Ultrastructural morphometric analysis of lesional skin: Mast cells from patients with systemic and nonsystemic mastocytosis

Tharp, Michael D.; Glass, Marcia J.; Seelig, Leonard L.

doi:10.1016/s0190-9622(88)70043-2

Cited by 18 publications

(6 citation statements)

References 26 publications

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“…We observed a more diffuse (interstitial) distribution of MCs in MPCM in comparison to healthy skin, which has also previously been reported 20 . MC size in MPCM slightly exceeded that of mastocytomas and controls 19,22 . MPCM has historically been called “urticaria pigmentosa” because of the pigmentation in lesional skin.…”

Section: Discussionsupporting

confidence: 86%

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Validation of dermatopathological criteria to diagnose cutaneous lesions of mastocytosis: importance of KIT D816V mutation analysis

Gebhard¹,

Horny

Kristensen

et al. 2022

Acad Dermatol Venereol

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Background Cutaneous lesions of mastocytosis (CLM) are often subtle and may require biopsy. However, dermatohistopathological criteria for CLM remain undefined. Objectives To establish criteria for CLM by validating histological and molecular parameters. Methods In skin samples from Caucasian patients with CLM and controls (atopic dermatitis, chronic urticaria, pruritus, tissue from tumor safety margin excisions), mast cell (MC) numbers, size, shape, distribution, immunostainability with a large panel of markers, pigmentation and presence of KIT D816V mutation were analysed. Results Forty‐seven CLM patients (32 maculopapular cutaneous mastocytosis (MPCM), 15 mastocytomas) and 36 controls were included. Mastocytomas were easily identified by densely packed cuboidal MCs. In MPCM, skin MC density in CD117 stains was higher in CLM patients than in controls (P < 0.0001) and values correlated closely (r = 0.65, P < 0.0001) to results in tryptase stains. The optimized upper dermis cut‐off number of 62 MC/mm2 had a sensitivity and specificity of 92% in both stainings, corresponding to approximately 12 MC/high power field (HPF). MC size was larger in MPCM than in controls (P = 0.01). Interstitial (= not perivascular or periadnexal) MCs and stronger basal pigmentation of the epidermis were indicative of MPCM (P < 0.0001 each) and clusters of >3 nucleated MC/HPF exclusively found in MCPM. Surface markers CD2, CD25 and CD30 stained T‐lymphocytes, but only negligibly CLM MC. The KIT D816V mutation in formalin fixed paraffin embedded (FFPE) skin was evaluable in 87.5% of MCPM patients and had both 100% sensitivity and specificity. Conclusions MPCM can be predicted by major and minor criteria combined in a scoring model. Presence of D816V mutation in FFPE skin and MC density > 27/HPF are >95%‐specific major criteria for MPCM. MC densities 12/HPF, interstitial MC, clusters and basal pigmentation are minor criteria.

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Section: Discussionsupporting

confidence: 86%

“…20 MC size in MPCM slightly exceeded that of mastocytomas and controls. 19,22 MPCM has historically been called "urticaria pigmentosa" because of the pigmentation in lesional skin. In Caucasians, this was reflected in the histology by a strong or medium pigmentation.…”

Section: Discussionmentioning

confidence: 99%

Validation of dermatopathological criteria to diagnose cutaneous lesions of mastocytosis: importance of KIT D816V mutation analysis

Gebhard¹,

Horny

Kristensen

et al. 2022

Acad Dermatol Venereol

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“…Mast eell areas were also assessed by the digital analysis programme and revealed signifieant differences between the mean mast cell size in eases of mastoeytc5sis eompared to all other groups analysed. Tharp and eo-authors (16), using computerised morphometrie analysis of ultrastructural photography of mastocytosis, found that lesional mast cell size in patients with systemic mastocytosis was significantly larger than observed with non-systemic mastocytcjsis and normal age-matched ccjntrols. The elinieal data in our eases were insuflicient to divide our adult cases into systemic and non-systemie groups.…”

Section: Discussionmentioning

confidence: 98%

“…However, our control groups had significantly smaller mast eells than found by ultrastructural morphometry in adult eases. A possible exj^lanation for the different results may hinge on the vastly greater number of eells analysed by our digital analysis as eompared to the study using ultrastruetural morphometry (16).…”

Section: Discussionmentioning

confidence: 99%

Mast cell quantitation by image analysis in adult mastocytosis and inflammatory skin disorders

1992

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Mast cell numbers were quantitated in adult cases of mastocytosis demonstrating non-diffuse perivascular and upper dermal concentrations of mast cells. Using the Leder stain and computerised video image analysis, a mean of 382 (+/- 28 SE) mast cell per mm2 were counted in the superficial dermis in skin biopsies from 30 adult cases of mastocytosis, in contrast to a mean of 43 (+/- 5 SE) mast cells per mm2 in skin biopsies from 50 inflammatory dermatoses represented by subacute dermatitis, pigmented purpuric dermatosis, erythema multiforme, lichen planus and granuloma annulare. Ten skin biopsies showing no significant inflammation had a mean of 54 (+/- 7 SE) mast cells per mm2 in the upper dermis. The mean area of individual mast cells as assessed by image analysis in the mastocytosis group was 47.40 microns 2 (+/- 2.26 microns 2, SE) which was significantly different (P < 0.01) than the mast cell area (32.34 microns 2 +/- 2.22 microns 2, SE) in all other groups combined. Computerised video image analysis represents an alternative technique which is useful in assessing mast cell numbers and particularly mast cell size in adult cases of macular mastocytosis and in other dermatoses.

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“…The results of laboratory studies included the following values: red blood cells, 4.92 X 10'VL; hemoglobin, 124 g/L; hematocrit, 0.37; white blood cells, 13.3 X 10VL, with 0.36 neutrophils, 0.62 lymphocytes, and 0.02 monocytes; plate¬ lets, 530 X 10VL; and blood histamine, 72 ng/mL. Aspira¬ tion of his bone marrow disclosed no atypical cells, and there was no hepatomegaly, splenomegaly, or lymphadenopathy.…”

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confidence: 99%

The Familial Occurrence of Bullous Mastocytosis (Diffuse Cutaneous Mastocytosis)

Oku

Hashizume²,

Yokote³

et al. 1990

Arch Dermatol

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We studied four patients (a mother, her two daughters, and her son) with bullous mastocytosis, or diffuse cutaneous mastocytosis, whose genetic inheritance suggested an autosomal dominant pattern. The clinical characteristics included extensive bullae, numerous urticaria, pruritus, flushing, and pseudolichenified skin over all body surfaces without systemic organ involvement. The histopathologic findings disclosed a pronounced accumulation of mast cells in the dermis. Electron microscopic studies of lesional skin obtained in infancy showed round or spindle-shaped mast cells with numerous fingerlike villous protrusions. The cytoplasmic granules varied in size and shape, and the appearance of degranulation was markedly noted. In the adult, most mast cells had markedly decreased numbers of granules and cytoplasmic villi. Some cells displayed degenerative or necrotic appearances. These findings correlated well with the clinical course of these cases, which improved spontaneously over time.

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Ultrastructural morphometric analysis of lesional skin: Mast cells from patients with systemic and nonsystemic mastocytosis

Cited by 18 publications

References 26 publications

Validation of dermatopathological criteria to diagnose cutaneous lesions of mastocytosis: importance of KIT D816V mutation analysis

Validation of dermatopathological criteria to diagnose cutaneous lesions of mastocytosis: importance of KIT D816V mutation analysis

Mast cell quantitation by image analysis in adult mastocytosis and inflammatory skin disorders

The Familial Occurrence of Bullous Mastocytosis (Diffuse Cutaneous Mastocytosis)

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