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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 (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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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 (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 ., Competing Interests: Conflicts of Interest: None declared., (2017 BMJ Publishing Group Ltd.)

A lot of diseases occur on the skin of elderly persons. We report four elderly cases of bullous dermatosis that did not meet various differential diagnoses. Japanese, heart failure, atrophic skin and leg edema probably due to aging, as well as flaccid or tense bullae localized in legs were the common factors to our patients. Such conditions may be increased in coming aging society. Accordingly, it is worth regarding such symptom as the new clinical entity, which may comfort patients with similar condition and attract further attention.

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.

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.

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.

Introduction: Pretibial myxedema (PTM) is a rare manifestation of Graves' disease. There is paucity of data regarding long-term follow-up and response to treatment in PTM., Materials and Methods: Retrospective study wherein 30 patients of PTM presenting during 2001-2011 attending dermatology and endocrinology outpatient departments were analyzed., Results: Among 30 patients with PTM, 12 were males and 18 females with a ratio of 1 : 1.5 males/females. Four morphological forms were identified: plaques (18 patients), diffuse non-pitting edema of both lower legs (five), nodules (five), and elephantiasis lesions (two). Eighty percent were diagnosed with hyperthyroidism before the development of dermopathy. Twenty-six patients presented with ophthalmopathy. Fourteen patients with plaque had an excellent response to topical clobetasol propionate ointment and attained complete resolution by 3.6 years. Out of 16 patients treated with combination therapy, which included nine treated with topical corticosteroids/intralesional triamcinolone and seven treated with oral, intralesional, and topical corticosteroids, nine attained complete resolution in the lesions by 3.4 years, and none relapsed anytime during four years of post-treatment follow-up. However, the remaining patients (elephantiasis and diffuse forms) failed to achieve complete resolution., Conclusions: Plaques and nodules are common variants with a favorable clinical response to topical and intralesional corticosteroid; elephantine and diffuse forms responded poorly to therapy. Studies analyzing larger cohorts of patients with PTM and their long-term follow-up are limited, hence more such studies are required., (© 2015 The International Society of Dermatology.)

A 62-year-old man with a history of chronic alcohol abuse presented with severe pancreatic panniculitis associated with an acute exacerbation of chronic pancreatitis.

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., (© 2013 The Australasian College of Dermatologists.)

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.

Cytotoxic T-lymphocyte associated antigen-4 (CTLA-4) is a well-known molecule that regulates T cell activity, with polymorphisms at different regions of this gene having been associated with autoimmune conditions. Pretibial myxedema (PTM), also called Graves' dermopathy, is an autoimmune extrathyroidal manifestation of Graves' disease. We opted to investigate the relationship between three single nucleotide polymorphisms of the CTLA-4 gene (+49A/G, and -318C/T and -1147C/T) and PTM in Iranian patients with Graves' ophthalmopathy (GO). A total of 105 unrelated Iranian patients with GO from the outpatient endocrine clinic of a large university general hospital as well as 103 healthy controls were studied. The genomic DNA was extracted from venous blood samples by a salting out method, and the polymorphisms at +49, -318 and -1147 positions of the CTLA-4 gene were determined using the polymerase chain reaction-restriction fragment length polymorphism method. The GG genotype (OR = 6.000, 95% CI = 1.805-19.940, P = 0.005) and the G allele (OR = 2.653, 95% CI = 1.314-5.357, P = 0.009) at position +49 were significantly associated with PTM in the patient group. The same genotype and allele were also significantly more common among patients (with or without PTM) than controls. No significant association was found for the other two polymorphisms. In conclusion, the +49G allele is associated with increased risk of PTM in patients with GO. Studies with larger sample sizes are needed to confirm the results of the present study.

Dermatitis cruris pustulosa et atrophicans (DCPA) is a benign inflammatory skin disease of the younger population in the tropics. Although this pustular skin condition of particular topography is frequently seen by dermatologists in tropical countries, its origin remains unknown. We report the case of a young woman with DCPA associated with prurigo nodularis. A bacterial origin has not been demonstrated in this case. Histology showed an intraepidermal neutrophilic pustule with dermal and subcutaneous infiltration by neutrophils and eosinophils forming flame figures. Different pathogenic hypotheses are discussed with special regard to a potential relationship between DCPA and eosinophilic cellulitis.

Erythema nodosum is an inflammatory reaction of the skin characterized by tender erythematous patches or nodules, usually located on the lower extremities. This report illustrates an association of erythema nodosum with a rare malignancy and an uncommon infectious agent in humans. There are many diseases associated with erythema nodosum; we propose that hairy cell leukemia and group C streptococcus be considered among this list.

Acquired ichthyosis (AI) in association with systemic lupus erythematosus (SLE) is a rare dermatologic finding, with only 7 previously published cases worldwide. We report a 25-year-old black woman with AI associated with SLE. A skin biopsy specimen from the lower extremity showed histologic changes consistent with both ichthyosis vulgaris and SLE, a unique finding that has not been previously reported. We also review the world literature on AI and SLE.

Introduction: Elephantiasic myxoedema is very rare. We report 5 pretibial myxoedema cases observed in the Dermatological department of Le Dantec hospital in Dakar., Patients and Methods: Our 5 cases were 3 women (age: 52, 45 and 18 years) and 2 men (age: 54 and 32 years)., Results: All the cases were enormous, multinodular pachydermic, bilateral, elephantiasis in the low leg and associated to Graves 'disease. The duration was 4 month to 12 year. In 3 cases there was a Diamond syndrome which is a severe form. The cutaneous lesions were not influenced by medical treatment of thyroid disease. Systemic steroids achieved improvement of the elephantiasis in 1 case., Conclusion: Elephantiasis is a very unusual presentation of pretibial myxoedema and its occurrence doesn't depend to the intensity of thyrotoxicosis and its evolution. However in our cases, it was a severity indicator factor, like Diamond syndrome. Local and systemic steroids give very rarely improvement.

We report the case of a 33-year-old patient who had had insulin-dependent diabetes mellitus (IDDM) since he was 11 months old, and who presented with major perforating necrobiosis lipoidica (PNL) complicated by a well-differentiated epidermoid carcinoma. PNL is a rare clinical form of NL, always associated with diabetes. Only seven cases have been reported to date in the literature, and to the best of our knowledge, an association of epidermoid carcinoma and PNL has never been described. The development of a tumoral transformation on a classical NL plaque has only been described 12 times. The presence of an epidermoid carcinoma on a weakened background with permanent ulceration suggests that early surgical excision of the tumour and of the NL followed by a skin graft might be the treatment of choice. Radiotherapy seems to be a poor therapeutic option.