Age: 41.0
Sex: M
Vaccine: ANTH, FLUX
Symptoms: Xeroderma
Clinical narrative: This 100% disabled patient reported being in "great" health being able to run 5 miles per day with 50 lb pack, ran in marathons and was units team leader prior to receipt of AVA #1 and influenza vaccinations on 1/11/2003. Within 24 hours he reported experiencing subjective fever, malaise, generalized body aches lasting 1-2 days. (A review of available PMH identified concurrent evaluation for chronic bilateral knee/ankle pain- has had multiple bilateral knee/ankle surgeries since 10/2002). Approximately 1 hour after receipt of AVA #2 on 1/25/2003 he complained of similar body aches, malaise and a mild buzzing/vibratory feeling in ears and all over his body x 3-5 days. On 2/8/2003 he received AVA #3 with return of malaise, body aches and buzzing sensation (as above within 24 hour after vaccination). Approximately 1 week later he complained of increased malaise with onset of nausea, weakness, and diarrhea. His diarrhea was explosive watery (clear-brown) 3-4 times a day for 1-2 days before returning to soft formed stools (but not normal). Approximately 4-6 weeks later (March 2003) he reported his weight was down 50 lbs. He was eating but not as much and continued normal ADL, prep for leaving the country and was in physical therapy for knee/ankle injury. He worked as a Registered Nurse. Approximately 3 months later (May 2003) he advised his weight was stable. His wife advised he snored, was restless kicking and flailing about fighting in his sleep and had night sweats. She advised he appeared exhausted and was irritable. Concurrently he noted onset of a quarter size (1/2 inch to 1/2 dollar size) raised clear-yellow tinged fluid filled blister to mid-back lumbar region of his spine. This blister would last 2 days and reflare every 2-3 months with a gradually extending fine red-purple skin discoloration with each flare up. He reported with each "flare" he experienced malaise, fatigue, sweats, nausea, vomiting, fever, body aches, headaches, tinnitus, blurry vision and his chest hurt especially with breathing in. Each flare would last 5 days and resolve on own. (There were no 2003 medical records available to review). In July 2003 he reported he underwent surgery for chronic right knee-ankle pain. He also had chronic left knee pain. Treatment included bilateral knee braces, physical therapy and a variety of pain medications (NSAID, LORTAB to MS pill). His medical records (2/29/2012) documented other events occurring in 2003 during AVA administration included: counseling for traumatic memories of bridge collapse May 2002 and resulting ankle injury and house fire in 7/2003 and preparing for upcoming trip. He left in November 2003 for 1 year. This patient reported similar body aches, malaise and tingling sensations following AVA #4 on 7/1/2003, AVA #5 on 1/3/2004, and AVA #6 on 10/12/2004 that lasted about 5 days each with increased severity of arthritic discomfort and back skin discoloration. He did not recall if he had any adverse events following 11/23/2003 influenza and meningococcal vaccinations. A review of patient's 2005-2013 Electronic Medical Records documented medical evaluations every 1-2 week by multiple providers (PCP, physical therapy, pain management clinic, case worker, and various specialty providers) for above medical complaints. (There were no records prior to 2005 to review). Symptoms onset was reported to begin 2-6 weeks after receipt of AVA #3 and prior to his 11/2003 trip. Symptoms included weakness, malaise, joint pain with swelling and redness, Raynaud's like phenomenon, and a butterfly rash with flu-like symptoms lasting 4-7 days that would resolve spontaneously or with steroid use and has a skin discoloration to his lower back. He advised when this area gets warmer it develops a deeper bluish pattern and even blisters at times. His appetite is poor during these flare ups (weight stable about 261 lbs), and they are occurring about twice a month with severe fatigue and pain and longer durations (2011-2012). His erythematous reticular rash was diagnosed a possible livedo reticularis and vasculitis. A comprehensive review of his electronic medical records started in 2005 with providers documenting recurrent visits for chronic pain to bilateral knees, shoulders, neck, lower back, and other joints with diagnosis of traumatic arthritis (on Diclofenac and morphine tablets 9/27/2005), and chronic DJD (1/24/2006) to lower back, an MRI (2/6/2006) showing mild focal discal protrusion to posterior lateral region right L5S1, mild degenerative changes to articular facets mid lumboscacral region, and mild broad based disc bulge to L5S1. An evaluation 6/14/2006 for chest pain documented "skin with no rash or petechiae". An hsCRP on 6/21/2006 was <0.20. An exam on 11/11/2006 diagnosed dry scaling skin lesion to hands and fingers with diagnosis of hand eczema and treated with IM SOLU-MEDROL and topical Triamcinolone cream; in 12/2006 his hands cracked and bleed and he was diagnosed with xeroderma. On 1/25/2007 he reported recent epidural steroid injection for lower back and radiating leg pain with providers noting back was nontender with reticular rash. He advised providers told him he had "seronegative RA". A rheumatology consult on 5/1/2007 documented complaints of morning stiffness to wrists, hands, knees, ankles with swelling that took 1-2 hours to easing. His exam documented "all fibromyalgia trigger points positive" with reticular rash to lower back. Diagnostic and lab studies reported an old x-ray with mild DJD to back and right hip, uric acid 4.9, ESR 3, CRP <0.2, ANA/RF negative, Lyme test negative. He was diagnosed with fibromyalgia (FMS) with "no evidence of inflammatory arthritis". Diagnoses also included arthralgia with DJD treated with steroid and NSAIDs, and bradycardia (40-56) of unknown etiology (has had comprehensive cardiac evaluation (labs, echo, ETT, cardiac cath). Further evaluation of his skin disorder continued into 2008 with complaints of non-itchy rash with blisters x 2 years (using cold packs to back) with right hand rash, scales and fissures since 2003 with diagnoses of erythema ab-igne-like cold injury and tinea manuun given. On 7/9/2008 dermatology exam documented a large water blister 1-2 inches on back that is painful when flares and itches. Blister drying and present x 2 weeks with constitutional symptoms of headache, nausea, vomiting, diarrhea, joint pain, lower extremity edema, lower back pain and shortness of breath that ease with IM TORADOL and SOLU-MEDROL followed by tapering dose of oral prednisone (20 mg x 1 week; 15 mg x 1 week, 10 mg x 1 week, and 5 mg x 1 week). His exam showed an "unusual pattern of connected confluent violaceous patches - reticular pattern, atrophic, slightly scaly, blanchable and erythematous with a 2.5 cm healing vesicle like blister above right buttock". Bunch biopsies of site showed non-specific subepidermal bulla. In 2009 he described a widespread "Crushing-pressure feeling" to his muscles that feels like "bursitis" in his joints that did not respond to CoQ10, CYMBALTA and LYRICA (4/2/2009) so he discontinued this treatment for fibromyalgia but does take omega-3 and magnesium. Rheumatology on 4/3/2009 reported change in symptoms to include rash to back/buttock that was pruritic, painful blisters up to 2 inches which would scab and then peel. Underlying skin is raw and states hands would be hypersensitive with swollen joints/digits and has increased redness to his face and anterior trunk. He also reports numbness and tingling to fingers/hands in am. Spells were 1-2 times every few months but now 2-3 times a month lasting for 2-3 days. Prednisone tapers help. Lab review 6/06 CPR 75.85 (record not found, did fine 6/21/06 hsCRP of 0.20); 6/08 cANCA and pANCA negative; 10/08 RF negative, ESR 3, CRP 0.48, heavy metals negative; 11/08 ANA negative; 12/08 Vit D25-OH was 22, ESR 4, CRP 0.3, CPK 178, hepatitis B/C negative; 1/09 CBC and CMP wnl; 2/09 UA VMA, PORPH, and 5HIAA negative (note: not all above labs found except in provider notes). Negative ANA, ANCA panel (nl anti MPO and antiPR3), negative aPL, normal C3 and C4 (no CH50), and normal SPEP. Providers gave a working diagnosis of FMS and livedo reticularis (possible vascular origin IE vasculitis or antiphospholipid) with differential diagnoses of erythromelalgia vs CRPS vs autoimmune disease vs ubiquinone deficiency (doubt as no change symptoms with CoQ10), bullous pemphigoid, pemphigus vulgaris, Sjorgren's w'livedo, antiphospholipid antibody w/livedo, cryofibrinogen dz, autoimmune vasculitis, inflammatory bowel dz, mastocytosis, or paraneoplastic dx and others. Patient wondered if pain now related to AVA vaccines. He was referred to gastroenterology in 2009 for steroid induced gastritis where he complained of long standing, intermittent, nausea/vomiting (no heartburn), diarrhea with stools up to 12 x per day with no blood, and aversion to eating with dysgeusia prior to and during "flares" of dermatologic and arthritic symptoms. His exam was normal with normal colonoscopy with no signs of celiac, IBD, cancer, etc. He did have an internal hemorrhoid. His UGI endoscopy was also normal. His Vit D level was normal at 32.4 and free testosterone was low at 1.8 (11/2/2009). April - October 2011 labs listed hsCRP at 0.4, ESR 3 then 2, TSH 2.33, normal chemistry (except chol) CBC, MRSAPCR and MRSAAGR both negative. His chronic pain was being followed by pain management clinic since 2005, and in December 2005 he was hospitalized x 3 days for severe fatigue, myalgia-athralgia, shortness of breath, and chest pain. His CT chest = pulmonary arterial hypertension, no pulmonary embolism; CT abd/pelvis = fatty liver infiltration; venous DVT LE negative; echo = mild increase PA systolic pressure; EKG nonspecific ST-T changes lateral leads, possible T wave inversions with 1 15 second burst of SVT (150-160 bpm- he did feel heart race) on Beta blockers, hyperlipidemia. Labs: normal BNP, d-dimer, troponin I x3, CK/CKMB, ESR, hsCRP, CBC, CMP, TSH, B12, COAG profile, PT/PTT/INR, etc. His discharge diagnoses included pleurisy, dyspnea on exertion due to pleurisy/pulmonary hypertension, and pulmonary hypertension. Secondary diagnosis included hypertension, hyperlipidemia and autoimmune with symptoms of lupus, rheumatoid arthritis, and/or Raynaud's since 2003, and erythematous reticular rash of uncertain etiology (12/30/2011). In February 2012 he was referred to endocrinology for hypogonadism and was placed on testosterone patch which made him feel better. He advised his back rash flare used to occur every 6 months and now every other week. He also complained of changes in blood pressure. Dermatology on 7/31/2012 "diagnosed bullous erythema ab igne". This is a rare presentation of erythema ab igne with subepidermal bullae on H/E as an extensive negative endocrinologic and rheumatologic work-up, distribution and clinical presentation of plaque, history of heating pad use and overall well-appearing with no evidence of vasculitic disease. Rheumatology follow up on 7/2012 was for polyarthralgia and recurrent bullous livedo reticular rash noted inconclusive workup for bullous disease and autoimmune vasculitis. Again recommended repeat biopsy of active vesicle. Labs included normal CRP, ESR, CCP Ab IgG/IgA 4, and cryoglobulins not detected. EMG for possible carpal tunnel syndrome bilateral wrists was normal. X-rays bilateral hands showed ununited ulnar styloid fractures with mild degenerative changes at triscaphe joint and base of thumb. MCP joints have diffuse mild degenerative changes to first three joints. Degenerative changes to interphalangeal joint. Podiatry on 8/20/2012 diagnosed plantar fibromyalgia for heel and dorsal forefoot pain. X-rays bilateral feet showed no bony abnormalities (7/23/2012). Rash biopsy on 9/12/2012 noted "horizontally oriented loosely arranged collagen fibers spindly or stellate fibroblasts and vertically oriented capillaries indicating scar replacing upper dermis and epidermis with regenerative features. Capillaries tend to cluster in uppermost dermis resembling microscopic angioma-like structures. Melanin incontinences was minimal if any. Consistent with Erythema ab igne. Other labs in 2012 listed one elevated ESR 30 (and CRP 0.7 on 9/12/2012) otherwise normal tryptase, CBC, chemistry, urinalysis, HemA1C, LH, FSH. In 2013 his 25OH V was 13 with testosterone 2.65 normal chemistry, troponin I, CBC, TSH, A-helic (negative), magnesium, LFT, and mild elevation cholesterol (on meds). Current "flares" are 2-3 times a month for 3-5 days. Other symptoms that continue as of 2012 included night time muscle cramps (worse in legs) and arthralgia to all joints which was worse on awakening. His pain would ease about 1 hour after up and moving. His headaches did include nausea with no visual disturbances starting in back of neck and radiating up over top of his head into the forehead region from 2/10 to 10/10. He also complained of tingling vibratory sensation from his head to toes during rash / headache flares. He was medically discharged from duty in 2006 for bilateral knee/ankle pain and PTSD with 70% disability. In 2012, his wife quit work to care for him. He continues to follow up with multiple providers at the Hospital.