What is the treatment for generalized pruritus in Crest syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)?

From the Guidelines

For generalized pruritus in CREST syndrome, a stepwise approach is recommended, starting with nonsedative antihistamines such as fexofenadine 180 mg or loratadine 10 mg, as suggested by the British Association of Dermatologists' guidelines 1. When considering treatment options, it's essential to prioritize those with the strongest evidence and fewest side effects.

• Topical treatments, including moisturizers and emollients, can be applied multiple times daily to help relieve itch.
• Oral antihistamines, such as cetirizine 10mg daily or hydroxyzine 25mg three times daily, can be used to reduce itching, but sedative antihistamines should be reserved for short-term or palliative use due to their potential side effects 1.
• For persistent symptoms, consider gabapentin starting at 300mg daily and gradually increasing to 300mg three times daily, or pregabalin starting at 75mg twice daily, as these have been shown to be effective in relieving pruritus in some cases 1.
• UVB phototherapy may also be effective in managing generalized pruritus, particularly in cases where other treatments have failed 1. It's crucial to note that while alternative therapies like acupuncture and acupressure have been suggested as potential treatments for generalized pruritus, the current evidence is limited, and they should not be considered as first-line therapies 1.
• Patients should avoid hot showers, use mild soaps, wear loose cotton clothing, and maintain adequate hydration to minimize triggers.
• Regular follow-up is essential to monitor treatment response and adjust therapy as needed.

From the FDA Drug Label

For symptomatic relief of anxiety and tension associated with psychoneurosis and as an adjunct in organic disease states in which anxiety is manifested. Useful in the management of pruritus due to allergic conditions such as chronic urticaria and atopic and contact dermatoses and in histamine-mediated pruritus The treatment for generalized pruritis in Crest syndrome with hydroxyzine (PO) is for the management of pruritus due to allergic conditions.

• Key points:
  • Hydroxyzine is useful in the management of pruritus due to allergic conditions.
  • It can be used for histamine-mediated pruritus. 2

From the Research

Treatment for Generalized Pruritis in CREST Syndrome

There is limited information available on the treatment of generalized pruritis in CREST syndrome. However, based on the available evidence, the following treatment options can be considered:

• Topical anti-inflammatory therapies such as hydrocortisone (2.5%), triamcinolone (0.1%), or tacrolimus ointment 3
• Neuropathic topical treatments such as menthol, pramoxine, or lidocaine can be used either alone or in combination with immunomodulatory agents such as topical steroids 3
• Systemic oral or injectable treatments such as dupilumab or methotrexate may be considered for patients who do not respond to topical therapies 3
• Gabapentin, antidepressants such as sertraline or doxepin, or opioid receptor agonist/antagonists such as naltrexone or butorphanol may be effective for neuropathic pruritus 3
• Avoiding dry climate, heat, alcohol compress, ice packs, frequent bathing and washing, intake of very hot and spicy food, intake of alcohol, contact with irritant substances, excitement, strain and stress, and allergens may help alleviate symptoms 4
• Using mild nonalkaline soaps, moisturizers, bathing oils, lukewarm water while bathing, soft cotton clothing and night creams/lotions, relaxation therapy, autogenic training, psychosocial education, educating patients to cope with itching and scratching, and educational programs may also be beneficial 4

Diagnosis of Generalized Pruritis in CREST Syndrome

The diagnosis of generalized pruritis in CREST syndrome involves a thorough history, review of symptoms, and laboratory evaluation 5, 3, 4, 6. The following tests may be considered:

• Complete blood cell count
• Complete metabolic panel
• Thyroid function testing to evaluate for hematologic malignancy, liver disease, kidney disease, or thyroid disease 3
• Screening for systemic diseases associated with pruritus, such as disorders of iron metabolism, chronic kidney disease, chronic liver disease, endocrine/metabolic diseases, hematological disorders, and malignant solid tumors 5, 3, 4, 6

Management of Generalized Pruritis in CREST Syndrome

The management of generalized pruritis in CREST syndrome requires a comprehensive approach that takes into account the underlying cause of the pruritus 5, 3, 4, 6. The following steps may be considered:

• Identifying and treating any underlying systemic disease associated with pruritus
• Using topical and systemic treatments to alleviate symptoms
• Avoiding triggers that exacerbate symptoms
• Educating patients on how to cope with itching and scratching
• Providing psychosocial support and education to patients and their families 4