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Vitiligo in association with Erythema dyschromicum perstans

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Twenty seven years old female patient two years ago after delivery has noticed appearance of irregular hypo- and achromic macules on her trunk, extremities and face. Two months ago she has seen on her …

Twenty seven years old female patient two years ago after delivery has noticed appearance of irregular hypo- and achromic macules on her trunk, extremities and face. Two months ago she has seen on her trunk and extremities oval gray-blue hyperpigmented macules which are accompanied from a slight pruritus.
She has common complains of weight reduction of 5-6 kg, palpitation, sleep disturbance, fatigue and some joint pain.
Clinically our patient is IV phototype. She has two different type of exanthema. First type - vitiligo is presented from symmetrical distributed over the trunk, extremities and face hypopigmented and achromic macules from 0,5 cm to 20 cm in diameter. The second type exanthema has symmetrical distribution and involves abdomen, back and proximal part of extremities. The lesions are gray-blue macules with oval shape and size from 0,5 cm to 2 cm in diameter. There is no change in mucous membrane.
Deviations of the investigations include slight elevated ECR, reduced HGB, HCT, MCV, MCH, MCHC, monocytosis, reticulocytosis, low serum Fe, increase TIBC, decrease LDH, positive serological test for H. pylori, increased Tg-Ab and TSH-RAb, very low TSH, elevated FT4, nasal smear – S. aureus, vaginal smear – S. agalactiae. Ultrasound of thyroid gland shows normal topic, size, structure and enhanced blood flow.
Conducted by the clinical laboratory research fund and consultative examinations are specified comorbidities Grave’s disease, iron deficiency anemia, bacterial colpitis, and chronic gastritis.
Histopathological examination of the edge or the hyperchrome lesion show minor hydropic degeneration of basal layer, sparce, superficial, perivascular lymphocyte infiltrat, and macrophages containing melanin (incontinentia pigmenti).
Differentially were discussed lichen planus, postinflammatory hyperpigmentation, contact dermatitis, fixed drug reaction.
Based on the anamnesis, clinical picture, laboratory results and conducted histological examination answer the question what is this second type exanthema is Erythema dyschromicum perstans.
Conducted treatment for accompanying diseases is with Ciprofloxacin, Ferrous sulfate, Vitamins, Thiamazol, eradication therapy for H. pylori and local application of Mupirocin nasal ointment. We have made 7 procedures UVB 311 nm narrow band with slight improvement.
There are only few previously described cases of Erythema dyschromicum perstans & vitiligo in the same patient. These cases include patients with darker skin. In both diseases there is HLA-DR4 association in the pathogenesis. There are some common features between two diseases which include predominance of cytotoxic T-cell and almost the same ratio of CD4/CD8, Ia antigen positivity in the dendritic cells in epidermis and dermis and increased number of epidermal Langerhans cells.
- Disclaimer- This PPT is loaded as student material "as is", from the VRF Vitiligo Master Class Barcelona November 2011; VRF does not endorse or otherwise approve it.

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  • 1. Vitiligo in association with Erythema dyschromicum perstans Violina Todeva 1 E. Hristakieva, D. Karshakova, D. Gancheva 1 S. Yordanova 2 1 University Clinic Stara Zagora, Bulgaria Department of Dermatology and Venereology 2 Medical Institute - Ministry of Interior, Bulgaria Department of Endocrine Diseases
  • 2. Anamnesis
    • Duration: 2 years
    • Trigger: childbird
    • Presentation: irregular hypo- and achromic macules
    • Localisation: trunk, extremities, face
    • Subjective complaints: (-)
    • Therapy: supplements
    • P . M . R . , ♀ 2 7 years
  • 3. Clinical Findings Mucous membranes and skin appendages - unchanged IV phototype
    • Symmetrical distribution
    • Trunk, extremities, face
    • Diameter – 0,5 – 20 cm
    • Irregular shape
    • Sharply circumscribed
    • Hypopigmented macules
    • Achromic macules
  • 4. Clinical Findings Mucous membranes and skin appendages - unchanged
    • Symmetrical distribution
    • Trunk, extremities
    • Diameter – 0,5 – 2 cm
    • Oval shape, gray-blue macules
  • 5. Investigations
    • Hematological tests
      • ESR - 20/ 40 mm ; HGB - 110g/l; HCT - 0,34 L/L; MCV - 80 fL; MCH - 25,2 pg; MCHC - 315 g/L; Mo - 11%; Ret - 9 ‰ ;
    • Biochemical tests
      • Serum Fe - 6,1 µmol/L ; TIBC - 65,5 µmol/L , LDH - 105 U/L
    • Serological tests
      • H. pylori - 8,0 U/ml
    • Immunological tests
      • Tg-Ab - 219 IU/ml, TSH-RAb - 7 IU/mL
    • Hormonal tests
      • TSH - 0,038 mIU/L, FT4 - 26,07 ng/L
    • Microbiological tests
      • Nasal smear - S. aureus; vaginal smear - S. agalacticae
    • Parasitological tests
      • Negative
    • Urine
      • Referent
    Laboratory tests
  • 6. Investigations
    • Ultrasound of thyroid gland
      • normal topic, size and structure
      • enhanced blood flow
    Instrumental tests:
    • Endocrinologist
    • Haematologist
    • Gastroenterologist
    • Rheumatologist
    Consultations:
  • 7. Comorbidities Grave’s disease Iron deficiency anemia Bacterial colpitis Chronic gastritis
  • 8. Hystopathology
    • Epidermal changes
      • increased epidermal pigment
      • hydropic degeneration of basal layer
    • Dermal changes
      • incontinentia pigmenti (Macrophages containing melanin)
      • sparce, superficial, perivascular lymphocyte infiltrat
    H&E x 20 H&E x 40
  • 9. Erythema dyschromicum perstans
    • Contact dermatitis
    • Fixed drug reaction
    • Addison disease
    • Hemochromatosis
    • Secondary syphilis
    • Urticaria pigmentosa
    • Macular amyloidosis
    • Late pinta
    • Leprosy
    • Lichen planus – pigmentosus et actinicus
    • Postinflammatory hyperpigmentation
  • 10. Therapy
    • Emollients
    • Mupirocin nasal ointment
    Systemic Local
      • Ciprofloxacin 2x500 mg
      • Ferrous sulfate 1x325 mg
      • Vitamins (Vit.B6, Vit.B9, Vit.C)
      • Thiamazol 2x10 mg
      • H. pylori eradication
    • UVB 311 nm narrow band
    Physical
  • 11. Vitiligo
    • Incidence
      • 0,1-3%
    • Peak
      • 2 nd and 3 rd decade
    • Children
      • 25% of all patients
    • Gender
      • ♂ : ♀ up to 1:1,3
    • Ethnic, racial incidence
      • equal
    • Socioeconomic factors
      • irrelevant
    • Family history
      • 20% - 30%
    • Monozycotic twins
      • 23% concordance
    Epidemiology Dermatology for skin of color, A. Kelly, S. Taylor, 2009, p. 317-318, p.99
  • 12. Etiology & Pathogenesis of Vitiligo
    • Autoimmune hypothesis, humoral and cell-mediated immune aberrations
      • Common linkages with other autoimmune diseases
      • Increased frequency of organ specific Ab
      • Melanocyte Ab, tyrosinase and tyrosinase-related proteins 1 and 2 Ab, MCHR1 Ab
      • Activation of cytotoxic T-lymphocyte, decrease in helper T-lymphocyte
      • Elevated levels of IL-2 in blood and skin, lesional skin expression TNF- α , IL-6, IF- γ
    • Genetic predisposition
      • Multifactor, polygenetic disorder
      • HLA-DR4 , -Dw7, -DR7, -DR1, -B13, -Cw6, -DR53, -A19
      • Chromosomes – 1, 2, 7, 8, 11, 17, 19, 22
    • Viral infection
      • CMV
      • HSV
    • Neural theory
      • Segmental vitiligo
      • Peripheral nerve ending released compounds that may inhibit melanogenesis – neuropeptide Y
      • Lesional autonomic dysfunction – increased sweating
    • Oxidative stress
      • Blood – low catalase and glutathione, elevated superoxide dismutase, xanthine oxidase
      • Skin – defective recycling of tetrahydrobiopterin, increased hydrogen peroxide, decreased catalase
    • Self-destruction/ Autocytotoxic
      • Formation of phenolic compounds during synthesis of melanin
      • Environmental compounds – catechols, phenols, sulfhydryls
    Dermatology for skin of color, A. Kelly, S. Taylor, 2009, p. 318-319
  • 13. Clinical manifestation of Vitiligo
    • Localized
      • Focal: one or more patches in the same area;
      • Segmental: limited to a dermatome or Blashko lines;
      • Mucosal: only mucous membranes.
    • Generalized
      • Vulgaris – disseminated lesions without region predilection;
      • Acrofacialis – distal extremities and facial (periorificial)
        • Lip-tip variety
    • Universalis
      • Complete or almost complete depigmentation
    • Clinical variants
      • Trichrome , Quadrichrome, Pentachrome
      • Confeti type
    Thieme Clinical Companions, Dermatology, W. Sterry et all, p.375 Fitzpatrick , s Dermatology in general medicine Klauss Wolf , 2008, 617-618
  • 14. Vitiligo & associated disorders Rook’s Textbook of Dermatology, 2010, p.58.46 Fitzpatrick’s Dermatology in general medicine Klauss Wolf , 2008, 619
      • Thyroid – Graves disease , Hashimoto thyroiditis
      • Addison disease
      • Diabetes mellitus
      • Hypoparathyroidism
      • Pernicious anemia
      • Alopecia areata
      • Myasthenia gravis
      • Autoimmune polyendocrine
      • syndromes
    AUTOIMMUNE DISEASES Vogt-Koyanagi-Harada syndrome Ocular disorders Alezzandrini syndrome Morphoea lichen sclerosus Aseptic meningitis Premature graying Poliosis (Leucotrichia) Malignant melanoma Halo naevus VITILIGO
  • 15. Classification of the APS
    • APS-1
      • Chronic candidiasis , C hronic hypoparathyroidism , Addison’s disease (at least two present)
    • APS- 2
      • Addison’s disease (always present) + autoimmune thyroid diseases and/or type 1 diabetes mellitus
    • APS- 3
      • Autoimmune thyroid diseases associated with other a utoimmune diseases (excluding Addison’s disease and/or hypoparathyroidism)
    • APS- 4
      • Combinations not included in the previous groups
    Betterle C.; Zanchetta R., Update on autoimmune polyendocrine syndromes (APS). Acta Biomed Ateneo Parmense 2003;74:9-33 Autoimmune polyendocrine syndromes
  • 16. Autoimmune polyendocrine syndromes type III Betterle C.; Zanchetta R., Update on autoimmune polyendocrine syndromes (APS). Acta Biomed Ateneo Parmense 2003;74:9-33 AUTOIMMUNE POLYENDOCRINE SYNDROMES TYPE 3 AUTOIMMUNE THYROID DISEASES Hashimoto’s thyroiditis Idiopathic Myxoedema Asymptomatic thyroiditis Endocrine exophthalmus Grave’s disease Endocrine Diseases 3 A Gastrointestinal Apparatus 3 B Skin / Hemopoietic system/ Nervous system 3 C Collagen Diseases/ Vasculitis 3 D Type 1 DM Hirata’s syndrome Premature ovarian failure Lymphocitic hypophysitis Neurohypophysitis Atrophic gastritis Pernicious anemia Coeliac disease Chronic inflamm. bowel diseases Autoimmune hepatitis Primary biliary cirrhosis Sclerosing cholangitis Vitiligo Alopecia Autoimmune thrombocytopenia Autoimmune hemol. anemia Anti-phospholipid syndrome Miastenia gravis Stiff-mann syndrome Multiple sclerosis LES LED Mixed connectivitis Rheumatoid arthritis Reactive arthritis Sclerodermia S ö gren`s syndrome Vasculitis + + + +
  • 17. Erythema dyschromicum perstans, 1961
      • Oswaldo Ramiretz, San Salvador, 1957
      • Los cenicientos, Ashy dermatosis
    • Epidemiology
      • predominantly III-VI Fitzpatrick skin type
      • slightly higher incidence in women
      • peak 2 nd and 3 rd decades
    • Etiology :
      • intestinal whipworm infection
      • endocrine disorders (thyroid disease)
      • radiographic contrast media
      • ammonium nitrate ingestion
      • occupational cobalt allergy
      • chronic hepatitis C
      • HIV infection
      • exposure to chlorothalonil – fungicide in banana plantation
    Marion Sulzberg 1895–1983 Dermatology for skin of color, A. Kelly, S. Taylor, 2009, p. 167
  • 18.
    • Genetic susceptibility
    • Aberrant immune response targeting basal cell layer antigens
    • Abnormal cell mediated immunity
    • Increased expression of intercellular adhesion molecule 1 and MHC class II ( HLA-DR4 ) within the basal cell layer
    • Ia antigen expression of keratinocytes
    • OKT5 and OKT6 staining of Langerhans’ cells
    • Presence of thrombospondin receptor CD36
    • Cellular dermal infiltrate express CD69 and cytotoxic cell marker CD94
    Erythema dyschromicum perstans Pathogenesis Discussion
    • Lichen planus pigmentosus
    • Lichen planus actinicus
    • Postinflammatory hyperpigmentation
    Dermatology for skin of color, A. Kelly, S. Taylor, 2009, p. 167 Erythema dyschromicum perstans, R. Schwarz, MD; Chief Editor: Dirk M Elston, MD EDP spares oral, genital mucosa, scalp, nails, palms, soles
  • 19. Discussion
    • Few cases of Erythema dyschromicum perstans & vitiligo in the same patient
    • Darker skin
    • HLA-DR4 association
    • A Gross et all, Source Institute of Biomedicine, Caracas, Venezuela
      • Predominance of cytotoxic T-cell
      • Ia antigen positivity
      • Increased Langerhans cells in epidermis
    Erythema dyschromicum perstans and vitiligo, Narayan S Naik MD, Dermatology Online Journal 9(4): 25 From the Ronald O. Perelman Department of Dermatology, New York University Mononuclear cell subpopulations and infiltrating lymphocytes in erythema dyschromicum perstans and vitiligo. Gross A, Tapia FJ, Mosca W, Perez RM, Briceño L, Henriquez JJ, Convit J.SourceInstitut of Biomedicine, Caracas, Venezuela.
  • 20. Conclusion Alter immune regulation TRICHROME VITILIGO VULGARIS & GRAVE’S DISEASE & ERYTHEMA DYSCHROMICUM PERSTANS Immune cell participation Autoimmune pathogenesis
  • 21. Thank you for your attention !

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