Viewpoints in dermatology • Correspondence
Correspondence
Panniculitis in juvenile dermatomyositis doi: 10.1111/ced.12576 Panniculitis is uncommon in children, and when diagnosed, underlying diseases (especially connective tissue disease) should be excluded. We report a 15-year-old patient diagnosed with juvenile dermatomyositis (JDM). Five years previously, the patient had developed an episode of muscular weakness with limited mobility, occasional dysphagia, weight loss, and cutaneous lesions in the malar region and on the dorsa of the hands (Fig. 1a,b). Electromyography of the femoral quadriceps showed a myopathic pattern. Laboratory tests (complete blood count, liver and renal function, thyroid function) were normal except for lactate dehydrogenase (LDH) at 974 IU/L (normal range 130– 250 IU/L) and creatine phosphokinase (CPK) of 1051 IU/L (24–170 IU/L). The patient was negative for anti-doublestranded DNA, antinuclear antibodies, and anti-SM, antiURNP and anti-Jo-1 antibodies, but positive for anti-SSA. Magnetic resonance imaging showed diffuse alteration in signalling in the scapular and pelvic muscles. Changes in the muscle biopsy were compatible with JDM.
The patient was admitted to our clinic with a widespread eruption consisting of tender, painful, nonulcerated subcutaneous nodules, mainly located on the proximal upper and lower extremities, and on the back (Fig. 1c,d). She did not have fever or any associated infectious symptoms, but reported generalized loss of strength and fatigue. Her medications included monthly intravenous methylprednisolone bolus (30 mg/kg), weekly oral methotrexate (10 mg/week), oral folic acid (5 mg/week) and oral hydroxychloroquine (200 mg/24 h). Laboratory tests including complete blood count, acute phase proteins, and liver and renal function were normal except for LDH (547 IU/L) and aldolase (10.9 IU/L; normal range 1.0–7.5 IU/L). Under the suspicion of JDM-associated panniculitis, we took a cutaneous biopsy. In the sections, a subtle vacuolar degeneration of the basal cells was noted at the dermoepidermal junction, and a dense lymphohistiocytic infiltrate with several plasma cells was present in the lobules of the subcutaneous fat, preserving the septum. No vasculitis was observed, and calcification was absent. Periodic-acid–Schiff staining was negative. These findings were consistent with our clinical diagnosis (Fig. 2). (b)
(a)
(c)
(d)
Figure 1 (a) Photodistributed erythema
over the malar region; (b) lichenoid erythemathous papules overlying the knuckles, consistent with Gottron papules; (c,d) tender, erythematous, non-ulcerated nodules on the back and legs.
ª 2015 British Association of Dermatologists
Clinical and Experimental Dermatology
1
Correspondence
(a)
(b)
Figure 2 (a) Lymphohistiocytic inflammatory infiltrate in the lobules of the subcutaneous tissue; (b) no granulomatous structures or
features of vasculitis were observed. Haematoxylin and eosin, original magnification (a) 920; (b) 9100.
The patient was treated with a course of three intravenous methylprednisolone boluses (0.5 mg/kg), oral prednisone (60 mg daily) and oral ciclosporin (150 mg twice daily), which resulted in complete resolution of the panniculitis and the musucular weakness within 4 weeks. Once this episode ceased, dosing of the drugs was tapered, and 12 months later the patient remains stable on oral metrotrexate 7.5 mg/weekly, and oral folic acid (5 mg/ week) and oral hydroxychloroquine (200 mg/24 h). In 1924, Weber and Grey reported the first case of DM-related panniculitis,1 and to our knowledge, only six paediatric patients have been described previously.2 In our institution, a referral paediatric hospital, 21 cases of JDM have been diagnosed in the last 12 years, but this is the first case of JDM-associated panniculitis. DM-related panniculitis consists of a mostly lobular panniculitis occurring concurrently with muscle weakness, which suggests an aetiopathogenetic relationship between the two.3 It is of interest that the striking inflammatory presentation occurred in our case in the setting of a full-dose immunosuppressive treatment, similar to previous reports.4,5 Infection should be carefully excluded in patients prior to upgrading immunosuppressive therapy. Supporting the suggestion of other authors, we believe that JDM-related panniculitis might be underdiagnosed, and it should be included in the differential diagnoses of subcutaneous lesions in patients with JDM.4
2
Clinical and Experimental Dermatology
M.-M. Otero Rivas,1 A. Vicente Villa,1 L. Gonz alez Lara,1 2 3 M. Su~ nol Capella, J. Ant on L opez, and M.-A.Gonz alez Ense~ nat1 1 Dermatology Department, 2Pathology Department and 3Pediatric Rheumatology Department, Hospital Sant Joan de Deu, Passeig de Sant Joan de Deu No. 2-08950, Esplugues de Llobregat, Barcelona, Spain E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 5 August 2014
References 1 Weber FP, Gray AMH. Chronic relapsing polydermatomyositis with predominant involvement of the subcutaneous fat (panniculitis). Br J Dermatol 1924; 36: 544–60. 2 Douvoyiannis M, Litman N, Dulau A, Ilowite NT. Panniculitis, infection, and dermatomyositis: case and literature review. Clin Rheumatol 2009; 28: S57–63. 3 Winkelman WJ, Billick RC, Srolovitz H. Dermatomyositis presenting as panniculitis. J Am Acad Dermatol 1990; 23: 127–8. 4 Ghali FE, Reed AM, Groben PA, McCauliffe DP. Panniculitis in juvenile dermatomyositis. Pediatr Dermatol 1999; 16: 270–2. 5 Neidenbach PJ, Sahn EE, Helton J. Panniculitis in juvenile dermatomyositis. J Am Acad Dermatol 1995; 33: 305–7.
ª 2015 British Association of Dermatologists
Correspondence
Panniculitis in juvenile dermatomyositis doi: 10.1111/ced.12576 Panniculitis is uncommon in children, and when diagnosed, underlying diseases (especially connective tissue disease) should be excluded. We report a 15-year-old patient diagnosed with juvenile dermatomyositis (JDM). Five years previously, the patient had developed an episode of muscular weakness with limited mobility, occasional dysphagia, weight loss, and cutaneous lesions in the malar region and on the dorsa of the hands (Fig. 1a,b). Electromyography of the femoral quadriceps showed a myopathic pattern. Laboratory tests (complete blood count, liver and renal function, thyroid function) were normal except for lactate dehydrogenase (LDH) at 974 IU/L (normal range 130– 250 IU/L) and creatine phosphokinase (CPK) of 1051 IU/L (24–170 IU/L). The patient was negative for anti-doublestranded DNA, antinuclear antibodies, and anti-SM, antiURNP and anti-Jo-1 antibodies, but positive for anti-SSA. Magnetic resonance imaging showed diffuse alteration in signalling in the scapular and pelvic muscles. Changes in the muscle biopsy were compatible with JDM.
The patient was admitted to our clinic with a widespread eruption consisting of tender, painful, nonulcerated subcutaneous nodules, mainly located on the proximal upper and lower extremities, and on the back (Fig. 1c,d). She did not have fever or any associated infectious symptoms, but reported generalized loss of strength and fatigue. Her medications included monthly intravenous methylprednisolone bolus (30 mg/kg), weekly oral methotrexate (10 mg/week), oral folic acid (5 mg/week) and oral hydroxychloroquine (200 mg/24 h). Laboratory tests including complete blood count, acute phase proteins, and liver and renal function were normal except for LDH (547 IU/L) and aldolase (10.9 IU/L; normal range 1.0–7.5 IU/L). Under the suspicion of JDM-associated panniculitis, we took a cutaneous biopsy. In the sections, a subtle vacuolar degeneration of the basal cells was noted at the dermoepidermal junction, and a dense lymphohistiocytic infiltrate with several plasma cells was present in the lobules of the subcutaneous fat, preserving the septum. No vasculitis was observed, and calcification was absent. Periodic-acid–Schiff staining was negative. These findings were consistent with our clinical diagnosis (Fig. 2). (b)
(a)
(c)
(d)
Figure 1 (a) Photodistributed erythema
over the malar region; (b) lichenoid erythemathous papules overlying the knuckles, consistent with Gottron papules; (c,d) tender, erythematous, non-ulcerated nodules on the back and legs.
ª 2015 British Association of Dermatologists
Clinical and Experimental Dermatology
1
Correspondence
(a)
(b)
Figure 2 (a) Lymphohistiocytic inflammatory infiltrate in the lobules of the subcutaneous tissue; (b) no granulomatous structures or
features of vasculitis were observed. Haematoxylin and eosin, original magnification (a) 920; (b) 9100.
The patient was treated with a course of three intravenous methylprednisolone boluses (0.5 mg/kg), oral prednisone (60 mg daily) and oral ciclosporin (150 mg twice daily), which resulted in complete resolution of the panniculitis and the musucular weakness within 4 weeks. Once this episode ceased, dosing of the drugs was tapered, and 12 months later the patient remains stable on oral metrotrexate 7.5 mg/weekly, and oral folic acid (5 mg/ week) and oral hydroxychloroquine (200 mg/24 h). In 1924, Weber and Grey reported the first case of DM-related panniculitis,1 and to our knowledge, only six paediatric patients have been described previously.2 In our institution, a referral paediatric hospital, 21 cases of JDM have been diagnosed in the last 12 years, but this is the first case of JDM-associated panniculitis. DM-related panniculitis consists of a mostly lobular panniculitis occurring concurrently with muscle weakness, which suggests an aetiopathogenetic relationship between the two.3 It is of interest that the striking inflammatory presentation occurred in our case in the setting of a full-dose immunosuppressive treatment, similar to previous reports.4,5 Infection should be carefully excluded in patients prior to upgrading immunosuppressive therapy. Supporting the suggestion of other authors, we believe that JDM-related panniculitis might be underdiagnosed, and it should be included in the differential diagnoses of subcutaneous lesions in patients with JDM.4
2
Clinical and Experimental Dermatology
M.-M. Otero Rivas,1 A. Vicente Villa,1 L. Gonz alez Lara,1 2 3 M. Su~ nol Capella, J. Ant on L opez, and M.-A.Gonz alez Ense~ nat1 1 Dermatology Department, 2Pathology Department and 3Pediatric Rheumatology Department, Hospital Sant Joan de Deu, Passeig de Sant Joan de Deu No. 2-08950, Esplugues de Llobregat, Barcelona, Spain E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 5 August 2014
References 1 Weber FP, Gray AMH. Chronic relapsing polydermatomyositis with predominant involvement of the subcutaneous fat (panniculitis). Br J Dermatol 1924; 36: 544–60. 2 Douvoyiannis M, Litman N, Dulau A, Ilowite NT. Panniculitis, infection, and dermatomyositis: case and literature review. Clin Rheumatol 2009; 28: S57–63. 3 Winkelman WJ, Billick RC, Srolovitz H. Dermatomyositis presenting as panniculitis. J Am Acad Dermatol 1990; 23: 127–8. 4 Ghali FE, Reed AM, Groben PA, McCauliffe DP. Panniculitis in juvenile dermatomyositis. Pediatr Dermatol 1999; 16: 270–2. 5 Neidenbach PJ, Sahn EE, Helton J. Panniculitis in juvenile dermatomyositis. J Am Acad Dermatol 1995; 33: 305–7.
ª 2015 British Association of Dermatologists
