Diagnose It: Itchy, Bumpy Lesions

NEWS/ QUIZ

2 MIN QUIZ

A 45-year-old male visits his nephrologist and presents symptoms of numerous bumpy lesions on his arms, legs and back. The lesions are extremely itchy, and the patient has been frustrated by the lack of sleep and level of burning and stinging from lesions that will not seem to heal and seem to be spreading to other parts of his body. The patient reports increased anxiety due to disrupted sleep patterns and intense cycles of severe itching. After an evaluation, the patient is found to have ^~80 individual nodules. The patient is currently on treatment for chronic kidney disease (CKD), type 2 diabetes mellitus and hypertension. Even though the patient has successfully managed CKD with medication with few complications, he is worried that these lesions are related, and permanent, which has added to his anxiety.

What is the diagnosis?

Select the correct answer

A) Pemphigoid Nodularis

B) Prurigo Nodularis

C) Epidermolysis Bullosa

D) Hypertrophic Lichen Planus

E) Neurotic Excoriations

CORRECT

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INCORRECT

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Answer is: B

Patients with prurigo nodularis (PN) present with numerous, intensely itchy hyperkeratotic nodules. In addition to itch, patients with PN can experience pain, burning, stinging, and alterations in lesion temperature.^, While PN lesions can appear on any part of the body, the arms, legs, and trunk are most affected. Anywhere from a few to more than 100 lesions are often symmetrically distributed on the trunk and typically display the “butterfly sign” on the back.^1-3 Severely inflamed PN lesions often erode and bleed because of scratching.^1,2

Recent research into the pathophysiology of these intensely itchy PN lesions shows that interleukin 31 (IL-31), an inflammatory cytokine, directly stimulates sensory nerves through upregulation of IL-31 receptor alpha (IL-31-RA), generating the intense sensation of itch experienced by patients with PN.⁴ Skin biopsies of PN patients show a thickening of dermal nerve fibers with a decreased density in intraepidermal nerve fibers and increased expression of neuropeptides, which leads to the activation of cytokine receptors, like IL-31-RA, on the sensory nerves that cause inflammation and the intense itch experienced by patients with PN.^3-5 In fact, studies of PN lesions show a 50-fold increase of IL-31 in mRNA expression compared with healthy skin.²

Although PN can occur without any underlying disease, many patients with PN have associated dermatologic, systemic, neurologic, and psychological diseases of mixed origin that present along with PN.⁶ Systemic diseases commonly associated with PN include atopic dermatitis, attention deficit/hyperactivity disorder, cerebrovascular disease, chronic kidney disease, chronic obstructive pulmonary disease, types 1 and 2 diabetes, heart failure, HIV, hypertension, and cancer.^1,7 Regardless of the underlying cause, PN is a distinct dermatologic disease that can be diagnosed by a dermatologist or other qualified physician and requires management of symptoms, since the pathophysiology of the disease is more difficult to address.^2,3

^References:

1.Huang AH, Williams KA, Kwatra SG. Prurigo nodularis: epidemiology and clinical features. J Am Acad Dermatol. 2020;83(6):1559-1565. doi:10.1016/j.jaad.2020.04.183

2.Kowalski EH, Kneiber D, Valdebran M, Patel U, Amber KT. Treatment-resistant prurigo nodularis: challenges and solutions. Clin Cosmet Investig Dermatol. 2019;12:163-172. doi:10.2147/CCID.S188070

3.Ständer S, Pereira MP, Berger T, et al. IFSI-guideline on chronic prurigo including prurigo nodularis. Itch. 2020;5(4):e42. doi:10.1097/itx.0000000000000042

4.Datsi A, Steinhoff M, Ahmad F, Alam M, Buddenkotte J. Interleukin-31: the “itchy” cytokine in inflammation and therapy. Allergy. 2021;76(10):2982-2997. doi:10.1111/all.14791

5.Williams KA, Huang AH, Belzberg M, Kwatra SG. Prurigo nodularis: pathogenesis and management. J Am Acad Dermatol. 2020;83(6):1567-1575. doi:10.1016/j.jaad.2020.04.182 (P.3, C.2, Ph.2, L.1-17) (P.2, C.2, Ph.3, L.6-8)

6.Iking A, Grundmann S, Chatzigeorgakidis E, Phan NQ, Klein D, Ständer S. Prurigo as a symptom of atopic and non-atopic diseases: aetiological survey in a consecutive cohort of 108 patients. J Eur Acad Dermatol Venereol. 2013;27(5):550-557. doi:10.1111/j.1468-3083.2012.04481.x

7.Huang AH, Canner JK, Khanna R, Kang S, Kwatra SG. Real-world prevalence of prurigo nodularis and burden of associated diseases. J Invest Dermatol. 2020;140(2):480-483.e4. doi:10.1016/j.jid.2019.07.697

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