A case of concurrent systemic sclerosis and scleredema

Mitchell, Drew C; Agnihothri, Ritesh; Scott, Glynis; Korman, Benjamin; Richardson, Christopher T.

doi:10.1016/j.jdcr.2019.09.004

Cited by 3 publications

(7 citation statements)

References 8 publications

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“…Scleredema is a sclerotic skin disease that can be divided into three types. Type 1 is often preceded by an acute fever or inflammatory disease and is very common in middle-aged women [ 5 ]. Type 2 often carries a long progressive course without a history of infection or diabetes [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Type 1 is often preceded by an acute fever or inflammatory disease and is very common in middle-aged women [ 5 ]. Type 2 often carries a long progressive course without a history of infection or diabetes [ 5 ]. Meanwhile, type 3 is often associated with diabetes and obesity and is common among middle-aged men [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Type 2 often carries a long progressive course without a history of infection or diabetes [ 5 ]. Meanwhile, type 3 is often associated with diabetes and obesity and is common among middle-aged men [ 5 ]. In comparison, our patient had a prolonged slow course of the disease for four years but also had repeated attacks of throat infection making this presentation suggestive of both type 1 and 2.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…It is characterized by linear atrophy and/or hardness of the skin, subcutis, and muscle and bone involvement. Lesions start as an erythematous or violaceous linear indurated moderate atrophic plaque and then proceed to hypopigmented or depigmented sclerotic deep furrows [ 5 - 7 ]. The signs of morphea that are seen in histopathology include the line sign, the cookie-cutter sign, and the square biopsy sign, additionally, a high number of eccrine glands and the presence of interstitial mucin are also mentioned in various studies [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology

Alhunaif¹,

Alsarheed²,

Almutairi³

et al. 2023

Cureus

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Scleredema is a rare sclerotic skin disorder that typically develops in conjunction with diabetes, monoclonal gammopathy, or infection and commonly involves the neck, back, trunk, and arms. Scleredema can be categorized into three types according to its cause. The local examination of the lesion is characterized by non-pitting induration that follows a symmetrical spread with sparing of the hands and feet. We present a case of a 19-year-old female patient that presented to the outpatient clinic complaining of facial skin lesions that have been progressive for four years. The skin lesions were found to involve the neck, back, trunk and both arms sparing the hands and there was no systemic involvement of the disease. The patient is non-diabetic but reported frequent attacks of tonsillitis over the past months. Initially, punch biopsy showed no signs of scleredema; however, repeated biopsy at follow-up confirmed the presence of mucin deposits which are suggestive features of scleredema. Due to the similarities with various other diseases, the diagnosis requires clinical and histopathological exclusion which makes the diagnosis quite challenging. It almost always starts in the neck but can present initially in rare cases from the face spreading symmetrically. Close follow-up and continuous monitoring are necessary for systemic organ involvement.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology

Alhunaif¹,

Alsarheed²,

Almutairi³

et al. 2023

Cureus

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Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema

Knobler,

Geroldinger‐Simić,

Kreuter

et al. 2024

Acad Dermatol Venereol

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The term ‘sclerosing diseases of the skin’ comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first‐ and advanced‐line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

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Scleroderma Associated with Monoclonal Gammopathy of Undetermined Significance

MohamedSharif

Abdelmaged

Avila

et al. 2022

SJMS

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A 53-year-old female presented with itchiness in her back. She has a 5 years history of tightness of the skin on her face, neck, and torso bilaterally. She did not have other symptoms suggestive of systemic sclerosis (scleroderma), and her rheumatologic workup was negative. Skin biopsy showed increased dermal mucin confirming a diagnosis of scleredema. Further workup with serum protein electrophoresis (SPEP) showed an M-spike, confirming the diagnosis of monoclonal gammopathy of undetermined significance MGUS as the underlying pathology.

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A case of concurrent systemic sclerosis and scleredema

Cited by 3 publications

References 8 publications

Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology

Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema

Scleroderma Associated with Monoclonal Gammopathy of Undetermined Significance

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