Cases Of Urticaria Pigmentosa Research Articles

Perivascular mast cells in urticaria pigmentosa.

We have quantified perivascular mast cells in cases of urticaria pigmentosa, urticaria, and dermal hypersensitivity reactions. To facilitate reproducibility, the mast cells were counted for a precisely defined vessel unit. These vessel units were divided arbitrarily into those < or = 55 microns and > 55 microns in largest diameters. Urticaria pigmentosa showed an average of 6.4 +/- 1.9 and 22.8 +/- 13.2 mast cells for vessel unit < or = 55 microns and > 55 microns, respectively. Urticaria yielded a lower number of mast cells: 1.5 +/- 0.2 and 2.9 +/- 0.9 for the same respective vessel units. Dermal hypersensitivity reactions revealed an average of 1.6 +/- 0.4 and 2.2 +/- 0.7 mast cells, and the normal skin showed 1.5 +/- 0.3 and 2.4 +/- 0.6 mast cells for each of the vessel units of < or = 55 microns and > 55 microns. The perivascular mast cell distributions of urticaria pigmentosa are statistically different from those of urticaria and dermal hypersensitivity reactions with p < 0.0001. No statistical difference was noted between urticaria, dermal hypersensitivity reactions, and normal skin. The percentages of vessel units < or = 55 microns with > or = 5 mast cells and vessel units > 55 microns with > or = 10 mast cells were determined for each case. The average percentage of vessel units for the former and latter in urticaria pigmentosa was 82.6% and 58.9%, respectively. Urticaria yielded 0% and 0.2%, respectively. None of vessel units in the dermal hypersensitivity reactions or normal skin contained more than 5 mast cells in vessel units < or = 55 microns and more than 10 mast cells in vessel units > 55 microns. Cases of urticaria pigmentosa can be distinguished from other cutaneous eruptions containing mast cells using a simple counting technique on Giemsa stained sections.

Immunoreactivity of normal and neoplastic human tissue mast cells with macrophage-associated antibodies, with special reference to the recently developed monoclonal antibody PG-M1

There is increasing evidence in favor of the hypothesis that human tissue mast cells (MCs) are progeny of hemopoietic stem cells and are closely related to cells of the mononuclear phagocyte system. To test this hypothesis we investigated the immunoreactivity of normal/reactive MCs in 12 lymph node and tumor specimens and neoplastic MCs in 27 tissue samples from patients with various types of mastocytosis (urticaria pigmentosa, n = 13; cutaneous mastocytoma, n = 4; systemic mastocytosis, n = 6; and malignant mastocytosis, n = 4) with a panel of eight antibodies that stain macrophages or immune accessory cells and are reactive on routinely processed (paraffin-embedded, formalin-fixed) tissue. The MCs were stained by three of the macrophage-associated antibodies (namely, KP1 [CD68], Ki-M1P, and PG-M1 [CD68]), but were not stained by three other antibodies (namely, HAM56, MAC387, and LN5) or antibodies detecting immune accessory cells (DAKO-CD35 and anti-S-100 protein). While KP1 stained normal/reactive and neoplastic MCs in all the specimens investigated, Ki-M1P stained neoplastic MCs in nearly all the cases of mastocytosis but did not stain normal/reactive MCs. PG-M1 also failed to stain normal/reactive MCs and stained MCs in only approximately half of the specimens from cases of mastocytosis. Among these were most of the cases of systemic and malignant mastocytosis, but only a minority of the cases of cutaneous mastocytosis and a very few cases of urticaria pigmentosa. To summarize, (1) MCs display immunohistochemical staining properties resembling those of cells of the mononuclear phagocyte system but not those of macrophage derivatives belonging to the immune accessory cell compartment, and (2) PG-M1 and Ki-M1P are unique among the macrophage-associated antibodies investigated in that they do not stain normal/reactive MCs but exhibit preferential reactivity with the more atypical MCs in cases of systemic and malignant mastocytosis.

Epidermal Mast Cells

Tissue from 134 patients with neurodermatitis and prurigo, pseudoepitheliomatous hyperplasia, pemphigus, and urticaria pigmentosa was examined qualitatively for epidermal mast cells. Epidermal mast cells were found in all of the diseases that were studied except dermatitis herpetiformis. Pemphigus vegetans and dermatitis vegetans were frequently associated with the presence of epidermal mast cells. In other pseudoepitheliomatous diseases, such as tuberculosis verrucosa cutis, blastomycosis, and bromoderma, epidermal mast cells were present in many cases. Four of eight patients with acral dermatitis with elevated IgE blood levels had intraepidermal mast cells; the number was much lower in patients with usual neurodermatitis and prurigo nodularis. Only two of ten cases of alopecia mucinosa showed epidermal mast cells. A single epidermal mast cell was found in ten cases of urticaria pigmentosa. Chronic inflammation associated with epidermal cell proliferation appeared to correlate with the presence of epidermal mast cell.

Histochemical Study of Mast Cell Changes and Alkaline Phosphatase Activity in Urticaria Pigmentosa

Five cases of urticaria pigmentosa in patients of different ages and of both sexes were studied for mast cell changes and alkaline phosphatase activity. It has been found that mast cells were very rich in the enzyme as indicated by the black colour after the Gomori’s stain.

5-hydroxytryptamine (serotonin) and 5-hydroxytryptophan in mast cells of human mastocytosis.

With use of the formaldehyde condensation method of Flack and Hillarp, skin specimens taken from one patient with solitary mastocytosis and four patients with urticaria pigmentosa were examined under a fluorescence microscope. In the case of solitary mastocytosis, some of mast cells in the dermis showed intense yellow fluorescence, which was no doubt due to the presence of serotonin or its immediate precursor, 5-hydroxytryptophan. In four cases of urticaria pigmentosa, no yellow fluorescence could be detected on the mast cells infiltrated around the vessels. However, there were a few mast cells which showed yellow fluorescence, in the dermis of a patient with urticaria pigmentosa. From a biochemical point of view, human mast cells, too, seem to consist of two distinctly different types of cells: monoamine-forming and non-amine-forming mast cells.

Histochemical demonstration of acid phosphatase activity in various blood cells.

Acid phosphatase activity of various blood cells were histochemically observed on peripheral blood and bone marrow smears using the azo dye method which had been reported in my previous paper, and following results were obtained. 1) In the neutrophilic granulocyte series, the largest acid phosphatase score was obtained in immature cells, which might include promyelocytes, myelocytes, and metamyelocytes. Mature neutrophils were stained moderately, and blast cells showed weak staining. 2) Eosinophils showed strong activity, and basophils seemed to have weak activity. 3) Monocytes disclosed stror4g activity, and lymphocytes slight activity. 4) Erythroblasts had slight activity, and it seemed that they lost gradually the enzyme activity with the advance of cell maturation. 5) In plasma cells, reticulum cells and megakaryocytes, strong activity of the enzyme was demonstrated. 6) Mast cells showed moderately strong activity on the bone marrow smears from a case of urticaria pigmentosa.

BULLOUS URTICARIA PIGMENTOSA IN INFANCY.

Two cases of urticaria pigmentosa in infants are presented. These children initially developed a generalized vesiculobullous eruption without pigmented nodules or macules. Review of the literature revealed six similar reported cases. Clinical features, histopathology, and diagnosis are discussed.

HODGKIN'S DISEASE CONCURRENT WITH URTICARIA PIGMENTOSA.

A case of urticaria pigmentosa is reported in which systemic progression seems to have become a mixture of mastocytosis and Hodgkin's disease. The question of chance association or more meaningful relationship is unresolved.

Generalized flushing of the skin with urticaria pigmentosa.

In 1869, Nettleship 1 described an unusual type of urticaria which bears the name urticaria pigmentosa. For the next 80 years it was regarded as a comparatively rare skin condition characterized by yellow or brown macules or nodules, which, when rubbed vigorously, produced a whealing or urtication localized to the pigmented areas. Apart from the occasional report of rare solitary nodular and bullous lesions, the only other significant observation during this period was the finding of Unna, 2 in 1887, that the skin manifestations of urticaria pigmentosa were characterized by a marked infiltrate of tissue mast cells. In 1949, Ellis 3 reported the first case of urticaria pigmentosa in which an autopsy was performed. This one-year-old Negro girl had the typical skin lesions of urticaria pigmentosa from birth, associated with gastrointestinal upsets and hepatosplenomegaly. The autopsy revealed increased numbers of mast cells in the areas of portal

Urticaria pigmentosa; mastocytosis; a consideration of various manifestations.

As a graduate student it was a happy day when one first saw a case of urticaria pigmentosa. Classically it would be a child with scattered macular pigmentation which would urticate when the lesions were irritated (Darier's sign). This was satisfying and for the moment represented all there was to know of the clinical manifestations of this condition. However, with increasing clinical experience, it became increasingly clear that urticaria pigmentosa was not a single simple condition. The concept of a typical case of urticating macular pigmentation (Fig. 1), beginning usually in the first six months of life (congenital) and tending to disappear at puberty, had to be expanded. This broadened concept had to include solitary lesions which had the appearance of some unusual type of nevus (Fig. 2) or xanthoma (Fig. 3); a bullous type (Fig. 4) with solitary or disseminated lesions; a nodular or

Bullous Urticaria Pigmentosa

While macular, maculonodular, and nodular forms of urticaria pigmentosa are well recognized, the occurrence of vesicular and bullous lesions in this disease is regarded as a rarity (Unna, 1896; Pusey, 1907; Stelwagon, 1918; Macleod, 1920; Ormsby and Montgomery, 1948). But it is possible that vesicular and bullous lesions in urticaria pigmentosa may be commoner than has hitherto been supposed, since cases will undoubtedly have been recognized but not reported in the literature and also since purely bullous forms of the disease may well escape recognition when biopsy has not been performed or even perhaps when metachromatic staining methods have been omitted. Little (1905-1906), reviewing the literature since the first description of urticaria pigmentosa by Nettleship (1869), noted four cases in which bullous lesions had been present. Finnerud (1923), discussing cases of urticaria pigmentosa which had been reported since 1906, found eight instances in

Urticaria pigmentosa: Report of a case of the bullous variety in a newborn infant

Summary 1. A case of urticaria pigmentosa in an infant is reported. 2. The special features of interest in this case are the presence of the full-blown disease picture at birth and the bullous character of many of the lesions. 3. Attention is called to the absence of cases reported in the recent pediatric literature. For that reason the disease picture and its pathognomonic clinical sign (urtication on stroking of the lesion) are described. 4. The presence of mast cells in abnormal numbers in the corium on histologic examination is pointed out as a characteristic pathologic feature.

URTICARIA PIGMENTOSA (NODULAR TYPE) WITH A SUMMARY OF THE LITERATURE

Some manifestations of urticaria pigmentosa are rarely seen. In his excellent summary of the literature up to and including 1905, E. Graham Little 1 pointed out that only sixteen cases of urticaria pigmentosa had been reported in America out of a total of 154, while the English literature contained sixty-two cases, the German thirty-nine, and the French twenty-four. He showed that of 138 cases in which the sex was recorded, eighty-six, or 61.7 per cent., occurred in males and fifty-two in females, and that of 142 cases in which the age was given, seventy-nine, or 55 per cent., began at or before the sixth month of age and twenty-two, or 14 per cent., began after puberty (13 years). Also, of 123 cases in which the type of lesion was stated, eighty-three were of the macular, twelve of the nodular and twenty-eight of the mixed type. The disorder proved to be

Case of Urticaria pigmentosa in a Girl aged 10

Case of Urticaria pigmentosa in a Girl aged 10

Sections from a Case of Urticaria Pigmentosa in an Adult

Sections from a Case of Urticaria Pigmentosa in an Adult