Worsening indurated pink translucent nodules and severe hyperkeratosis of the lower extremies: A Case of Elephanasic Prebial Myxedema. Jason Solway, DO, Oben Ojong, DO, John Moesch, DO, Michael R. Heaphy, Jr, MD, Mahew Mahoney, MD. Largo Medical Center, Largo, Florida LEARNING OBJECTIVES FIGURES DISCUSSION • Recognizing elephan9asis pre9bial myxedema (PTM) • EPIDEMIOLOGY: Elephantiasic pretibial myxedema (PTM) is the 1 2 3 most severe variant of non-filarial myxedema occurring in only 1% • Understanding pathophysiology of elephan9asis PTM. of patients with Grave’s disease1. • Creang treatment plan for paent suffering from • PATHOPHYSIOLOGY: It is theorized that T-cells stimulate elephan9asis PTM. shared antigens between the thyroid and pretibial tissue and release TGF-B and IL1-alpha that stimulate fibroblasts to produce • Ddx for elephan9asis PTM. and deposit mucin-like glycosaminoglycans in tissue. The pretibial fibroblasts may be more sensitive to this stimulation2. • The Pretibial area is favored secondary to hydrostatic forces, decreased lymphatic cytokine clearance and dependent position3. • CLIINICAL: Grossly enlarged and disfigured appendage, usually CASE PRESENTATION with functional restriction and cosmetic concerns for the patient. Cutaneous changes include non-pitting edema of lower extremities that does not resolve with elevation. The initial cobblestone • A 61 year old white woman presented with bilateral appearance later becomes mossy and verrucous. Because hair HPI: follicles are prominent, it produces the characteristic peau lower extremity derma99s, swelling and skin thickening that d’orange appearance 3,6. began 5 years ago; shortly before she was diagnosed with Figures 1-3: Clinical images of indurated 1-2cm thick violaceoous plaques with • Ulceration and bacterial seeding with recurrent cellulitis or fungal Graves disease (Figures 1-3). interspersed pink translucent nodules; associated deep fissuring and non pitting edema. infections are common, with patients complaining of pain or 6 Figure 3: Active serous drainage with overlying yellow-white crust on pretibial area. pruritus . • Large amounts of mucinous deposition are seen in • Paent’s symptoms progressively worsened post PATHOLOGY: the reticular dermis. There is a lack of angioplasia and thyroidectomy and achievement of euthyroid state with hemosiderin. Sparse lymphocytic deposition in perivascular levothyroxine. Previous diagnoses included cellulis and 4 5 6 spaces and moderately increased mast cell deposition are seen3. lymphedema treated with mulple failed aempts of oral • The number of collagen fibers is reduced with increased edema, 4-5 anbiocs. No family history of related condions. No and occasional acanthosis, hyperkeratosis, and papilomatosis . • TREATMENT: Cosmesis and restoration of function are the previous biopsy was obtained. primary aims in ENV treatment3. • Therapeutic modalities like complete decompressive • PHYSICAL EXAM: Indurated, 1-2cm thick violaceous physiotherapy, topical corticosteroids with occlusive dressing, plaques with interspersed pink translucent nodules; psoriatane, octreotide and weight reduction have proven beneficial7. associated deep fissures with ac9ve serous drainage and • Tobacco cessation is imperative as it has been linked to overlying yellow-white crust on bilateral pre9bial areas, autoimmune manifestations of Grave’s disease7. ankles and dorsal feet. (Figures 1-3) Plantar surface was covered by thick scale. Figures 4-5: H&E of a 6mm punch biospy on the right shin and right dorsal foot. REFERENCES • Other physical exam findings included proptosis, Hyperkeratosis , papillomatosis, and acanthosis of the epidermis. Large quanes of mucin are exophthalmos and surgical scar on the anterior neck. deposited within the re9cular dermis, causing collagen bundles to separate and the dermis to thicken. 1. Humbert P, Dupond JL, Carbillet JP. Pretibial myxedema: an overlapping clinical manifestation of autoimmune thyroid disease. Am J Med. 1987;83:1170-1171. A grenz zone of normal collagen is also observed. 2. Korducki JM, Loftus SJ, Bahn RS. Stimulation of glycosaminoglycan production acids in localized in cultured human retroocular fibroblasts. Invest Ophthalmol Vis Sci 1992 59 (3): 409-16 • DX: Aer obtaining informed consent 2 biopsy specimens Figure 6: Colloidal iron stain demonstrating an abundance of mucin in the throughout May; 33 (6): 2037-42 were obtained for hematoxyllin-eosin and other special 3. Fatourechi V. Pretibial myxedema: pathophysiology and treatment options. American Journal of the dermis. Clinical Dermatology. 2005 6(5):295-309. stains (Figures 3-6). 4. Schwartz KM, Fatourechi V, Ahmed DD, et al. Dermopathy of Graves’ disease (pretibial myxedema): long-term outcome. J Clin Endocrinol Metab 2002 Feb; 87 (2): 438-46. 5. Sanders LJ, Slomsky JM, Burger-Caplan C. Elephantiasis nostras: an eight-year observation of progressive nonfilarial elephantiasis of the lower extremity. Cutis. 1988 Nov; 42(5):406-11. • Elephanasis pre bial myxedema was diagnosed based on 6. Ruocco E, Puca RV, Brunetti G, Schwartz RA, Ruocco V. Lymphedematous areas: privileged sites for tumors, infections, and immune disorders. Int J Dermatol. 2007 Jun; 46(6):662. these clinical and histological findings. 7. Susser WS, Heermans AG, Chapman MS, et al. Elephantiasic pretibial myxedema: a novel treatment for an uncommon disorder. J Am Acad Dermatol 2002 (May); 46 (5): 723-726. .