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Dermatological manifestations of systemics diseases & vice versa. For medical specialists.

Dermatological manifestations of systemics diseases & vice versa. For medical specialists.

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    Derma Git. Derma Git. Presentation Transcript

    • Gastrointestinal Manifestations of Dermatologic Disorders Prepared by: Dr.Mohammad Shaikhani. Assistant professor Sulaimani University College of Medicine Dept of Medicine. [email_address] For Derma postgraduate students
    • Introduction:
      • Primary dermatologic diseases may involve the GIT or systemic diseases involving the skin, GI tract& liver simultaneously.
      • The correct diagnosis relies on the ability of the gastroenterologist to recognize the underlying dermatologic disorder& include.
      • Examples:
      • IBD/Hepatitis C/Celiac disease
      • Epidermolysis bullosa/Mastocytosis/Hereditary hemorrhagic telangiectasia/Melanoma
    • Epidermolysis Bullosa : case
      • A 16-year-old girl with a H/O epidermolysis bullosa& recurrent mouth ulcers presented with chest pains on swallowing &painful oral lesions.
      • An upper endoscopy was performed
    • Epidermolysis Bullosa :
      • Is an inherited disorder characterized by the formation of blisters after minor skin trauma.
      • Scarring may result.
      • This disorder can affect any epithelialized organ.
      • Skin Findings
      • Tense, fluid-filled blisters, erosions, crusts form in response to minimal trauma.
      • Scarring frequently occurs.
    • GIT features :
      • Poor dentition
      • Esophageal strictures.
      • Malabsorption
      • Severe constipation.
      • Anal fissures.
      • Trauma from food boluses leads to bullae,ulceration, scarring of the esophageal mucosa with formation of strictures, more frequently in the proximal than the distal esophagus
      • Dysphagia is common.
      • Pyloric atresia associated with the junctional form& carries a very poor prognosis.
    • EB :Pathophysiology
      • Mutations of genes encoding proteins located at epidermis/dermis, junction forming a link from basement membrane to the dermis.
      • 3 forms are recognized.
      • 1. Simplex: dominant mutations in genes controlling cytokeratins 5/14/tonofilaments& presents with blisters in the lowermost part of the keratinocytes.
      • 2.The junctional form: recessive mutations in the laminin-5 (type XVII collagen), affecting hemidesmosomes in the dermis or 6/ 4 integrin genes , characterized by blisters in the lamina lucida.
      • 3. Dystrophic form:dominant or recessive mutations in type VII
      • collagen, resulting in blisters beneath the lamina densa&other organs as esophagus, where these same proteins reside.
    • Management :
      • Supportive.
      • Minimizing trauma with a soft diet, attention to wound care, adequate nutrition.
      • Antireflux measures /PPI may be helpful to minimize eso damage.
      • Transplantation of genetically modified epidermal stem cells to improve the adhesion properties of primary keratinocytes currently is investigated.
      • The risk of mucosal trauma from endoscopic procedures should be considered before proceeding.
      • Esophageal strictures dilated with balloons rather than bougies.
    • Mastocytosis: Case
      • A 59-year-old woman with recurrent attacks of abdominal pain, nausea, vomiting& profound dyspnea comes into ER with chest tightness.
      • She had self-administered IM epinephrine without improvement.
      • In the ER, she was tachypneic with diffuse wheezes on exam.
      • She was placed on solumedrol IV.
      • The CXR was negative.
      • H/O mast cell activation syndrome.
      • Her skin showed patchy erythema on the face / arms.
    • Mastocytosis : Case
    • Mastocytosis :derma features
      • Urticaria pigmentosa,diffuse cutaneous mastocytosis, solitary mastocytoma of the skin.
      • The yellow-tan macules involve the extremities, trunk, abdomen, but spare the palms, soles, scalp.
      • Biopsy reveals multifocal aggregates of mast cells
      • Cutaneous mastocytosis, usually is spontaneously regressive when it develops in childhood.
      • Patients with systemic mastocytosis generally are older &usually present with infiltration of multiple organs.
      • Cutaneous disease is present in only about half of all patients.
    • Mastocytosis :GIT features
      • Infiltration of liver causes hepatomegaly, liver dysfunction, ascites
      • Histologic features include aggregates of mast cells both in sinusoids& periportal areas,but fibrosis is uncommon.
      • Intestinal involvement associated with nausea, vomiting, abd pain.
      • Malabsorption, diarrhea,weight loss, hypoalbuminemia are prominent in some patients.
      • Histamine release, increase gastric acid leading to PUD with a risk of bleeding/ perforation.
      • In systemic mastocytosis, common physical findings include splenomegaly, lymphadenopathy&bone pain/myalgia secondary to the infiltration of the musculoskeletal system&BM involvement, result in anemia& pancytopenia.
    • Mastocytosis : Management
      • GI manifestations can be managed with interferon alfa& 2-chlorodeoxyadenosine.
      • The recent identification of kit / PDGFRA mutations have modified the therapeutic algorithm.
      • Although a subset of patients with kit juxtamembrane mutations (eg, K509I) appear to have a imatinib-responsive disease, the vast majority with a mutation at codon 816 may be resistant.
      • Symptomatic treatment of mast cell mediator release usually is managed with H1/H2- blockers & cromolyn sodium.
      • CSs are reserved for recurrent or refractory symptoms.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease): Case
      • A 68-year-old man with HHT was admitted with a decreasing Hb
      • His past history was notable for a partial lung resection of pulmonary (AVMs)& recent mild CHF.
      • He was noted to have telangiectases on the face& chest, a bruit in the right upper quadrant&dark brown stool positive for blood.
      • A CXR revealed large AVMs in the left lower lobe, as did the chest&abdominal CT) scans which also showed hepatic AVMs.
      • The patient underwent an upper endoscopy; bleeding medium-sized AVMs were seen in the duodenum&jejunum.
    •  
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):
      • HHT, a multisystem disease, genetically mediated disorder of fibrovascular tissue.
      • The definitive diagnosis based on the Curaçao criteria, require presence of at least 3 of the following 4 clinical features:
      • (1) Spontaneous, recurrent epistaxis.
      • (2) Telangiectases at characteristic sites as lips, oral cavity, fingers, or nose
      • (3) visceral lesions as cerebral or spinal AVMs, GI tract telangiectasias, pulmonary AVMs, hepatic AVMs
      • (4) A family history of HHT in a first-degree relative.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):Skin
      • Telangiectases that may be punctate, linear, or spider-like on the upper body& in the mouth, including the tongue, nasal mucus membranes&nail beds.
      • They characteristically blanch partly with pressure but do not pulsate.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • HHT should be considered in any patient with bleeding from the GI tract or with anemia& a history of epistaxis.
      • In any patient with bleeding, the gastroenterologist should examine the lips& tongue for telangiectases.
      • Telangiectases involve GITorgans in 11%–25%, with the stomach &small bowel being the source of bleeding more than the colon.
      • The manifestations of bleeding, as IDA, generally begin in the fifth decade.
      • The gastroenterologist also should recognize the variety of clinical manifestations that occur secondary to hepatic AVMs, including high-output CHF, portal hypertension& biliary ischemia.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • The exact clinical manifestation depends on which vascular beds (hepatic artery, hepatic vein, portal vein) the predominant shunting is between (ie,hepatic artery to hepatic vein leads to high-output heart failure).
      • Juvenile polyposis may occur in patients with HHT& vice versa.
      • Patients with early onset GI bleeding should be screened for juvenile polyps given their malignant potential.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • The most common symptom, epistaxis, clinically becomes prominent in puberty.
      • Recurrent epistaxis is managed conservatively with nasal ointment , tamponade, but local treatments including endonasal laser coagulation or argon plasma coagulation & septodermoplasty.
      • Finally, antifibrinolytics as aminocaproic acid/ tranexamic acid have met with some success.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • GI involvement: there is an inverse relationship between the number of telangiectatic lesions in the gut&the hematocrit.
      • Capsule endoscopy has shown a 56% prevalence of telangiectases in the small bowel.
      • Because the bleeding generally is limited to slow oozing, the primary management should be frequent checks of the Hb on a weekly basis to identify patients who need transfusions if oral or intravenous iron is not sufficient to increase Hb to > 10 g/dL
      • Estrogen/progesterone should be tried
      • Argon plasma&embolization.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • Pulmonary / cerebral complications including pulmonary hypertension, paradoxic emboli, air emboli, cerebral abscesses may come on silently secondary to pulmonary AVMs&need to be managed proactively.
      • In patients with pulmonary AVMs, routine antibiotic dental prophylaxis is recommended for both dental & endoscopic procedures.
      • Prophylactic management of the pulmonary AVMs by embolization or surgical resection should be considered when AVMs are : 3 mm.
      • Endothelin-receptor antagonists have been tried.
    • Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease):GIT
      • Patients with hepatic AVMs can present with HF, edema, increased liver function tests, ascites, variceal bleeding, or encephalopathy,managed intensively with medical means.
      • Embolization is used only for palliation given the incidence of hepatic necrosis.
      • Invasive procedures as liver biopsy or ERCP should be avoided given the respective risks for bleeding / sepsis.
      • When medical management fails, referral for a liver transplant used with increasing success.
      • Improvement in nose bleeds/telangiectases with the vascular endothelial growth factor antagonist, bevacizumab, with interferon, suggesti trial in HHT AVMs.
    • Melanoma: case
      • A 92-year-old man presented with a 2-week history of dull, constant periumbilical pain associated with nausea, bilious emesis, , decreased appetite.
      • Physical examination revealed a soft abdomen with epigastric tenderness.
      • Abdominal radiographs revealed changes consistent with a small-bowel obstruction.
      • CT scan showed a small bowel mass with intussusception
      • Five years previously, the patient had had a malignant mole removed from his right forearm
    • Melanoma: case
    • Melanoma:
      • Melanoma is a malignant tumor arising from melanocytes related to both genetic predisposition& sun exposure.
    • Melanoma:skin
      • Superficial spreading melanoma is the most common type.
      • It generally presents as an asymmetric macule with irregular borders, color variation& an enlarging diameter to > 6 mm.
      • It usually is found on the trunk in men&on the legs in women from 30 to 50 years of age.
      • Nodular melanoma is less common & generally a blue- or blackcolored nodule that may ulcerate & develops rapidly over several months, generally on the trunk, head, or neck
    • Melanoma:GIT
      • Skin for suspicious lesions should be an integral part of PE.
      • Melanoma is the 5th most common cancer in men& the 6th most common cancer in women.
      • Referral to a dermatologist is advised for any patient noted to have atypical, or a large number of, nevi.
      • RFs: fair skin with freckles, H/O intermittent sun burns& dysplastic nevi or melanoma.
      • Melanoma is the most common metastatic tumor to the GI, so any patient with H/O melanoma, even in the distant past& symptoms of anemia, IO&/or intussusception, bleeding, or abd pain should be evaluated for metastases.
      • Barium/CT may not detect all tumor deposits reliably.
    • Melanoma:management
      • Surgical excision curative for stages I& II with no nodal or metastatic disease.
      • Stage III: evidence of either microscopic or macroscopic nodal disease , treated with wide excision/ nodal dissection; interferon-alfa 2b as adjuvant therapy.
      • Stage IV with metastases are treated with chemotherapy, radiation,immunotherapy, but the prognosis is very poor, with only 5% surviving 5 years.
      • Melanoma in the GI tract may be primary or secondary.
      • A primary melanoma can arise from any bowel site.
      • More commonly,melanoma is metastatic from a prior skin lesion.
    • Melanoma:management
      • Importantly, metastases may present at the time of the primary diagnosis or decades later.
      • Metastatic deposits to the gut may be identified by using fluorodeoxy-glucose PET, which appears to offer greater diagnostic accuracy than CT/Ba.
      • Although the prognosis is very poor for metastases to the liver / GI tract, surgical resection has resulted in both efficacy in palliating symptoms as obstruction /bleeding & improved survival in select cases.
    • Hepatitis C:
      • Chronic HCV is associated with extrahepatic manifestations, including dermatologic conditions as mixed cryoglobulinemia, porphyria cutanea tarda& lichen planus.
      • The efficacy of IFN on skin features are highly variable.
      • dermatologic side effects of IFN therapy are common, with or without HCV infection& may complicate assessment of HCV-associated dermatologic disorders.
      • Pruritic,eczematous lesions are most common& often resolve on completion of therapy without dosage alteration.
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    • IBD: Dermatological features
      • Skin - erythema nodosum 2-4%.
      • Pyoderma gangrenosum 1-2%
      • Mouth - aphthous ulcers 10%.
      • A wide variety of cutaneous conditions are associated with IBD.
      • In general, their course parallels the GI status,but can precede or follow the diagnosis of the IBD.
      • Dermatologists can help clinicians to reach the correct diagnosis & the best treatment approach, but treatment of the underlying IBD is essential for the control of the cutaneous associated conditions.
    • IBD: Dermatological features
      • Seen in 20%, slightly higher in CD than in UC& include:
      • Pyoderma gangrenosum(PG).
      • Bowel-associated dermatosis-arthritis syndrome.
      • Cutaneous Crohn’s disease.
      • Erythema nodosum
      • Avariety of vasculitis.
    • IBD dermatological features: PG
      • The most severe dermatologic manifestation.
      • Are ulcerating necrotic plaques on their lower extremities.
      • 60% with PG have an associated disease, with Crohn’s being the most frequent but other diseases including RA,AML, MM& HIV.
      • Certain drugs, like G-CSF& interferon
      • 2-10% with IBD will eventually develop PG.
      • Often parallels the severity of their disease,but may precede or follow episodes of IBD& may initially have a flare associated with IBD, eventually become chronic& protracted.
    • IBD dermatological features: PG
      • Often have atypical presentations with pustular &necrotic flares.
      • PG tends to start with a single or multiple small pustular lesions that eventually evolve into large ulcerated lesions.
      • Another feature is pathergy: i.e. , new lesions triggered by trauma.
      • A pustular lesion triggered by trauma may not evolve into an ulcer &may be the only cutaneous sign of IBD.
      • The peristomal variant,develop adjacent to the colostomy.
    • IBD dermatological features: PG
      • DD:
      • Infections: deep fungal infs, bacterial pyodermus, TB.
      • Vasculitis, Wegener’s granulomatosis, hallogenodermas, secondary to high ingestion of iodine or bromides present with suppurative ulcerated lesions resembling PG.
    • IBD dermatological features: PG
      • Treatment of the associated disease is the critical point.
      • In IBD, infliximab is the treatment of choice
      • For patients with other associated conditions, the standard of care is systemic steroids, either oral prednisone or methyl-prednisolone, with cyclosporine or mycofenolate.
      • Other treatments include thalidomide andmycophenolate mofetil.
      • Other treatment: plasmapheresis, IV Ig,absorption apheresis.
      • Surgery as debridement and allografts avoided utill the lesions are quiescent because of pathergy.
      • Localized: treated topically with potent steroids or tacrolimus or intralesional triamcinolone.
    •  
    • IBD dermatological features: Bowel-Associated Dermatosis-Arthritis Syndrome (bowel bypass syndrome)
      • Also be associated with IBD secondary to an overgrowth of bacteria: Streptococci , Dientamoeba fragilis , or E coli .
      • Present with flu-like symptoms.
      • Bacteria proteoglycan activate complement causing pap/pustules, severe tenosynovitis &pathergy of cutaneous lesions.
      • Microscopy shows multiple neutrophils & a low level of vasculitis.
      • Treatment is for the underlying IBD, with resection, if indicated.
      • Systemic steroids must be avoided, as may worsen the problem.
      • Probiotics as used for pouchitis& antibiotics: minocycline, erythromycin, or metronidazole, may be useful.
    • IBD dermatological features: Cutaneous CD.
      • A granulomatous, noncaseating skin lesion separated from the GI tract by normal skin.
      • It is very rare: 20% have a negative GI history& only subsequently develop IBD.
      • The lesions may be adjacent to the GI tract at both ends; perioral or perianal, or more distant& noncontiguous metastatic lesions.
      • PPD &CXR done to differentiate from TB& sarcoidosis.
      • Trt: Metronidazole of choice& If only a few lesions, topical or intralesional steroids may be useful.
      • Ssulfasalazine/azathioprine being effective.
      • Surgical excision is often complicated by wound dehiscence.
    • IBD dermatological features: EN
      • The most common complication of IBD, other than aphthous dermatitis.
      • Presents with multiple,painful, deep nodules on extremities
      • The nodules,unlike PG do not become fluctuant or ulcerated.
      • In some,may be large& highly inflamed, while in others minimal skin changes with only deep pain.
      • Occurs in 3–10% UC& 4–15% CD
      • The nodules tend to appear during the acute phase of IBD.
      • Unlike PG—a chronic, ongoing process—it is short-lived process.
      • DD: Srept inf, TB, Sarcoid.
    • IBD dermatological features: EN
      • The treatment should be aimed at the triggering event.
      • NSAIDs like indomethacin, are often prescribed for 2 or 3 wk, along with bed rest, leg elevation, prednisone taper, intralesional or intramuscular triamcinolone in patients with severe flares.
      • Second-line therapy, rarely needed as colchicine, hydroxchloroquine, or dapsone.
    • Celiac dis dermatological features:
      • Dermatitis herpetiformis is well known accompanying CD
      • DH: Itchy, blistering skin, rash usually on elbows, knees, buttocks, back, diagnosed with skin biopsy
      • Cutaneous,mucosal, nail, hair findings were detected in 74.5%, 27.3%, 20.0%,7.3% of patients, respectively.
      • The most prevalent dermatologic diagnosis was xerosis (69.1%).
      • No significant relationship was detected between the cutaneous findings& the duration of illness.
      • However, the duration was longer in patients with mucosal findings compared to those without mucosal findings.
      • It was found that all patients without cutaneous findings were on a strict gluten-free diet.
    •  
    • Celiac dis dermatological features:
      • Pathophysiology: an abnormal small intestinal permeability allow the crossing of endogenous or exogenous antigens may provoke the immunological response, common immune mechanisms, vascular alterations & vitamin/aminoacid deficiency secondary to malabsorption.