Novel teprotumumab treatment of severe thyroid dermopathy; ototoxicity as an adverse side effect.

Pretibial myxedema, more generally thyroid dermopathy, results from mucopolysaccharide accumulation in the dermis, typically between the knee and dorsal foot. Thyroid dermopathy presents in Graves disease, but can occur in Hashimoto thyroiditis, primary hypothyroidism, and euthyroid patients. Treatment of thyroid eye disease with teprotumumab is established in the literature, with few case reports also showing improvement in pretibial myxedema. Reported is a 76-year-old man with thyroid eye disease and pretibial myxedema treated with teprotumumab; improvement was demonstrated in both conditions. He developed "muffled" hearing as an adverse effect, a complication not widely published in the dermatology literature. At 18 months post-treatment, his symptoms are stable without recurrence, but hypoacusis persists. Given the long-term efficacy and side-effects, dermatologists should recognize the potential benefits and risks of using teprotumumab for thyroid dermopathy. A baseline audiogram may be considered prior to therapy. Additionally, longitudinal data is needed to document the benefits and risks of this novel therapy.

Elephantiasis nostras verrucosa in the setting of lymphoedema tarda and neurofibromatosis type 1.

Elephantiasis nostras verrucosa (ENV) comprises an uncommon skin disease characterised by dermal fibrosis with hyperkeratotic, verrucous and papillomatous lesions that usually occur after chronic lymphoedema. We describe the case of a 56-year-old-man with neurofibromatosis type 1, no known surgical history, no chronic medication and no travel history, presenting with worsening non-pitting oedema and impressive foul-smelling mossy plaques and cobblestone-like nodules in both legs and feet, especially on the right, compatible with the diagnosis of ENV.

Unilateral granuloma annulare in association with pyoderma gangrenosum and chronic lymphocytic leukemia.

Granuloma annulare (GA) is a fairly common inflammatory skin condition with a range of clinical subtypes. We describe an unusual case of unilateral GA confined to the thigh on a previously amputated limb. A man in his 80s with a past medical history of below-knee amputation of the left leg owing to severe leg ulcers from pyoderma gangrenosum, chronic lymphocytic leukemia, and dyslipidemia developed a slowly spreading eruption on the distal stump spreading proximally. On physical examination, he had numerous non-scaly violaceous papules and annular plaques from the stump to the lateral, medial, and anterior thigh. Histology confirmed a diagnosis of GA. The extensive, chronic lesions make this presentation of GA very unusual in that it shares features of both localized and generalized forms. Moreover, the temporal and spatial association with pyoderma gangrenosum is unique and may reflect a related inflammatory pathway.

Hidradenitis suppurativa, neutrophilic dermatoses and diverticular disease in a young African-American patient.

Hidradenitis suppurativa (HS) is a chronic skin disorder of the terminal follicular epithelium of apocrine sweat glands, manifesting as painful and exudative papules, pustules, cysts or nodules. This inflammatory condition often presents with other systemic and cutaneous disorders. We present the case of an African-American man with HS who was also diagnosed with neutrophilic dermatoses and diverticular disease. Neutrophilic dermatosis was identified based on histopathology findings. Our patient underwent multiple surgeries for flaring of his skin condition. Colchicine and doxycycline were started, but the patient was not able to tolerate them. Humira was planned for treatment of HS and neutrophilic dermatosis but could not be pursued because of the pericolic abscess. Colonoscopy and radiological investigation revealed multiple colonic diverticuli, for which he initially underwent percutaneous drainage followed by surgical removal of sigmoid mass and colocutaneous fistula. Culture from the specimen revealed abnormal growth of Actinomyces.

Milia-like idiopathic calcinosis cutis in a child with Down syndrome.

Idiopathic calcinosis cutis refers to progressive deposition of crystals of calcium phosphate in the skin and other areas of the body, in the absence of any inciting factor. Idiopathic calcinosis cutis may sometimes take the form of small, milia-like lesions. Most commonly, such milia like lesions are seen in the setting of Down syndrome. Herein, we report a 5-year-old girl with multiple asymptomatic discrete milia-like firm papules distributed over the face and extremities. A diagnosis of milia-like idiopathic calcinosis cutis associated with Down Syndrome was provisionally made and was confirmed by histopathology and karyotyping.

Eosinophilic dermatosis of hematologic malignancy.

We report a 68-year-old woman with chroniclymphocytic leukemia, who developed numerous,pruritic, edematous, and vesicobullous skin lesionsof the face and extremities over the course of severalmonths. The diagnosis of eosinophilic dermatosis ofhematologic malignancy (EDHM) was made basedon the clinical history and histopathologic features.Owing to the possible link between EDHM and amore aggressive underlying CLL, she was startedagain on chemotherapy. This case serves as areminder that, although the precise pathogenesis ofEDHM remains unclear, the paraneoplastic disorderis the result of immune dysregulation. Patientswho develop EDHM should undergo prompthematologic/oncologic evaluation.

Necrolytic migratory erythema associated with fatty liver disease and the psuedoglucagonoma syndrome.

We report a 48-year-old woman with a past medical history of psoriasis, nonalcoholic steatohepatitis (NASH), and type II diabetes mellitus, who presented to the emergency department with a 1 week history of erosive annular plaques with associated atrophy and telangiectasias on her legs bilaterally, thighs and buttock, histopathologically consistent with necrolytic migratory erythema. Although classically associated with a pancreatic glucagonoma, this patient experienced this figurate erythema in the setting of fatty liver disease with no glucagonoma. The rarity of pseudoglucagonoma syndrome, or necrolytic migratory erythema occurring in the absence of a glucagonoma, warranted the discussion of this case.

Stasis papillomatosis with cardiac complications and vein insufficiency.

We report the case of a 73-year-old man with massive swelling of the lower extremities, with a chronic and rather uncommon form of stasis dermatitis - stasis papillomatosis. The patient was also diagnosed with severe heart failure, including dilated cardiomyopathy, hypothyroidism that required a substantial dose of exogenous tyrosine, microcytic and megaloblastic anemia, iron deficiency, and type 2 diabetes. The cause of stasis dermatitis lesions is not completely understood. It may be caused by the allergic reaction to some epidermal protein antigen formation or chronic damage to the dermal-epidermal barrier that makes the skin more sensitive to irritants or trauma. It has, however, been suggested that the term stasis dermatitis should be used to refer only to cases caused by chronic venous insufficiency, which belongs to a group of lifestyle diseases and affects both women and men more and more frequently.

Cutaneous reactive angiomatosis associated with erythema ab igne.

We present the case of a 49-year-old man with an 18-month history of an intermittently pruritic red-brown reticulate eruption of the medial legs, with occasional blistering. There were areas of scale, crust and superficial erosions. The patient worked long overnight shifts standing in front of hot machinery wearing shorts, and spent time sitting in front of a heater at home. A clinical diagnosis of erythema ab igne (EAI) was made. This settled with heater avoidance and protection of the legs with long trousers, as well as a topical corticosteroid. Histology showed features of cutaneous reactive angiomatosis, a rare condition with various associations but only previously reported once in association with EAI.