, ragged cuticles and hyperkeratosis of the ulnar thumb and radial fingers (mechanic’s hands). Other clinical features may include proximal myopathy, dysphagia, dysphonia, interstitial lung disease, respiratory failure, cardiomyopathy, myocarditis and conduction disorders.
Correspondence: Dr Melissa Carroll, Department of Dermatology, Royal Brisbane and Women’s Hospital, Butterfield Street, Herston QLD 4006, Australia. Email: [email protected] Melissa Carroll, MBBS. Nick Mellick, FRCPA. Godfrey Wagner, FACD. Conflict of interest: none. Submitted 4 July 2013; accepted 25 February 2014. © 2014 The Australasian College of Dermatologists
CASE REPORT A 60-year-old woman presented with a 3-week history of hot, indurated, tender plaques on her thighs and buttocks (Fig. 1). The plaques were exquisitely painful on sitting and lying. Two years previously she had been diagnosed with dermatomyositis with typical skin changes, proximal muscle weakness, elevated creatine kinase and confirmed myositis on magnetic resonance imaging and electromyography. Her current treatment was prednisone 10 mg twice daily. Adjuvant treatments had been ceased due to their adverse effects. Two months previously she commenced monthly i.v. Ig infusions 2 g/kg for a dermatomyositis flare. Three weeks previously cyclophosphamide 50 mg daily was added. This was ceased on presentation in case it had triggered the panniculitis. A deep incisional biopsy was performed on the lesional skin. The epidermis was mildly spongiotic with focal areas of lichenoid lymphocytic inflammation at the dermoepidermal junction and clusters of Civatte bodies (Fig. 2). The dermis had a superficial and deep perivascular and interstitial infiltrate of lymphocytes and histiocytes. Small numbers of neutrophils were present. Features in the subcutaneous tissue included the necrosis of fat lobules, scattered neutrophilic infiltrates and basophilic alteration resembling a saponification-like process. There were extensive regions of calcification (Fig. 3). A fresh tissue culture was not performed as an infective process was
Abbreviations: DP Ig
dermatomyositis-related panniculitis immunoglobulin
Dermatomyositis panniculitis
225
a
Figure 2 Mildly spongiotic epidermis, superficial and deep perivascular infiltrate, clusters of Civatte bodies.
b
treatment was continued. A clinical improvement was noted within 10 days with decreased heat and tenderness; however the plaques remained significantly firm to touch. The patient was discharged 2 weeks later on cyclophosphamide 50 mg alternating with 100 mg. Six months later the prednisone was weaned to 7.5 mg twice daily with cyclophosphamide 50 mg twice daily. The plaques were still firm to palpation and uncomfortable for the patient.
DISCUSSION
Figure 1 Exquisitely tender, indurated, hot plaques on the thighs showing (a) side view and (b) front view. Similar plaques were present on the buttocks.
DP was first published in medical literature in 1924.1 A literature review revealed only 23 adult cases worldwide since then and six juvenile cases, including one from Australia.2–8 No adult cases have been reported in Australia. Due to limited reports treatment guidelines have not been established. In nearly 50% of reported cases prednisone was the initial treatment and in 30% of cases it was the only systemic treatment. Responses ranged from a complete resolution at 6 weeks to relapse during the weaning process. Other cases were treated with i.v. methylprednisolone or adjuvant azathioprine, methotrexate, i.v. Ig, hydroxychloroquine or cyclosporin.
CONCLUSION considered unlikely. A diagnosis was made of lobular panniculitis occurring in a setting of dermatomyositis. Prednisone was increased to 25 mg twice daily and weaned to 10 mg twice daily over 10 days after the recommencement of cyclophosphamide 50 mg daily. I.v. Ig
DP is a rare manifestation of dermatomyositis. We describe an effective treatment of it with prednisone, cyclophosphamide and i.v. Ig in an acute presentation. Further reports may assist in improving the recognition and treatment of this condition. © 2014 The Australasian College of Dermatologists
226
a
M Carroll et al.
b
Figure 3 (a) Necrosis of fat lobules in the subcutis with degenerated neutrophils, apoptotic nuclei and calcium deposition and (b) high-power view.
REFERENCES 1.
2.
3.
4.
Weber FP, Gray AM. Chronic relapsing polydermatomyositis with predominant involvement of the subcutaneous fat. Br. J. Dermatol. 1924; 36: 544–60. Solans R, Cortes J, Selva A et al. Panniculitis: a cutaneous manifestation of dermatomyositis. J. Am. Acad. Dermatol. 2002; 46: S148–150. Suwa A, Hirakata M, Hama N et al. An adult case of dermatomyositis complicated with cecum perforation and panniculitis. Nihon Rinsho Meneki Gakkai Kaishi 1997; 20: 60–6. Laraki R, Genestie C, Wechsler J et al. Juvenile dermatomyositis and panniculitis-type subcutaneous T-cell
© 2014 The Australasian College of Dermatologists
5.
6.
7.
8.
lymphoma. A case report. Rev. Med. Interne 2001; 22: 978–83. Basanovic´ J, Brasanac D, Zivanovic´ D et al. Rare forms of panniculitis: subcutaneous sarcoidosis and panniculitis associated with dermatomyositis. Vojnosanit.Pregl. 2004; 61: 211–5. Abdul-Wahab A, Holden CA, Harland C et al. Calcific panniculitis in adult-onset dermatomyositis. Clin. Exp. Dermatol. 2009; 34: e854–856. Arias M, Hernández MI, Cunha LG et al. Panniculitis in a patient with dermatomyositis. An. Bras. Dermatol. 2011; 86: 146–8. Girouard SD, Velez NF, Penson RT et al. Panniculitis associated with dermatomyositis and recurrent ovarian cancer. Arch. Dermatol. 2012; 148: 740–4.
