Localized form of mastocytosis. Degranulation must be
avoided during all anesthetic procedures (physiological and psychological
Urticaria Perstans Hemorrhagica with Pigmentosa;
Localized Infantile Mastocytosis; Nettleship E Disease type I;
Although rare, it is most often seen in children, however it can also occur in adults. Affects males and females equally.
Both autosomal and recessive inheritance have
been evocated, however sporadic cases have also been reported.
Unknown. When mast cells proliferate, histamine
and heparin are released into the skin. Mastocytosis presents with characteristic
skin lesions of urticaria and telangiectasis.
Urticaria pigmentosa is a localized form of
mastocytosis. It is generally benign and is usually self-limited. Diagnosis
is clinically evocated in a child older than age 2 years with skin lesions
affecting the neck, arms, legs, and trunk. The rash consists of
reddish-brown spots that turn into hives when they are rubbed hard or
scratched. Certitude diagnosis can be obtained with skin biopsy.
Skin lesions can also include bullous
mastocytosis and telangiectasia macularis eruptiva perstans (multiple hyperpigmented
telangiectatic macules, located primarily on the trunk and on the extremities). In the
early form, resolution of the condition by adolescence or early adulthood is common. In
the form beginning after 5 years of age, the abnormal collections of mast cells sometimes
involves other organs. It can affect bone, gastrointestinal tract, lymphatics,
spleen, and liver, resulting in clinical signs such as abdominal pain or gastric ulcer. It
is even possible to observe an overgrowth of mast cells progressing to a malignant
out-of-control process called mast cell leukemia. Mast cells can abruptly release
vasoactive mediators, such as histamine, heparin, and prostaglandins, under some physical
stresses or in response to drugs. Severe general reaction with shock can occur.
Obtain full medical and physical history. One must
review the signs and symptoms of previous cardiovascular instability, i.e., hypotension, loss
of consciousness, vomiting, and severe flushing and sweating. Evaluate organ involvement
Both regional and general anesthesia can
produce life-threatening complications. Avoid mastocyte degranulation (e.g., mild
temperature in the operating room), psychological and physical stress.
Resuscitation drugs should be present in the operating room. Careful with the insertion of nasal
tubes (easy bruising and nose bleeds).
Many drugs have been associated with
mast cell degranulation (lidocaine, morphine, oxymorphone, codeine,
d-tubocurarine, metocurine, acetylsalicylic acid, etomidate, thiopental,
succinylcholine, enflurane, isoflurane methylparaben and para-aminobenzoic
acid). Consider use of intradermal skin tests to predict which medications may cause problems
and those that may be used. Regional anesthesia should be preferred when possible because it
restricts the use of several different drugs. (One should prefer amide-type over
ester-linked local anesthetics.) Consider preoperative administration of
H1 and H2 blockers and steroids. Preoperative sedation can be
useful (e.g., hydroxyzine) to reduce patient stress. Avoid atropine. Muscle relaxants are not
contraindicated but it is recommended to choose those ...