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NOTALGIA PARESTHETICA.

Introduction

Notalgia paresthetica (NP) is a common, yet seldom reported condition characterized by chronic pruritus in the interscapular and paravertebral region with periodic remissions and exacerbations. The disease was first described by the Russian neurologist Astwazaturow in 1934. Analogous to meralgia paresthetica or cheiralgia paresthetica, the name of the disorder is derived from the ancient Greek words noton, "back" and algia, "pain" (13).

The disease primarily affects adults, more frequently women than men (4), however, some hereditary cases have been reported in younger patients (even 6-year-old children) when it is associated with multiple endocrine neoplasia type 2A (MEN 2A) (5).

Etiology

The exact etiology of NP still has not been fully elucidated, however, it is widely accepted that NP is a sensory neuropathy caused by alteration and damage to the cutaneous branches of the dorsal primary rami of thoracic spinal nerves, most commonly T2 through T6. The posterior rami of spinal nerves T2 through T6 are anatomically unique in that they pass through the multifidus spinae muscle at a right-angle (90-degree) course en route to the epidermis, and therefore may be more susceptible to injury, which is why Massey and Pleet, as well as Pecina et al. suggested nerve entrapment as the underlying cause of NP (Fig. 1) (2,3). The damage to spinal nerves may be caused by impingement from degenerative changes in the spine (for example, osteoarthritic changes, kyphosis, vertebral hyperostosis) or compression by muscle fibers (6-8). Unfortunately, typically there is no strong correlation between spinal pathology demonstrated on imaging studies and the dermatomal localization of symptoms in patients with NP (9).

Furthermore, increased dermal innervation, various viscerocutaneous reflex mechanisms, neurotoxicity of certain chemicals and hereditary susceptibility to peripheral neuropathy have also been implicated in the pathophysiology of NP (2,6,10-13). In patients with MEN 2A, the etiology appears to differ and an association with the codon 634 RET mutation has been reported in the literature (5,7).

Clinical Presentation

Patients with NP typically present with intermittent, paroxysmal pruritus, which tends to vary in intensity, and is often accompanied by pain, hyperesthesia and various paresthesias including burning, as well as cold sensation, tingling, surface numbness, tenderness and foreign body sensation. These sensations are usually felt medially or inferior to the scapula and lateral to the thoracic spine. The disorder is typically unilateral, however, interscapular and bilateral distribution of symptoms has also been reported (6,14). There are usually no known triggering or alleviating factors, although one review in the literature suggests that patients with MEN 2A demonstrate amelioration of symptoms following exposure to sunlight (7).

A typical dermatologic finding is hyperpigmentation of the affected area with irregular and indistinct borders with occasional keratotic or atrophic surfaces; it has been reported in two-thirds of published cases in the literature (Fig. 2) (6). This skin manifestation is believed to be a result of chronic scratching and rubbing or, as other authors have suggested, a consequence of neurogenic release of substance P into the skin, which not only leads to pruritus, but also causes proliferation of epidermal cells, arterial smooth muscle cells and fibroblasts (15,16).

Diagnosis

The diagnosis of NP is most frequently made on the basis of reported symptoms and specific area affected. Physical examination and detailed medical history regarding vertebral and thoracic trauma, degenerative disorders or malignancies, vertebral fracture, cervical disc disease, osteoarthritis and familial history should be obtained, while in certain cases imaging methods may be considered (4,6,7,17). Since NP can be an early manifestation of MEN 2A, in younger patients a blood test to check the level of calcitonin should be performed as a screening method for medullary thyroid carcinoma (6,7,18).

In addition, other more common pruritic diseases such as pigmented contact dermatitis, pityrosporum folliculitis, parapsoriasis, neurodermatitis and primitive cutaneous amyloidosis should be excluded prior to diagnosing a patient with NP, which is commonly a clinical diagnosis of exclusion (6).

Treatment

Management of NP is difficult and still presents a clinical challenge, as the condition is often resistant to multiple therapies. Just as the pathogenesis of NP remains unclear, there is no well-defined treatment, although numerous treatment modalities have been reported, with variable results. Conventional antipruritic therapies such as antihistamines and topical cortico-steroids do not address the neuropathic origin of NP and show poor effect (6).

Potential treatment includes topical anesthetics, capsaicin and tacrolimus, intralesional corticosteroids, cutaneous botulinum toxin type A injections, gaba-pentin, oxcarbazepine, amitriptyline, surgical decompression, paravertebral local anesthetic blocks, transcu-taneous electrical nerve stimulation (TENS), electrical muscle stimulation (EMS), exposure to narrow band UV-B radiation, spinal manipulation, physical therapy applications, osteopathic manipulative treatment and acupuncture (Table 1). Most of these therapeutic options have been described in a small number of patients and with varying degrees of success.

Conclusion

There are fewer than 100 cases of NP described in the scientific literature to date. Although it is not a severe disorder per se, the lack of efficient treatment and understanding of the condition greatly affects patient quality of life. Therefore, further research regarding the underlying pathophysiology is of great importance in order to appropriately manage and treat NP patients.

References

(1.) Ellis C. Notalgia paresthetica: the unreachable itch. Dermatol Pract Concept. 2013;3(1):3-6, http://dx.doi.org/10.5826/dpc.0301a02

(2.) Massey EW, Pleet AB. Notalgia paresthetica. JAMA. 1979; 241(14):1464.

(3.) Pecina MM, Krmpotic-Nemanic J, Markiewitz AD. Tunnel Syndromes: Peripheral Nerve Compression Syndromes. 3rd edn. Boca Raton, London, New York, Washington D.C.: CRC Press; 2001. p. 167-70.

(4.) Chiriac A, Podoleanu C, Moldovan C, Stolnicu S. Notalgia paresthetica, a clinical series and review. Pain Pract. 2016; 16(5):E90-1, http://dx.doi.org/10.1111/papr.12436

(5.) Alcantara F, Feito M, Albizuri F, Beato M, De Lucas R. Notalgia paresthetica and multiple endocrine neoplasia syndrome 2a: a case report. Pediatr Dermatol. 2016;33(5):e303-5, http://dx.doi.org/10.1111/pde.12907

(6.) Raison-Peyron N, Meunier L, Acevedo M, Meynadier J. Notalgia paresthetica: clinical, physiopathological and therapeutic aspects. A study of 12 cases. J Eur Acad Dermatol Venereol. 1999;12(3):215-21.

(7.) Shumway NK, Cole E, Fernandez KH. Neurocutaneous disease: neurocutaneous dysesthesias. J Am Acad Dermatol. 2016;74(2):215-28, http://dx.doi.org/10.1016/j.jaad.2015.04.059

(8.) Savk E, Savk O, Bolukbasi O, Culhaci N, Dikicioglu E, Kara-man G, Sendur N. Notalgia paresthetica: a study on pathogenesis. Int J Dermatol. 2000;39(10):754-9.

(9.) Huesmann T, Cunha PR, Osada N, Huesmann M, Zanelato TP, Phan NQ, Gontijo GM, Marziniak M, Stander S. Notalgia paraesthetica: a descriptive two-cohort study of 65 patients from Brazil and Germany. Acta Derm Venereol. 2012;92 (5):535-40, http://dx.doi.org/10.2340/00015555-1344

(10.) Springall DR, Karanth SS, Kirkham N, Darley CR, Polak JM. Symptoms of notalgia paresthetica may be explained by increased dermal innervation. J Invest Dermatol. 1991;97(3): 555-61.

(11.) Eisenberg E, Barmeir E, Bergman R. Notalgia paresthetica associated with nerve root impingement. J Am Acad Dermatol. 1997;37(6):998-1000.

(12.) Savk O, Savk E. Investigation of spinal pathology in notalgia paresthetica. J Am Acad Dermatol. 2005;52(6):1085-7, http://dx.doi.org/10.1016/j.jaad.2005.01.138

(13.) Perez-Perez L, Allegue F, Fabeiro JM, Caeiro JL, Zulaica A. Notalgia paresthesica successfully treated with narrow-band UVB: report of five cases. J Eur Acad Dermatol Venereol. 2010;24(6):730-2, http://dx.doi.org/10.1111/j.1468-3083.2009.03479.x

(14.) Shin J, Kim YC. Neuropathic itch of the back: a case of notalgia paresthetica. Ann Dermatol. 2014;26(3):392-4, http://dx.doi.org/10.5021/ad.2014.26.3.392

(15.) Wang CK, Gowda A, Barad M, Mackey SC, Carroll IR. Serratus muscle stimulation effectively treats notalgia paresthetica caused by long thoracic nerve dysfunction: a case series. J Brachial Plex Peripher Nerve Inj. 2009;4:17, http://dx.doi.org/10.1186/1749-7221-4-17

(16.) Tanaka T, Danno K, Ikai K, Imamura S. Effects of substance P and substance K on the growth of cultured keratinocytes. J Invest Dermatol. 1988;90(3):399-401.

(17.) Alai NN, Skinner HB, Nabili ST, Jeffes E, Shahrokni S, Saemi AM. Notalgia paresthetica associated with cervical spinal stenosis and cervicothoracic disk disease at C4 through C7. Cutis. 2010;85(2):77-81.

(18.) Perez-Perez LC. General features and treatment of notalgia paresthetica. Skinmed. 2011;9(6):353-8.

(19.) Wallengren J, Klinker M. Successful treatment of notalgia pares-thetica with topical capsaicin: vehicle-controlled, double-blind, crossover study. J Am Acad Dermatol. 1995;32(2 Pt 1):287-9.

(20.) Andersen HH, Sand C, Elberling J. Considerable variability in the efficacy of 8% capsaicin topical patches in the treatment of chronic pruritus in 3 patients with notalgia paresthetica. Ann Dermatol. 2016;28(1):86-9, http://dx.doi.org/10.5021/ad.2016.28.L86

(21.) Ochi H, Tan LX, Tey HL. Notalgia paresthetica: treatment with topical tacrolimus. J Eur Acad Dermatol Venereol. 2016; 30(3):452-4, http://dx.doi.org/10.1111/jdv.12830

(22.) Layton AM, Cotterill JA. Notalgia paraesthetica--report of three cases and their treatment. Clin Exp Dermatol. 1991; 16(3):197-8.

(23.) Perez-Perez L, Garcia-Gavin J, Allegue F, Caeiro JL, Fabeiro JM, Zulaica A. Notalgia paresthetica: treatment using intrader-mal botulinum toxin A. Actas Dermosifiliogr. 2014;105(1): 74-7, http://dx.doi.org/10.1016/j.adengl.2013.09.003

(24.) Weinfeld PK. Successful treatment of notalgia paresthetica with botulinum toxin type A. Arch Dermatol. 2007;143(8): 980-2, http://dx.doi.org/10.1001/archderm.143.8.980

(25.) Maari C, Marchessault P, Bissonnette R. Treatment of notalgia paresthetica with botulinum toxin A: a double-blind randomized controlled trial. J Am Acad Dermatol. 2014;70 (6):1139-41, http://dx.doi.org/10.1016/j.jaad.2013.12.006

(26.) Weber PJ, Poulos EG. Notalgia paresthetica. Case reports and histologic appraisal. J Am Acad Dermatol. 1988;18(1 Pt 1):25-30.

(27.) Savk E, Bolukbasi O, Akyol A, Karaman G. Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica. J Am Acad Dermatol. 2001;45(4):630-2, http://dx.doi.org/10.1067/mjd.2001.116228

(28.) Maciel AA, Cunha PR, Laraia IO, Trevisan F. Efficacy of gabapentin in the improvement of pruritus and quality of life of patients with notalgia paresthetica. An Bras Dermatol. 2014;89(4):570-5, http://dx.doi.org/10.1590/abd1806-4841.20142777

(29.) Loosemore MP, Bordeaux JS, Bernhard JD. Gabapentin treatment for notalgia paresthetica, a common isolated peripheral sensory neuropathy. J Eur Acad Dermatol Venereol. 2007;21(10):1440-1, http://dx.doi.org/10.1111/j.1468-3083.2007.02256.x

(30.) Yeo B, Tey HL. Effective treatment of notalgia paresthetica with amitriptyline. J Dermatol. 2013;40(6):505-6, http://dx.doi.org/10.1111/1346-8138.12154

(31.) Williams EH, Rosson GD, Elsamanoudi I, Dellon AL. Surgical decompression for notalgia paresthetica: a case report. Microsurgery. 2010;30(1):70-2, http://dx.doi.org/10.1002/micr.20702

(32.) Goulden V, Toomey PJ, Highet AS. Successful treatment of notalgia paresthetica with a paravertebral local anesthetic block. J Am Acad Dermatol. 1998;38(1):114-6.

(33.) Savk E, Savk O, Sendur F. Transcutaneous electrical nerve stimulation offers partial relief in notalgia paresthetica patients with a relevant spinal pathology. J Dermatol. 2007;34(5):315-9, http://dx.doi.org/10.1111/j.1346-8138.2007.00279.x

(34.) Richardson BS, Way BV, Speece AJ 3rd. Osteopathic manipulative treatment in the management of notalgia paresthetica. J Am Osteopath Assoc. 2009;109(11):605-8.

(35.) Stellon A. Neurogenic pruritus: an unrecognised problem? A retrospective case series of treatment by acupuncture. Acupunct Med. 2002;20(4):186-90.

(36.) Fleischer AB, Meade TJ, Fleischer AB. Notalgia paresthetica: successful treatment with exercises. Acta Derm Venereol. 2011; 91(3):356-7, http://dx.doi.org/10.2340/00015555-1039

Sazetak

NOTALGIA PARESTHETICA

M. Situm, M. Kolic, N. Franceschi i M. Pecina

Notalgia paresthetica je ucestalo, iako slabo prepoznato stanje koje obiljezava lokalizirani kronicni pruritus gornjeg dijela leda, a najcesce se javlja u zena srednje zivotne dobi. Uz svrbez u bolesnika se takoder moze javiti osjecaj zarenja ili hladnoce, trnci, utrnulost, osjetljivost i osjecaj stranog tijela. Uz to, na mjestu simptoma cesto se javlja podrucje hiperpigmentirane koze. Iako postoji vise hipoteza, etiologija ove bolesti slabo je poznata. Opce je prihvaceno da je notalgia paresthetica senzorna neuropatija uzrokovana ostecenjem straznjih ogranaka torakalnih kraljeznicnih zivaca od T2 do T6. Unatoc mnogim razli-citim terapijskim metodama koje su bile promjenjivoga uspjeha i najcesce pruzale privremeno olaksanje, do danas nema uspjesnoga lijecenja ove bolesti.

Kljucne rijeci: Pruritus; Notalgia, bolesti; Parestezija; Hiperestezija

Mirna Situm (1), Maja Kolic (1), Nika Franceschi (1) and Marko Pecina (2)

(1) Department of Dermatology and Venereology, Sestre milosrdnice University Hospital Center, Zagreb, Croatia; (2) Department of Orthopedic Surgery, School of Medicine, University of Zagreb, Zagreb, Croatia

Correspondence to: Prof. Mirna Situm, MD, PhD, Department of Dermatology and Venereology, Sestre milosrdnice University Hospital Center, Vinogradska c. 29, HR-10000 Zagreb, Croatia

E-mail: mirna.situm@kbcsm.hr

Received December 27, 2017, accepted September 10, 2018

doi: 10.20471/acc.2018.57.04.14
Table 1. Treatment alternatives for notalgia paresthetica

Topical

Capsaicin (19,20)     0.025% cream 5 x daily for 1 week, followed by 3 x
                      daily for 5 weeks: 70% of patients reported
                      improvement, however, most experienced relapse
                      within a month after treatment; 8% patches: 3
                      patients experienced immediate relief of itch,
                      duration varied considerably
Tacrolimus (21)       0.1% ointment twice daily: reduction in both
                      intensity and frequency of itch was reported in 5
                      of 7 patients after 6 weeks of treatment, a
                      tolerable burning sensation was the only side
                      effect reported, symptoms generally returned after
                      discontinuation of treatment
Anesthetics (22)      Lidocaine and prilocaine cream under occlusion 2 x
                      daily: complete resolution of symptoms in 2 out of
                      3 patients, the effect was not maintained after
                      stopping treatment
Intralesional
Botulinum             4 U per superficial injection, spaced 2 cm apart:
toxin A (23-25)       16 U in one patient, 72 U in another patient led
                      to resolution of pruritus and decrease of
                      hyperpigmentation for over 18 months; another
                      study in 5 patients and later a randomized,
                      placebo-controlled, double-blind clinical trial
                      in 20 patients failed to replicate these results
Corticosteroids (26)  Triamcinolone 2.5 mg/mL: success in treatment of
                      2 patients
Oral
Oxcarbazepine (27)    6-month treatment, initial dose of 300 mg twice
                      daily and increased to 600 mg twice daily or 900
                      mg twice daily to achieve adequate relief:
                      alleviation of symptoms in 4 out of 5 patients, 1
                      patient withdrew due to side effects (dizziness
                      and headache)
Gabapentin (28,29)    Initiated at a dose of 300 mg at night and
                      increased to 600 mg at night: resulted in absolute
                      resolution of pruritus in 1 patient, after
                      discontinuation of treatment, pruritus returned;
                      300 mg/day for 4 weeks: improvement in itching
                      observed in all patients, side effects (mild
                      gastric discomfort, dizziness) were well tolerated
Amitriptyline (30)    10 mg daily for 9 months: satisfactory reduction
                      of pruritus in 1 patient, sustained for at least 1
                      month after stopping treatment
Other
Surgery (31)          Surgical decompression of the cutaneous nerve:
                      resolution of pruritus for at least 4 months in 1
                      patient
Nerve block (32)      Local anesthetic block in 1 patient using 5 mL of
                      0.75% bupivacaine combined with 40 mg of
                      methylprednisolone acetate achieved disappearance
                      of symptoms for over 1 year
TENS (33)             5 sessions a week for 2 weeks, 50-100 Hz, 20 min
                      duration, 40-75 [micro]s pulse width: mostly mild
                      amelioration of itch was attained in a group of
                      15 patients
EMS (15)              30 seconds on and 30 seconds off for 15 minutes
                      twice daily, 70 Hz with a pulse width of 300
                      [micro]s: relief of pain beginning within days
                      upon starting EMS, recurrence of pain after
                      discontinuation of stimulation for a prolonged
                      period of time, maintenance of analgesic effects
                      with only intermittent stimulation in 4 cases of
                      NP with injury to the long thoracic nerve
Narrow band           Mean of 32.8 sessions and mean cumulative dose of
UV-B (13)             33.76 J/[cm.sup.2]: relevant improvement, even
                      resolution (in 2 out of 5 patients) of pruritus
                      was achieved for at least 6 months
Osteopathic           20-min session, suboccipital decompression, muscle
manipulative          energy, inhibition and other soft tissue
treatment (34)        techniques, fascia release: improvement of
                      symptoms in 1 patient
Acupuncture (35)      Deep intramuscular stimulation acupuncture to the
                      paravertebral muscles in the affected area, 2-6
                      treatments: in 16 patients, absolute or partial
                      alleviation of pruritus was observed, relapse
                      occurred within 1-12 months
Exercise (36)         Stretching and strengthening exercises that
                      elongate the spine and strengthen postural
                      muscles: resolution of symptoms in 2 patients
Physiotherapy (6)     Spinal and paraspinal physiotherapy (ultrasound,
                      radiation, multimodal physiotherapy):
                      4 of 6 patients showed improvement of symptoms
                      that lasted for 1-9 years

TENS = transcutaneous electrical nerve stimulation; EMS = electrical
muscle stimulation
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Author:Situm, Mirna; Kolic, Maja; Franceschi, Nika; Pecina, Marko
Publication:Acta Clinica Croatica
Date:Dec 1, 2018
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