Hyaline vascular‐type unicentric Castleman disease presenting as a subcutaneous nodule in a child

Abstract

nal pain, spontaneous ecchymosis, headaches and ear rubefaction (exceptionally described). Darier’s sign was negative in most patients (5/8 presented dermatographism). We concluded that these forms of “telangiectatic mastocytosis” are not rare and probably unrecognized. It must be suspected in patients with inexplicable telangiectases associated with polymorphous symptomatology. Classical TMEP may be considered as a more infrequent subset among telangiectatic mastocytosis. Perhaps many cases are not diagnosed due to hesitation in performing biopsies of trivial lesions and the wide spectrum of additional symptomatology, which may be confused with other diverse unrelated conditions (i.e. hereditary hemorrhagic telangiectasia, unilateral nevoid telangiectasia, inflammatory ear conditions and many others in which telangiectases are not considered when making a diagnosis). In my experience, these symptoms are usually accepted by most patients and recognized as something inherent to themselves; they are often derived to other specialists, including neurologists, gastroenterologists, cardiologists and even psychiatrists, with no final diagnosis due to the improbable knowledge to link their predominant symptoms with their telangiectases. These symptoms must be obtained by focused interrogation. Repeated epistaxis and spontaneous tachycardia are some of the more frequent I have observed subsequently. Memorable ones were a female diagnosed with rosacea: while being treated with an antihistaminic for acute urticaria she told me that doing so had ceased a 1-year persistent diarrhea; and another woman with telangiectases, under psychiatric control for repeated fainting when nervous (sign exceptionally described). As Moon et al. have stated, there is no cut-off value for the increased number of mast cells; it would be useful to know their average count of mast cells (9400) because it has been the initial methodology used in many previous reports. I believe that telangiectatic mastocytosis does exist, requiring a high index of suspicious for its diagnosis. It apparently runs a benign course, and its symptomatology greatly improves with an antihistaminic, oral sodium cromoglycate or both.