Atlas of dermatopathology: Urticaria pigmentosa

+ Introduction

+ Literature

+ Support

+ Skin biopsy

+ Non-tumorous skin diseases

+ Skin tumors

+ Mesenchymal tumors

+ Cysts of the skin and subcutis

+ Soft tissue lesions

+ Some lesions of the oral cavity

+ Dermatovenerology

+ Collection of histological slides by prof. Günter Burg

+ Collection of histological slides by prof. Werner Kempf

+ Collection of histological slides of dr. Ram Chandra Adhikari

Urticaria pigmentosa

Introduction

This process is caused by accumulation of mastocytes within the dermis. There are 5 different types of this disease, some of them malignant. The amount of mastocytes is increased, but in some cases the increase is not conspicuous and cannot be deected without using special stainings.

Etiology, pathogenesis

increased amount of mastocytes intradermally

Clinical signs

generalized process, mostly on the trunk and extremities
small macules and papules
red or red-brown color (increased epidermal melanin after histamin stimulation)
Darier's sign: papules reacts on mechanic irritation by urtica and erythema
pruritus, sometimes systemic symptoms: migraine, nausea, GIT spasms, hypo- and hypertension
hepatosplenomegaly rarely, infiltration of the bone marrow, (mastocytic) leukemia
some cases self-limited, some malignant

Pictures

Urticaria pigmentosa, chest: Urticaria pigmentosa, Clinical picture (936)

Urticaria pigmentosa, trunk: Urticaria pigmentosa, Clinical picture (937)

Urticaria pigmentosa, back: Urticaria pigmentosa, Clinical picture (938)

Urticaria pigmentosa: Urticaria pigmentosa papular nonbullous, Clinical picture (6372) Urticaria pigmentosa papular nonbullous, Clinical picture (6370) Urticaria pigmentosa papular nonbullous, Clinical picture (6371)

Further examples of urticaria pigmentosa:

Histology

Typical papular urticaria pigmentosa is characterized by dense aggregates of monomorphous mastocytes intradermally (can be diagnosed using HE staining). Other cases require special staings and clinical data. Scattered eosinophils are often present.

Special staings: Giemsa, kresyl violet, toluidin blue, naphtol-ASD chloracetate esterase.

Pictures

Urticaria pigmentosa: Urticaria pigmentosa, HE 10x (430) Urticaria pigmentosa, Giemsa 10x (429) Urticaria pigmentosa, kresyl violet 100x (428) Urticaria pigmentosa, Giemsa 100x (427)

Next case: Urticaria pigmentosa, HE 10x (433) Urticaria pigmentosa, HE 40x (437) Urticaria pigmentosa, Giemsa 10x (432) Urticaria pigmentosa, Giemsa 40x (436) Urticaria pigmentosa, Giemsa 100x (431) Urticaria pigmentosa, kresyl violet 10x (434) Urticaria pigmentosa, kresyl violet 20x (435)

Next case, with dense accumulation of mastocytes: Urticaria pigmentosa, HE 20x (423) Urticaria pigmentosa, HE 40x (424) Urticaria pigmentosa, kresyl violet 40x (425) Urticaria pigmentosa, kresyl violet 100x (422)

Urticaria pigmentosa, Darier's sign Urticaria pigmentosa, Darier's sign, HE 40x (1438) Urticaria pigmentosa, Darier's sign, HE 20x (1439)

Mastocytoma, chloracetatesterase: Urticaria pigmentosa, CHAE 20x (1436)

Mastocytoma, c-kit: Urticaria pigmentosa, c-kit 20x (1437)

Another case, semithin section: Urticaria pigmentosa (semi-thin section), toluidin blue 100x (426)

Electron microscopic pictures: Urticaria pigmentosa, electron microscope (625) Urticaria pigmentosa, electron microscope (626) Urticaria pigmentosa, electron microscope (627) Urticaria pigmentosa, electron microscope (628)

Next case, less conspicuous form with small number of irregular, spindle shaped mastocytes: Teleangiectasia macularis eruptiva perstans, HE 20x (414) Teleangiectasia macularis eruptiva perstans, HE 40x (417) Teleangiectasia macularis eruptiva perstans, Giemsa 20x (415) Teleangiectasia macularis eruptiva perstans, Giemsa 40x (416)

Macular urticaria pigmentosa, inconspicuous if stained with HE: Urticaria pigmentosa, papular, HE 40x (3566) Urticaria pigmentosa, macular, kresyl violet 40x (3531)