Multiple clustered dermatofibroma presenting in a segmental distribution

Bhabha, Friyana K; Magee, Jill; Ng, Su Yuen; Grills, Claire; Su, John; Orchard, David

doi:10.1111/ajd.12257

Cited by 10 publications

(15 citation statements)

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“…This presents as grouped 5-to 10-mm reddish-brown papules coalescing into a plaque up to 25 cm. [8][9][10][11][12][13][14][15][16][17][18][19][20] Fewer than 20 cases have been reported in the literature and most involve the thigh, hip, and back in patients in the first and second decades of life, 8,[11][12][13][14]18,20 although congenital cases of MCDF have also been reported. 9,10,15,16 Of the reported cases of MCDF, immunohistochemical staining for SMA had been performed in 8 cases.…”

Section: Discussionmentioning

confidence: 99%

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood

et al. 2017

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Plaque-like myofibroblastic tumor (PLMT) is a rare dermal spindle cell tumor which occurs in infancy or childhood within the first 4 years of life. The tumor is often pruritic and mostly presents on the lower back. We describe 2 cases with characteristic clinical and histological features of this entity, thus adding to the 10 cases which have so far been reported. Histologically, the lesion resembles a dermatofibroma. However, diffuse and uniform immunohistochemical staining with smooth muscle actin favors a myofibroblastic lineage. PLMT should be considered in the differential diagnosis of a dermal spindle cell tumor in the pediatric age-group.

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Section: Discussionmentioning

confidence: 99%

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood

et al. 2017

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“…Of note, some cases of clustered or plaque-like dermatofibromas appear to be different from the MCDF/PLDF/PLMT group. Most notable among these are four case reports of patients who also had multiple clustered or plaque-like dermatofibromas that, however, differed by their eruptive time course and, in three cases, by coincidence with the administration of immunosuppressive medication [27][28][29][30]. Our patient clearly does not fit into this group because her plaque exhibited very slow growth, and she had taken immunomodulatory medication only in the recent past.…”

Section: Discussionmentioning

confidence: 73%

Plaque-Like Dermatofibroma: Case Report of a Rare Entity

et al. 2021

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“…To our knowledge, our case represents only the second reported case in the UK . MCDF is characterized by the presence of multiple dermatofibromas clustered at a single anatomical site, although more recently, a case of MCDF in a segmental distribution was reported . Plaques of up to 250 mm in diameter, made up of coalescing reddish‐brown papules or nodules have been described, and new lesions can continue to appear at the periphery over a period of years .…”

Section: Discussionmentioning

confidence: 77%

Localized cluster of nodules in childhood

2017

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An 8-year-old girl presented with multiple, asymptomatic skin lesions localized to her right lower abdomen. These had first appeared 6 years previously, and had gradually increased in number over the intervening years. The patient was otherwise systemically well. On physical examination, a plaque 50 mm in size was found on the patient's right abdomen, made up of coalescing, firm, red-brown papules and nodules ranging from 4 to 10 mm in size (Fig. 1).Laboratory investigations gave normal results for full blood count, urea and electrolytes, liver function tests and protein electrophoresis. However, autoantibody screen revealed a positive titre of anti-smooth muscle antibodies (anti-SMA) (≥ 1 : 320); the rest of the autoantibody screen was negative. Histopathological findingsA punch biopsy was taken from one of the lesions, and histological examination revealed an unencapsulated dermal proliferation comprising of bland spindleshaped cells associated with collagen and scattered histiocytes (Fig. 2). Occasional foci of rounding off of collagen were noted at the periphery of the lesion. There was irregular acanthosis of the epidermis, with no increase in basal pigmentation or trichoblastic induction. The spindle-shaped cells stained positively for Factor XIIIa and negatively for CD34, SMA, desmin, CD99 and S-100P. CD34 and SMA highlighted the stroma around the proliferation and the blood vessels.What is your diagnosis? DiagnosisMultiple clustered dermatofibromas (MCDF).

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Multiple clustered dermatofibroma presenting in a segmental distribution

Cited by 10 publications

References 10 publications

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood

Plaque-Like Dermatofibroma: Case Report of a Rare Entity

Localized cluster of nodules in childhood

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