206 Correspondence 2 Lu D, Patel KA, Duvic M, Jones D. Clinical and pathological spectrum of CD8-positive cutaneous T-cell lymphomas. J Cutan Pathol 2002; 29:465–72. 3 Kempf W, Kazakov DV, Cozzio A et al. Primary cutaneous CD8 (+) small-to medium-sized lymphoproliferative disorder in extrafacial sites: clinicopathologic features and concept on their classification. Am J Dermatopathol 2013; 35:159–66. 4 Beltraminelli H, M€ ullegger R, Cerroni L. Indolent CD8+ lymphoid proliferation of the ear: a phenotypic variant of the smallmedium pleomorphic cutaneous T-cell lymphoma? J Cutan Pathol 2010; 37:81–4. 5 Suchak R, O’Connor S, McNamara C, Robson A. Indolent CD8positive lymphoid proliferation on the face: part of the spectrum of primary cutaneous small-/medium-sized pleomorphic T-cell lymphoma or a distinct entity? J Cutan Pathol 2010; 37:977–81. 6 Li XQ, Zhou XY, Sheng WQ et al. Indolent CD8+ lymphoid proliferation of the ear: a new entity and possible occurrence of signet ring cells. Histopathology 2009; 55:468–70. 7 Petrella T, Maubec E, Cornillet-Lefebvre P et al. Indolent CD8positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma? Am J Surg Pathol 2007; 31:1887–92. 8 Swick BL, Baum CL, Venkat AP, Liu V. Indolent CD8+ lymphoid proliferation of the ear: report of two cases and review of the literature. J Cutan Pathol 2011; 38:209–15. 9 Leinweber B, Beltraminelli H, Kerl H, Cerroni L. Solitary smallto-medium-sized pleomorphic T-cell nodules of undetermined significance: clinical, histopathological, immunohistochemical and molecular analysis of 26 cases. Dermatology 2009; 219:42–7. 10 Khamaysi Z, Ben-Arieh Y, Epelbaum R, Bergman R. Pleomorphic CD8+ small/medium size cutaneous T-cell lymphoma. Am J Dermatopathol 2006; 28:434–7. Funding sources: none. Conflicts of interest: none declared.

Development of chronic inflammatory demyelinating polyneuropathy in a patient receiving infliximab for psoriasis DOI: 10.1111/bjd.12572 DEAR EDITOR, A 49-year-old white man was referred to the Manchester Psoriasis Service with a 21-year history of severe chronic plaque psoriasis refractory to therapy with topical agents, phototherapy (TL-01), systemic agents (methotrexate, ciclosporin and fumaderm) and the biologic agents adalimumab and ustekinumab, including with concurrent ciclosporin therapy. Relevant history included alcohol excess. There was no family history of demyelination. Following a flare of psoriasis in June 2011, treatment was changed from ustekinumab (90 mg dosing regimen) with ciclosporin 2 mg kg 1 daily, to infliximab intravenously (5 mg kg 1 at weeks 0, 2 and 6, and thereafter at 8-week intervals). On initiation of infliximab, ciclosporin was discontinued but reintroduced at 2 mg kg 1 daily due to a flare in December 2011. Two weeks later, the patient reported sudden onset of weakness of his right hand and numbness of the fingertips bilaterBritish Journal of Dermatology (2014) 170, pp200–227

ally. With no objective sensory disturbance and unilateral right upper limb weakness, urgent assessment by a neurologist led to a provisional diagnosis of brachial plexopathy secondary to ciclosporin, which was stopped. Due to the severity of the patient’s psoriasis, infliximab therapy was continued. During January 2012 he reported worsening of sensory disturbance in the arms and new weakness in both legs. Neurological examination revealed reduced tone in the right upper limb, right forearm muscle wasting, right hand weakness in an ulnar and median nerve distribution. Reflexes were present in both arms with no sensory loss. The lower limbs had normal tone. Mild bilateral dorsiflexion weakness was noted. Sensation was normal; all reflexes were absent. Urgent investigations included magnetic resonance imaging of the brain and cervical spine, which was normal. Nerve conduction studies revealed changes compatible with a primary demyelinating neuropathy and met diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Cerebral spinal fluid findings demonstrated no pleocytosis or oligoclonal bands, protein and glucose were within normal parameters; nerve biopsy was not performed. With a working diagnosis of infliximab-associated CIDP, infliximab therapy was discontinued in February 2012 and ciclosporin reinstituted. The patient’s neurological condition deteriorated 3 months from onset of symptoms with marked global limb weakness with distal bias, sensory ataxia and severe neuropathic pain of the feet. Treatment with intravenous immunoglobulin (IVIg) was commenced [150 g over 5 days (04 mg kg 1)], with a second course a month later followed by concomitant oral prednisolone (40 mg daily) after 8 weeks, which resulted in a significant worsening of his condition. Clinical stabilization was achieved with a course of plasma exchange and continued monthly pulses of IVIg. One year after the initial onset of symptoms the patient’s distal limb power has improved, he is able to mobilize with a stick but continues to be profoundly sensory ataxic and IVIg dependent. Following withdrawal of infliximab, the patient’s psoriasis flared and now requires combination treatment with Fumadermâ (Almirall Hermal, Reinbek, Germany) 240 mg three times daily, acitretin 50 mg daily and ciclosporin 50 mg twice daily. CIDP is an acquired demyelinating disease of the peripheral nervous system. It typically presents between the ages of 30 and 60 years and is characterized by progressive symmetrical proximal and distal muscle weakness, sensory dysfunction and impaired balance.1 The course of the disease can be monophasic, relapsing and remitting or progressive.1 Various diagnostic criteria exist, utilizing findings from clinical, electrophysiological and laboratory testing.2 Because demyelination in CIDP is multifocal, clinical and pathological manifestations vary. The reported prevalence of 1–77 per 100 000 in Northern Europe3 may be an underestimation due to difficulty in the identification of ‘atypical’ cases.3 The exact triggers for CIDP are unknown. A number of disorders are associated with CIDP including organ transplantation, inflammatory bowel disease, diabetes, and malignant © 2013 British Association of Dermatologists

© 2013 British Association of Dermatologists

Alshekhlee et al.6

6

7

RA

50 M

Lozeron et al.10

5

49 M

RA

RA

RA

69 M

Solomon et al.9

4

45 F

AS

RA

73 F

Richez et al.5

2

47 M

RA

50 F

Jarand et al.4

1

3

Disease

Case age Author and (years) and sex Case reference

25 years

4 months

17 months

Following five doses of infliximab

Infliximab

Etanercept

1 year

1 week

Adalimumab Unknown; (total exposure worsening 4000 mg) after 2 years

Adalimumab

Infliximab

Infliximab (3 mg kg 1, total exposure 1000 mg) Etanercept

Treatment

Onset of symptoms in relation to treatment

Diagnostic of demyelinating polyneuropathy

Motor nerve conduction studies multifocal demyelinating neuropathy with partial conduction block in three nerves Severe axonal sensorimotor polyneuropathy

Demyelinating polyneuropathy with conduction block Mild neurogenic patterns

Electrophysiology

Lower limb Evidence of acquired weakness with demyelination including bilateral foot multiple conduction drop, severe blocks sensory ataxia, areflexia Numbness in both Evidence of hands after 1 year, progressive progressing to acquired proximal and distal demyelinating muscle weakness and sensory and areflexia after a motor peripheral further year polyneuropathy

Tingling in both hands and feet

Numbness and weakness both hands

Lower limb weakness, distal upper limb dysaesthesia

Lower limb weakness, upper and lower limbs dysaesthesia

Diplopia, left hand weakness and numbness

Symptoms

Antiganglioside

Antiganglioside

P/Q-type voltage-gated calcium channel antibody +ve N/A

Antiganglioside GM2 (IgM) +ve

Antiganglioside GM1 (IgM) +ve

Not performed

Antibody

Table 1 Summary of chronic inflammatory demyelinating polyneuropathy (CIDP) cases reported during tumour necrosis factor inhibitor therapy

ve

ve

Y

Y

Reduced dosage

Y

Y

Y

Y

2 years; remaining numbness in toes 2 years; improvement in activities of daily living IVIg after 2 months and prednisolone 1 mg kg 1; IVIg at 4-month intervals 2 years; some IVIg 2 g kg 1 daily for 5 days, clinical then after 6 improvement, weeks, then walking 8 weeks, thereafter unassisted every 8 weeks

Prednisolone

IVIg then azathioprine and prednisolone

18 months; partial improvement

6 months; neuropathy improved with improved EMG 6 months; mild paraesthesia and abnormal gait





35 months; some improvement

IVIg monthly

Discontinuation Immunosuppressant Follow-up/ of drug therapy outcome

Treatment

Correspondence 207

British Journal of Dermatology (2014) 170, pp200–227

British Journal of Dermatology (2014) 170, pp200–227 RA

Infliximab

Infliximab

Etanercept

Infliximab

10 months

44 months

32 months

31 months

5 months

17 months

8 months

6 weeks

16 months

30 months

Onset of symptoms in relation to treatment

Paraesthesia of all limbs, areflexia Proximal and distal weakness all limbs with paraesthesia Lower limb paraesthesia and ataxia Mild distal weakness lower limbs, distal paraesthesia all limbs Weakness lower limbs, paraesthesia distal upper limbs, diffuse areflexia Mild lower limb distal paraesthesia Paraesthesia all limbs, diffuse areflexia Distal paraesthesia lower limbs Proximal and distal upper and lower limb weakness and areflexia

Ataxia, foot drop, areflexia

Symptoms

Consistent with severe demyelinating neuropathy

Nerve conduction study abnormalities showed typical features of CIDP in eight of nine cases. Nerve conduction abnormalities were conduction block or temporal dispersion in six, reduced motor nerve conduction velocity in six, prolonged F-wave latency in three and distal prolonged latency in three

Electrophysiology

N/A

Antiganglioside antibodies were tested in four patients and were found to be present in three

Antibody

Y

Y

Y

N

Y

Y

Y

Y

Y

Y

IVIg and prednisolone

IVIg

IVIg and prednisolone

None

None

None

IVIg

IVIg

IVIg

IVIg

Complete regression, relapse NB: History of GBS 29 years earlier. 12 months; recovery, treatment discontinued

Stabilization

Stabilization

Stabilization

Stabilization then delayed improvement

Complete regression

Partial improvement, relapse with adalimumab Partial improvement Partial improvement

Discontinuation Immunosuppressant Follow-up/ of drug therapy outcome

Treatment

RA, rheumatoid arthritis; AS, ankylosing spondylitis; IM, inflammatory myositis; N/A, not available; IVIg, intravenous immunoglobulin; EMG, electromyography; GBS, Guillain–Barre syndrome.

Psoriasis Adalimumab

55 F

15

AS

AS

Etanercept

53 F

52 M

14

Ahmed et al.8

47 M

13

RA

Adalimumab

17

45 F

12

RA

Infliximab

IM

39 F

11

AS

66 M

36 M

10

Infliximab

Infliximab

Treatment

16

49 M

9

AS

RA

79 F

8

Seror et al.7

Disease

Case age Author and (years) Case reference and sex

Table 1 (continued)

208 Correspondence

© 2013 British Association of Dermatologists

Correspondence 209

melanoma where it has been hypothesized that carbohydrate epitopes shared by myelin sheath and melanoma act as target antigens. The usual therapeutic options for CIDP include oral corticosteroids, IVIg and plasma exchange. The prognosis is variable, some patients responding to cessation of the tumour necrosis factor (TNF) inhibitor, others requiring ongoing treatment with immunosuppressant therapy. The development of CIDP has been described in 17 patients treated with all TNF inhibitors licensed for use in psoriasis: infliximab, nine;4–7 etanercept, four;5–7 and adalimumab, four,7–10 one of whom received adalimumab for severe psoriasis.8 Our case is consistent with previous reports (Table 1). There are several hypotheses as to how TNF inhibitors may be implicated in the pathogenesis of CIDP, including: TNF inhibitor therapy leading to autoantibody production via reduced regulatory T-cell activity9; or through the removal of the support to peripheral neurons played by constitutive TNF-a.4 However, the mechanistic link is unclear, as TNF inhibitor therapy has not been discontinued7,10 in all such concomitant cases of CIDP, and paradoxically etanercept has been used successfully to treat the condition. The British Association of Dermatologists’ 2009 guidelines for biologic interventions for psoriasis recommend that if symptoms suggestive of demyelination develop during therapy, treatment be withdrawn and specialist advice sought. Reporting of such events via patient registries such as the British Association of Dermatologists’ Biologic Interventions Register is imperative to gain understanding of the role TNF inhibitors may play in the onset of peripheral demyelinating disease.

Acknowledgments A.C.F. is an MRC Clinical Training Fellow supported by the North West England Medical Research Council Fellowship Scheme in Clinical Pharmacology and Therapeutics, which is funded by the Medical Research Council (grant no. G1000417/94909), ICON, GlaxoSmithKline, AstraZeneca and the Medical Evaluation Unit. R.B.W. is an NIHR Senior Clinical Lecturer; C.E.M.G. is an NIHR Senior Investigator. 1

The Dermatology Centre, and Department of Neurology, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, University of Manchester, Manchester M6 8HD, U.K. E-mail: [email protected] 2

A.C. FOULKES1 L. WHEELER1 D. GOSAL2 C.E.M. GRIFFITHS1 R.B. WARREN1

References 1 Dalakas MC. Medscape. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol 2011; 7:507–17. 2 Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of

© 2013 British Association of Dermatologists

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4 5

6

7

8

9

10

Neurological Societies and the Peripheral Nerve Society – first revision. J Peripher Nerv Syst 2010; 15:185–95. Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010; 9:402–12. Jarand J, Zochodne DW, Martin LO et al. Neurological complications of infliximab. J Rheumatol 2006; 33:1018–20. Richez C, Blanco P, Lagueny A et al. Neuropathy resembling CIDP in patients receiving tumor necrosis factor-alpha blockers. Neurology 2005; 64:1468–70. Alshekhlee A, Basiri K, Miles JD et al. Chronic inflammatory demyelinating polyneuropathy associated with tumor necrosis factor-alpha antagonists. Muscle Nerve 2010; 41:723–7. Seror R, Richez C, Sordet C et al. Pattern of demyelination occurring during anti-TNF-a therapy: a French national survey. Rheumatology (Oxford) 2013; 52:868–74. Ahmed Z, Powell R, Llewelyn G et al. Chronic inflammatory demyelinating polyradiculoneuropathy complicating anti TNF a therapy for chronic plaque psoriasis. BMJ Case Rep 2011. doi: 10.1136/bcr.08.2011.4674. Solomon AJ, Spain RI, Kruer MC et al. Inflammatory neurological disease in patients treated with tumor necrosis factor alpha inhibitors. Mult Scler 2011; 17:1472–87. Lozeron P, Denier C, Lacroix C et al. Long-term course of demyelinating neuropathies occurring during tumor necrosis factoralpha-blocker therapy. Arch Neurol 2009; 66:490–7.

Funding sources: None. Conflicts of interest: A.C.F., L.W., D.G., R.B.W. and C.E.M.G. work in the U.K. National Health Service (NHS). C.E.M.G. has acted as a consultant and/or speaker for Abbott, Centocor, Incyte, Janssen, Leo Pharma, Novartis, Pfizer and Schering-Plough (now MSD), all of which manufacture therapies used in the treatment of psoriasis. R.B.W. has acted as a consultant and/or speaker for Abbott, Janssen, Leo Pharma, Pfizer, Novartis and Schering-Plough (now MSD), all of which manufacture therapies used in the treatment of psoriasis. A.C.F. has received educational support to attend conferences and/or acted as a consultant for Pfizer, Abbott, Janssen, Novartis and Leo Pharma, all of which manufacture therapies used in the treatment of psoriasis.

Successful therapy of refractory Hailey–Hailey disease with oral alitretinoin DOI: 10.1111/bjd.12582 DEAR EDITOR, Hailey–Hailey disease (HHD) is an autosomal dominant, acantholytic disorder caused by a loss of function mutation in the ATP2C1 gene leading to the insufficiency of a Golgi-associated Ca2+ transporter. Symptomatic therapy usually controls the disease; however, management can be challenging in recalcitrant cases, thus new and safe therapy options are needed. We describe two patients in whom treatment with oral alitretinoin resulted in near-complete remission. Patient 1 is a 33-year-old woman who presented with inguinal and anogenital HHD. During puberty, intensive local treatment provided symptomatic control. Later, she developed progressive and refractory disease that has persisted for 4 years

British Journal of Dermatology (2014) 170, pp200–227

Development of chronic inflammatory demyelinating polyneuropathy in a patient receiving infliximab for psoriasis.

206 Correspondence 2 Lu D, Patel KA, Duvic M, Jones D. Clinical and pathological spectrum of CD8-positive cutaneous T-cell lymphomas. J Cutan Pathol 2...
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