Scleromyxedema and lichen myxedematosus (LM) are rare disorders that fall along the spectrum of primary cutaneous mucinoses. Scleromyxedema is a systemic form that classically presents with generalized waxy papules, sclerodermoid eruption, and monoclonal gammopathy; LM is a localized form limited to the skin that classically presents with white, firm, waxy papules and lacks monoclonal gammopathy. According to diagnostic criteria established in 2001, the diagnosis of both conditions requires absence of thyroid disease. However, atypical cases that lack monoclonal gammopathy and that present with hypothyroidism have been reported, suggesting that these criteria may require revision. First, we report a case of a 58-year-old female with a history of Hashimoto thyroiditis and biopsy-proven scleromyxedema responsive to intravenous immunoglobulin therapy with delayed presentation of monoclonal gammopathy. Next, we report a case of a 54-year-old female with a history of hypothyroidism, Hodgkin’s lymphoma in remission after radiation and chemotherapy, and concurrent rheumatoid arthritis, with biopsy-proven LM temporarily responsive to systemic steroids. Our cases demonstrate that patients with papular mucinoses can have a multitude of concurrent and prior rheumatologic and endocrine conditions, including thyroid disease, which should not preclude a diagnosis of scleromyxedema and LM.

Background: Pediatric psoriasis is a common, disfiguring cutaneous disease that has significant consequences on patient quality of life. The current biologic regimens for moderate to severe pediatric psoriasis vulgaris include ustekinumab, and etanercept or adalimumab. The evidence for use of other biologic agents has been limited. Objective: To provide evidence of the efficacy of ixekizumab, an IL-17 inhibitor, in the treatment of refractory pediatric psoriasis and to summarize the use of ustekinumab, etanercept, and adalimumab in pediatric moderate to severe psoriasis vulgaris. Methods: A 15-year-old male with severe, refractory psoriasis vulgaris was treated with ixekizumab injections with the following dosing: 160 mg loading dose, followed by biweekly 80 mg for 12 weeks, then monthly 80 mg. The PubMed database was used to search the following terms: adalimumab, etanercept, pediatric, psoriasis, refractory, resolution, treatment, and ustekinumab. The relevant randomized controlled clinical trials generated by the search were summarized. Results: Four weeks after initiating ixekizumab injections, the patient had striking improvement in his psoriatic lesions with a decrease in baseline Physician's Global Assessment from 3 to 1 and a decrease in Psoriasis Area Severity Index from 32.1 at initiation to 2.7 at 1 month. Conclusion: Ixekizumab is not currently a Food and Drug Administration (FDA)-approved treatment for pediatric psoriasis. Ustekinumab and etanercept are both FDA approved and have been used with good, but incomplete responses in many cases. This case report illustrates the success of ixekizumab in the treatment of pediatric psoriasis refractory to traditional regimens and suggests IL-17 blockade may be highly effective in treating moderate to severe pediatric psoriasis.