Does the Aberrant Expression of CD2 and CD25 by Skin Mast Cells Truly Correlate with Systemic Involvement in Patients Presenting with Mastocytosis in the Skin?

Lange, Magdalena; Żawrocki, Anton; Nedoszytko, Bogusław; Wasąg, Bartosz; Niedoszytko, Marek; Jassem, Ewa; Nowicki, Roman; Żmijewski, Michał A.; Biernat, Wojciech

doi:10.1159/000368799

Cited by 14 publications

(16 citation statements)

References 30 publications

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“…The differential diagnosis for CM includes xanthogranuloma, leukemia cutis, metastasis, vascular tumors, adnexal tumors and melanocytic tumors . Cutaneous mastocytosis is diagnosed on the basis of a typical skin lesion morphology, a positive Darier sign, histopathology and the absence of systemic mastocytosis (SM) criteria . An extension study should include blood cell count, serum chemistries (including liver function tests), serum tryptase levels and bone marrow biopsy (to evaluate mast cell clusters, morphology, immunophenotype and KIT gene mutation) …”

Section: Discussionmentioning

confidence: 99%

“…In fact, while CD25 expression is highly suggestive of SM in patients with urticaria pigmentosa, it is a minor criterion for SM . Despite this evidence, Lange et al concluded that, even though skin mast cells display immunoreactivity for CD25 in most SM patients, aberrant expression of this antigen in skin mast cells is not as indicative of SM as would be its expression in bone marrow mast cells. In the case of our patient, however, the combination of strong CD25 positivity and marked pleomorphic mast cells was highly suggestive of systemic involvement, for which reason a bone marrow biopsy was performed to rule out malignancy.…”

Section: Discussionmentioning

confidence: 99%

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Pleomorphic mastocytoma in an adult

et al. 2017

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Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast-cell skin lesions and commonly develops in newborns and children. We report the case of a 35-year-old healthy Caucasian woman presenting with a 4-year history of a pruritic brown plaque on her left breast. Hematoxylin-eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pleomorphic mastocytoma in an adult

et al. 2017

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“…[7][8][9][10][11][12] When involving the skin, neoplastic mast cells tend to have normal cytological features and do not differ from reactive mast cells; hence, we currently lack a specific marker for neoplastic mast cells in the skin, 13,14 in contrast with the well-known marker CD25 for neoplastic cells in the bone marrow. [15][16][17][18][19][20] Although several studies have shown that CD25 expression by mast cells in the skin was predictive of SM, [21][22][23] this immunostaining may not be applicable to CM. In fact, it has been shown that CD25 expression in the skin is not a reliable marker.…”

Section: Introductionmentioning

confidence: 99%

“…In fact, it has been shown that CD25 expression in the skin is not a reliable marker. 23,24 Hence, in cases with very few neoplastic mast cells, the diagnosis is solely based on poorly defined, additional signs: the mast cell count (with different suggested cut-off values) [25][26][27][28][29][30] and other signs: (i) hyperpigmentation of the basal layer of the epidermis, (ii) dilated, more numerous blood vessels in the upper dermis, (iii) the presence of eosinophils, or (iv) fibrosis. 6,31 The diagnosis of challenging cases of CM or of skin involvement in SM therefore requires more reliable signs.…”

Section: Introductionmentioning

confidence: 99%

Establishing diagnostic criteria for mastocytosis in skin biopsies

Drabent¹,

Polivka²,

Agopian

et al. 2021

Histopathology

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Aims:The diagnosis of mastocytosis in skin biopsies can be challenging -particularly in cases with very few mast cells. More diagnostic criteria are needed. Methods and results: We analyzed 103 skin biopsies from patients with mastocytosis and compared them with biopsies from inflammatory skin lesions and normal skin. Using CD117 immunostaining, we determined the mast cell distribution pattern, the percentage of mast cells in the inflammatory infiltrate, and the mast cell count per mm². We found that a sheet-like or subepidermal distribution of mast cells was specific for mastocytosis. The most significant feature was the percentage of mast cells and not the mast cell count. We found that a mast cell percentage above 40% was fully specific in both adults and children but lacked sensitivity, especially in adults. In children, all cases with a percentage below 40% harbored a number of mast cells above 90 per mm², allowing a straightforward diagnosis. In adults, the diagnosis was more challenging and cases with less than 40% of mast cells could be diagnosed on account of a number of mast cells above 40 per mm², with 88.5% sensitivity and 95.2% specificity. Additional signs might be useful in difficult cases. However, CD25 immunostaining was not useful. Conclusions: We confirmed that the criteria currently applied in the bone marrow were not appropriate for the skin. Accordingly, we developed an algorithm for the diagnosis of mastocytosis in skin biopsies with a high level of interrater reproducibility (mean kappa 0.8).

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“…The authors also showed a positive correlation between the density of CD117 and CD25 positive mast cells in the skin infiltrates of systemic mastocytosis patients and the lack of a similar correlation in the skin of cutaneous mastocytosis patients. However, their conclusion is that the bone marrow mast‐cells immunophenotype is more specific for systemic mastocytosis than CD2 and/or CD25 expression on skin mast cells …”

Section: Discussionmentioning

confidence: 99%

Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo–epidermal junction

et al. 2015

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Cutaneous mastocytosis is a disease characterized by the infiltration and proliferation of mast cells in the skin. In children, the most common form of presentation is urticaria pigmentosa, while the diffuse cutaneous bullous mastocytosis is one of the rarest subtypes seen. The aim of this paper is to present a case of diffuse bullous mastocytosis with detection of IgM deposits at dermo-epidermal junction using direct immunofluorescence (DIF) microscopy. The diagnosis of diffuse bullous mastocytosis is a challenge, and DIF microscopy is necessary in order to exclude an autoimmune bullous disorder. However, IgM deposits at dermo-epidermal junction can be nonspecific, being found in a variety of skin disorders. A 6-month-old girl presented with bullous lesions and erosions on the scalp and the trunk. During hospitalization, further bullous lesions appeared, along with generalized erythrodermia. Skin biopsy revealed aspects of urticaria pigmentosa. Taking into account the clinical findings, the case was enclosed as bullous mastocytosis. Treatment included the avoidance of trigger factors, and administration of antihistamines along with a short-term course of systemic steroids. The evolution was favorable, with remission of the existing lesions and without occurrence of new ones.

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Does the Aberrant Expression of CD2 and CD25 by Skin Mast Cells Truly Correlate with Systemic Involvement in Patients Presenting with Mastocytosis in the Skin?

Abstract: Although sMCs displayed immunoreactivity for one of the neoplastic antigens in the majority of SM patients, the aberrant CD2 and/or CD25 expression on sMCs is not as indicative of SM as the BMMC immunophenotype.

Cited by 14 publications

References 30 publications

Pleomorphic mastocytoma in an adult

Pleomorphic mastocytoma in an adult

Establishing diagnostic criteria for mastocytosis in skin biopsies

Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo–epidermal junction

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