Diffuse cutaneous mastocytosis with multiple cutaneous mastocytomas

Abstract

A patient with diffuse cutaneous mastocytosis, present since birth, developed several cutaneous mastocytomas without any symptoms after puberty. Although the histological picture of the tumours, showing nuclear atypia, mitotic figures and varying metachromasia of the granules, raises suspicion of systemic involvement, this has not appeared so far. The absence of symptoms could be due to the synthesis of an abnormal secretory product by the mast cells, which is supported by the absence of oedema and dilated blood vessels in the presence of extensive degranulation and by the unusual ultrastructural picture of the mast cell granules, i.e. little electron density with the almost complete absence of lamellar and crystalline structures.

Presentation of cutaneous mastocytosis in 173 children

“…Similar nodules have been reported in the literature. 23 The palms and soles were involved in one of our cases ( Fig. 10) and this has also been reported previously.…”

Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature

“…The disease may present with various clinical features in children, teenagers and adults. Pseudoxanthomatous lesions, DCM with large, tumour‐like mastocytomas and pachydermia with extensive skin folding have been reported 9,14,24–30 . In our series, blistering variants were found in infants and small children whereas pseudoxanthomatous skin lesions developed after puberty in the patient who initially presented with infantile small vesicular form of DCM.…”

Clinical aspects of paediatric mastocytosis: a review of 101 cases

“…DCM, the most severe form of CM, which presents as erythroderma involving almost the entire skin. This form usually occurs at birth or in early infancy 10,11,22 . We observed widespread, haemorrhagic blisters which were the first clinical presentation of DCM or occurred in the first year of life.…”

“…This form usually occurs at birth or in early infancy. 10,11,22 We observed widespread, haemorrhagic blisters which were the first clinical presentation of DCM or occurred in the first year of life. It is noteworthy that bullous lesions do not represent a specific subset of the CM phenotype.…”