Age: 15.0
Sex: F
Vaccine: HPV4, HPV9
Symptoms: Oral contraception, Oral infection, Oral pruritus, Oropharyngeal pain, Pain
Clinical narrative: Mast Cell Activation Syndrome; This literature marketed information has been received from the authors in a published article title as stated below and refers to a 20-year-old female patient. The patient was healthy in her first year of life, but at age 1, shortly after starting amoxicillin for her first earache, she suffered a diffuse outbreak of hives. By the time she started kindergarten at 4-years-old, she was already showing unusual anxiety. She routinely complained even at that age of difficulty swallowing and breathing. Her maternal grandfather died when the patient was 5 years-old, and soon after this she appeared to develop "an irrational fear of dying," and she soon began suffering panic attacks. From about age 6 to 10 years-old, she routinely suffered severe idiopathic anxiety attacks. Around, a few months after, she suffered four straight weeks of vomiting. Menarche came a few weeks later and was unremarkable. When she was 13 years old, she suffered heat stroke while playing field hockey. By age 14, she was suffering substantial dysmenorrhea.Family history was notable for a mother with obesity, a hepatic hemangioma, and polycystic ovarian syndrome (PCOS), a father who had asthma, hypertension, arthritis, and bilateral hearing loss, a brother who had asthma and severe peanut allergy, a paternal grandmother who had had a penicillin allergy and died of complications of steroid-treated rheumatoid arthritis at 42, a paternal grandfather who had arthritis, asthma, and allergies to peanuts and chocolate and had had a stroke at 91, a maternal grandfather who had had hypertension and a dissecting aorta and then died from a pulmonary embolism five days after surgery for the dissection, a maternal grandmother who had obesity, asthma, alcoholism, and a sleep disorder. The patient denied any history of smoking, significant alcohol use, or illegal substance use.   On an unspecified date, when she was 15 years-old, she got her first dose with unidentified Merck hpv vaccine administered, also reported as Gardasil (Lot #, expiration date, dose, route of administration and anatomical location of vaccine were not reported) for prophylaxis vaccination.   On an unspecified date, two months later she began suffering severe fatigue and developed a new pruritic oral sensitivity to a variety of foods. She also developed persistent non-bloody diarrhea. Multiple evaluations by her pediatrician, including blood and stool testing, were all unrevealing except for reportedly showing evidence of prior Epstein-Barr virus infection. She was told by multiple providers that her problems were all psychosomatic. She continued schedule with the second and third dose. At 16-year-old, she was referred to a pediatric gastroenterologist, who performed upper endoscopy and diagnosed eosinophilic esophagitis (EoE). Other than Proton pump inhibitors (PPIs) (which did not help any of her symptoms) and budesonide, no specific treatment was recommended, and she found that budesonide (and, later, prednisone, too) caused extreme exhaustion, irritability, and hunger, and overall she "felt like death" while on budesonide or prednisone. She then consulted a naturopath who recommended she eliminate "the top 8 allergens" from her regimen, a change which helped only "a little bit", but at the same time she started developing other issues such as brain fog, severe joint and muscle pains, temperature sensitivities, hot flashes, freezing episodes, and marked worsening of anxiety. She also started suffering presyncopal and syncopal episodes. She also had impacted wisdom teeth extracted around this time and about a month later suffered a bout of lockjaw followed by infection at one of the extraction sites. She was given codeine for this but found it ineffective, and it only seemed to cause marked weakness and memory loss. She then was evaluated at a center for eosinophilic disorders. The diagnosis of EoE was confirmed, and she was told she might have eosinophilic gastroenteritis, so bidirectional endoscopy was pursued, which found no eosinophils in either the upper or lower gastrointestinal tract, an improvement attributed to the dietary changes she had already implemented. Her consultant was not convinced at that point that she had EoE, so he recommended she drink milk for three months and then undergo repeat endoscopy, but she knew this would make her sick, so she demurred.  She then underwent allergy testing and was told by those evaluators that they had never seen anybody so allergic. She pursued a severe elimination diet, which resolved her diarrhea but did not help any of her other symptoms. At 17-years-old, she relocated to see if a new environment might be helpful, which helped somewhat for some of her symptoms, but it was stressful living with an alcoholic relative. She also was evaluated by a gynecologist shortly before moving and was told she might have polycystic ovarian syndrome (PCOS), for which a daily oral contraceptive was started. The contraceptive quickly resolved the dysmenorrhea but did not seem to help any of her other symptoms. None of her symptoms improved. Joint pains began worsening. She started suffering severe headaches. She found she was markedly intolerant of gluten and soy, exposure to which would acutely exacerbate many of her symptoms. A relative first suggested to her that mast cell activation disorder might be at the root of the patient's symptoms, soon leading to evaluation. Her chief complaints at the time of the initial evaluation were fatigue, joint and muscle pains, food sensitivities, and brain fog. Past medical history obtained at that time was additionally notable for hair loss, nausea, vomiting, cognitive dysfunction, abdominal pain, infantile eczema, allergic rhinitis, drug allergy, gastroesophageal reflux, epistaxis, and chest pain. On a full review of systems at the initial evaluation, although she denied any issues with tinnitus or adenopathy/adenitis, she endorsed a wide range of other, chronic/recurrent, episodic/intermittent and/or waxing/waning issues including subjective (rarely objective) fevers, flushing, feeling cold much of the time, fatigue (often to the point of exhaustion), malaise, headaches, diffusely migratory aching/pain, diffusely migratory pruritus, unprovoked soaking sweats (mostly nocturnal), weight loss suspected to be due to two years of diarrhea and her dietary restrictions (but fairly constant hunger), irritation of the eyes, acute brief inability to focus vision (but ophthalmologic evaluations for this had all been negative), epistaxis (both nares had to be cauterized), easy bleeding, easy bruising, sinonasal congestion, coryza, post-nasal drip, intranasal sores, oral pruritus, occasional sore throats, fear of dysphagia (but not dysphagia itself), dyspnea (acute spells of this had driven her to the emergency department (ED), where evaluations had consistently been unrevealing), palpitations, non-anginal chest discomfort/pain, gastroesophageal reflux in the past, nausea, rare vomiting, diarrhea alternating with constipation (much more so the former), diffusely migratory abdominal discomfort including (usually post-prandial) bloating, urinary frequency, diffusely migratory weakness, a sense of diffusely migratory edema in her joints but no physically visible edema, diffusely migratory tingling/numbness (typically about the distal extremities), orthostatic and non-orthostatic presyncope, syncope, cognitive dysfunction (particularly memory, concentration, and word-finding), waxing/waning hair loss (sometimes severe), odontalgia despite good attention to dental hygiene, poor nail growth, diffusely migratory rashes (typically patchy macular erythema), "hives all the time" (most commonly on her abdomen), insomnia, frequent waking, non-restorative sleep, hypersomnolence, sleeptalking, occasional sleep paralysis, panic disorder, anxiety, laxity in multiple joints (but a spontaneously demonstrated Beighton score of 0/9), unusually vigorous reactions to insect bites (especially mosquito bites), and poor healing in general.   At the time of the initial evaluation, the patient listed her current medications as sertraline 50 mg once daily and a daily oral contraceptive taken principally to address her PCOS. She listed her current allergies as hives to amoxicillin and "feeling horrendous" to prednisone and budesonide. Outside records indicated drug allergies to penicillins and sulfa. Physical examination at the initial evaluation found an outwardly healthy appearing, trim young woman in no apparent distress, pleasant, cooperative, fully alert and oriented, easily independently ambulatory, presenting with her obviously appropriately concerned/supportive mother. Vitals were notable for BP 117/77, pulse 93, and weight 116 pounds (down from a peak of 140, she said). Key findings were her comfortable general appearance, HEENT benign, no plethora/pallor/diaphoresis/jaundice/bleeding/bruising, a very minimal extent of folliculitis about the forehead and upper back but otherwise no rashes at all (nor any hives), neck supple, no JVD or thyromegaly or carotid/abdominal/renal/femoral bruits, no palpable adenopathy or tenderness at any of the usual node-bearing sites, clear lungs, regular heart with no adventitious sounds, abdomen scaphoid and completely benign, no peripheral edema, and an unremarkable neurological exam. No costovertebral angle or spinal column tenderness was evoked on palpation and percussion about these areas. On a light scratch test on the upper back, mildly bright dermatographism (erythroderma only, no hives) quickly emerged and was fully sustained when last checked 10 minutes later.  Literature report:   Upon internal review the of Mast Cell Activation Syndrome (MCAs) was considered as medically significant.   This is one of several report from the same literature article.   A copy of the published article is attached as further documentation of the patient's experience.