Discrete Papular Lichen Myxedematosus and Scleromyxedema with Hypothyroidism: A Report of Two Cases

Shenoy, Adele; Steixner, Jacklyn; Beltrani, Vincent S.; Gottlieb, Alice B.

doi:10.1159/000498881

Cited by 4 publications

(5 citation statements)

References 15 publications

(27 reference statements)

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“…High-dose intravenous immunoglobulins 0 2 [36,37] 2 [17,47] 5 [40,41,[57][58][59] 14 [60][61][62][63][64][65][66][67][68][69][70][71][72][73] in combination 0 0 3 [17,38,47] 0 17 [46,48,49,55,[74][75][76][77][78][79][80][81][82][83][84][85][86] Systemic corticosteroids 0 0 2 [17,38] 1 [87] 8 [71,[88][89][90][91][92]…”

Section: Treatment Rct Ps Rs Cs Crmentioning

confidence: 99%

Scleromyxedema

Hoffmann

Enk

2020

J Deutsche Derma Gesell

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Summary Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome‐shaped or flat‐topped, waxy, slightly red to skin‐colored papules and sclerodermoid indurations. Neurological, rheumatological, cardiovascular, gastrointestinal, respiratory tract, renal and ophthalmologic manifestations can occur, with decreasing frequency. A serious and potentially lethal complication is the dermato‐neuro syndrome which manifests with flu‐like prodromes followed by fever, convulsions and coma. Untreated, scleromyxedema usually takes an unpredictable and potentially lethal progressive disease course over several years. According to a widely acknowledged classification by Rongioletti a diagnosis of scleromyxedema can be rendered when (1) generalized, papular and sclerodermoid eruption, (2) a histological triad of mucin deposition, fibroblast proliferation and fibrosis, and (3) monoclonal gammopathy are present, and (4) thyroid disease is absent. Apart from the classic microscopic triad, an interstitial granuloma annulare like pattern was also described. The pathogenesis of scleromyxedema is unknown. A potential role for various, as yet unknown serum factors has been discussed. An unequivocal causal relationship between paraproteinemia and disease manifestations could not be established to date. High dose intravenous immunoglobulins (IVIg) are the first‐line treatment of choice according to the most recent European guidelines.

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Section: Treatment Rct Ps Rs Cs Crmentioning

confidence: 99%

Scleromyxedema

Hoffmann

Enk

2020

J Deutsche Derma Gesell

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“…Due to a medical history of hypothyroidism and clinical presentation of venous disease, differential diagnosis was expanded to papular mucinosis, myxedema, and stasis dermatitis. Papular mucinosis, also known as localized lichen myxedematosus, presents with pretibial waxy papules and plaques, mostly without pruritus, systemic involvement, or thyroid dysfunction (3,4). Myxedema occurs in patients with Graves' disease with diffuse pretibial non-pitting edema and skin thickening with nodules (5).…”

Section: Discussionmentioning

confidence: 99%

A case report and differential diagnosis of pruritic pretibial skin lesions

Kecelj

Leskovec

Žgavec

2020

Acta Dermatovenerologica Alpina Pannonica Et Adriatica

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Pretibial pruritic papular dermatitis (PPPD) is a clinical entity first described in 2006. The etiology is uncertain; however, gentle chronic rubbing is likely to be the reason for the skin reaction. Pretibial pruritic lesions may reflect many different systemic diseases and dermatoses. We present a 61-year-old patient with a 2-year history of pruritic pretibial xerosis, keratotic erythematous to brownish papules, and excoriations. Differential diagnosis excluded papular mucinosis, myxoedema, stasis dermatitis, lichen simplex chronicus, prurigo nodularis, lichen amyloidosis, and lichen planus. Regarding clinical-histological correlation, we confirmed a diagnosis of PPPD.

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“…It is important to rule out thyroid disease as a possible etiology before concluding that the disease process is driven by the monoclonal gammopathy; this will help determine appropriate therapies. 4,5 Usually the monoclonal protein is associated with the IgG lambda subtype. Intravenous immunoglobulin often is considered as a first-line treatment of scleromyxedema and usually is administered at a dosage of 2 g/kg divided over 2 to 5 consecutive days per month.…”

Section: The Diagnosismentioning

confidence: 99%

Scattered Flesh-Colored Papules in a Linear Array in the Setting of Diffuse Skin Thickening

Hobayan¹

2022

Cutis

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A 76-year-old man presented to our clinic with diffusely thickened and tightened skin that worsened over the course of 1 year, as well as numerous scattered small, firm, flesh-colored papules arranged in a linear pattern over the face, ears, neck, chest, abdomen, arms, hands, and knees. His symptoms progressed to include substantial skin thickening initially over the thighs followed by the arms, chest, back (top), and face. He developed confluent cobblestonelike plaques over the elbows and hands (bottom) and eventually developed decreased oral aperture limiting oral intake as well as decreased range of motion in the hands. The patient had a deep furrowed appearance of the brow accompanied by discrete, scattered, flesh-colored papules on the forehead and behind the ears. Deep furrows also were present on the back. When the proximal interphalangeal joints of the hands were extended, elevated rings with central depression were seen instead of horizontal folds.WHAT'S YOUR DIAGNOSIS? a. localized lichen myxedematosus b. reticular erythematous mucinosis c. scleredema d. scleroderma e. scleromyxedema

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Discrete Papular Lichen Myxedematosus and Scleromyxedema with Hypothyroidism: A Report of Two Cases

Cited by 4 publications

References 15 publications

Scleromyxedema

Scleromyxedema

A case report and differential diagnosis of pruritic pretibial skin lesions

Scattered Flesh-Colored Papules in a Linear Array in the Setting of Diffuse Skin Thickening

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