Here's the whole story (I'm the "friend" and it's about my dad) I have pictures -- I'll try to post them.
75 y/o WM ? Family Physican from Kansas. Visited Minot, Haiti (near Cape Haitian), on a medical mission trip in 2010. Received routine pre-trip vaccines such as Tetanus booster and polio, hepatitis boosters, etc. Mainly saw pediatric patients in Haiti. No acute diarrheal illness related to the Haiti trip.
About 1 month after return from Haiti, developed a generialized, pruritic, erythematous, raised rash ?all over? with sparing of the palms, soles, & "bikini? line. Started as just a few small itchy bumps on trunk and legs. Now generalized (see Picts attached). Bowel changes since that trip changed from solid daily (pre-trip) to soft ?Cow patties? daily with an increased gastrocolic reflex. This rash is so severe he is wearing gloves and full body PJ?s to sleep at night, staining sheets with blood (bad enough that he has been requested to pay for sheets at hotels recently due to the amount of bleeding at night.) No other systemic s/sx of illness.
Other travel: Viet Nam 1965-6. Nicaragua 1977. Kenya ~ 10 years ago. Carribbean & Western Europe repeatedly. Has never had anything like this before.
PMHx: + PPD since internship 1960?s. Calcified granuloma RLL on CXR (stable). HTN. Basal Cell CA on the nose, resolved with serial Cryo. Colon Polyp @ age 71. Hay fever.
Social Hx: + Tobacco. + EtOH. No drugs. Married, wife has NO evidence of rash after 3 years of contact.
Surg Hx: Tonsils at age 10. Vasectomy. Rotator Cuff 2012 (Tore carrying luggage to a Derm CME.)
Meds: Bystolic, Vytorin, Tumeric, K-Dur, Ca/Vit D; HCTZ/Lisinopril; Amlodipine/olmesartan. Cialis prn. (Altering these meds had had no impact on the
rash.)
Allergies: ASA, Bee stings. ? Sulfa cream made rash worse.
Family Hx: F = ALS. M= CVA. 8 healthy kids.
LABS: (Most here are for the rash ? but I?ve included recent health maint stuff too just in case someone sparks a thought).
Incidental CT Chest & Abdomen both negative.
Leptospira Ab = Negative
Filiaria smear negative
Strongyloides serology negative.
Stool cultures negative. Stool O&P Negative.
Toxocara serology negative.
Toxoplasma negative IgG & IgM.
Trypanosoma cruzi Ab, IgG negative.
Trichinosis negative serum Ab.
Giardia stool negative & Antigen Negative.
Leishmaniasis (Visceral) Ab, S = negative.
Entamoeba histolytica negative.
Babesia negative.
HIV / RPR negative.
Hgb 14.6. WBC 9. Plts 162.
Eo?s 0.68 x 10(9)/L (reference 0.05-0.5).
Monocytes 17% (reference 1-11).
IgE elevated at 1,332 kU/L.
Sputum stain negative for malignancy.
LIPIDS: Chol 191, LDL 122, HDL 58, TG 79, Non-HDL-C 133.
Myeloperoxidase 450. Lp-PLA2 = 174. Hs-CRP 12.6. Fibrinogen 529.
NT-proBNP 293. Galectin-3 = 18.
Platelet Genetics normal.
Factor V-Leiden negative.
PT Gene Normal.
MTHFR C/T single mutation.
Insulin 8. Free Fatty Acids 0.71. Glucose 92. HbA1c = 4.8.
25OHD 59. Uric Acid 7.2. TSH 3.32.
Homocysteine 17. B12 = 640. Folate 620. Cystatin 1.23.
Celiac panel NEGATIVE.
Scabies scrapings negative (and treatment x 3 failed.)
Hepatitis A,B,C: all negative.
"Childhood allergy profile" LOW Positive for Soybean, Alternaria, Wheat, Peanut, Cockroach, and Walnut.
Punch Biopsies: Sorry, I don't have copies of the photomicrographs. Gross only. "Subacute dermatitis with eosinophils. "
2010: Spongiotic and psoriasiform dermatitis (granular cell layer intact, underlying superficial perivascular as well as interstitial lymphohistiocytic infiltrate seen. No eosinophilic or neutrophilic component seen. Iron stain negative for hemosiderin.
2011: Acantholytic dermatitis. DIF IgA, Ig, IgG, and C3 all negative.
2012: Lichen simples chronicus with superficial perivascular/interstitial chronic dermatitis, superficial dermal fibrosis, and focal scale crust.
2012: LSC with central features of prurigo nodularis, superficial perivascular/interstitial chronic dermatitis & superficial dermal fibrosis.
2012: Direct Immunofluorescence tests negative.
2012: Acantholytic dyskeratosis, Focal spongiosis.
MAYO (Rochester) Impression: Generalized Lichenified
Dermatitis with secondary excoriation, and Eosinophilia. Saw Derm, Travel Medicine, & Infectious Disease. Repeat biopsy with Mayo read as
?Chronic Dermatitis.? Outside slide review follows:
Pseudoepithelious hyperplasia, eosinophilic spongiosis, dermal fibrosis, mixed dermal inflammation with eosinophils. PAS-D, GMS and AFB Negative for fungal microorganisms. Other sides show ?focal acatholytic
dyskeratosis and mixed dermal inflammation with eosinophils.
Surface Cell IgG antibodies by CIFS on monkey esophagus substrate ?supports a diagnosis of pemphigus?? Titer 1:40 for Monkey Esophagus IgG.
FAILED: Bactrim x 2w. Doxycycline 100 PO BID x 30 days. Claritin & Zyrtec prn. Hydrocortisone cream. Clobetasol cream. Topical sulfa. Lindane head to toe x 3. (9/2010 when the rash was just a baby.) Phototherapy. Soriatane (acitretin) 10mg daily x 30 d. Cipro PO. Bleach baths. Looks like he's failing a recent course of mebendazole 100 PO BID x 3 days.
Exacerbated by Clobetasol & Saran Wrap.
Hesitant to use oral steroids due to risk of tuberculosis history.
Expert Opinions: 2 private Derm?s. V.A. hospitals x 2. Large Midwest university M.D. teaching hospital Derm. Mayo Clinic in Rochester. Multiple Derm conference where he has been going (for CME, of course!) and willingly taking off his clothes for anybody that wants to give an opinion ? most recently Harvard. He is actively trying to get help, but nobody
can figure this out! Most recently had an Ob/Gyn consult. ;-)
Currently using 1.25% Hydrocortisone in VaniCream and RoBathol bath oil (cottonseed oil) wraps BID-TID. Minimal improvement, no resolution with over a month of therapy.
Working Diagnosis has been GROVER?s DISEASE. BUT, it?s getting worse over the past 3 years without remission nor relenting.
Please chime in with your Internist, Infectious Disease,
Travel Med, Allergy & Immunology, or Dermatologic advice and wisdom!
