A Clinical and Histological Study of Urticaria Pigmentosa: Relationships between Mast Cell Proliferation and the Clinical and Histological Manifestations

Abstract

In 21 cases of urticaria pigmentosa (UP), clinical and histological observations and evaluation of mast cell (MC) volume density in the lesions using a morphometric point counting method were performed. The mutual correlations between clinical and histological findings were statistically assessed by a method of multiple regression analysis. Clinical items employed in the analyses were as follows: sex, the age of onset, the age of biopsy, the biopsy, the duration of lesions, the type of skin lesions, sites involved, the presence or absence of Darier's sign of Darier's sign and symptoms, and serum histamine level. Histological items included the localization and infiltration pattern of MC, the level of basal melanosis, the presence or absence of inflammatory cell infiltration, and the MC volume density in the lesions. Statistical significance of the partial regression coefficients was obtained for 6 pairs of the criteria (p = 0.05), including the age of onset and the age of biopsy, the age of onset and the level of basal melanosis, the duration of lesions and the level of basal melanosis, and the type of skin lesions and the level of basal melanosis. No significant correlations were observed between the MC volume density in the lesions and any of the other items. These results suggest that the basal melanosis in a UP lesion may not be a direct reaction to the transitory massive infiltration of MC, but rather be due to a relatively long-term effect of MC infiltration. Furthermore, the MC volume density in the lesion is not likely to be an important factor in determining the clinical manifestations of a UP lesion.

Paediatric mastocytosis: a systematic review of 1747 cases

“…Cutaneous features (Fig. e and Table S1) were reported in 70% of cases in the series . Darier sign was present in 90% of patients.…”

“…The type of initial lesions in patients with UP‐type lesions was reported in 35% of cases. These were macules (20%), papules (12%), maculopapules (41%), plaques (15%) or nodular‐type lesions (12%) . In four studies using the Hartmann and Henz classification, 63% of patients presented with maculopapular lesions, 21% with plaques and 16% with nodular‐type lesions …”

“…In total 90 articles were available. This included 43 series, 21 case reports with outcome data (> 3 years), 15 articles involving twins (one of which was also included in the 43 series), corresponding to 31 cases and 12 articles concerning MCL or MCS, corresponding to 12 cases (two concerning the same patients) . A two‐generation history of mastocytosis was mandatory for the positive familial history criterion.…”

Solitary mastocytoma mimicking granuloma faciale

“…Microscopically, mastocytoma is usually characterized by a dense diffuse or nodular mast cell infiltrate that may reach the subcutaneous tissue. An accompanying sparse to mild mixed inflammatory infiltrate may also be present . Here, we report, to the best of our knowledge, the first case of cutaneous mastocytoma microscopically resembling granuloma faciale (GF).…”

Mastocytosis and Skin Cancer: The Current State of Knowledge

“…Interestingly, intradermal injection of a CGRP antagonist was proven to avert this immunosuppressive effect of CGRP [ 114 ]. Additionally, CGRP was demonstrated to stimulate the proliferation of melanocytes and promote melanin production, which could potentially play a role in the observed basal hyperpigmentation in individuals with CM [ 115 ]. The impact of CGRP on melanoma is uncertain; CGRP was shown to inhibit melanogenesis and induce apoptosis in vitro [ 116 ].…”