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Dermatology department

Dermatology department. CASE ( 1 ). By. Yomna Mazid El-Hamd Assist. Lecturer. A -42- y old male Multiple skin lesions scattered allover the body One month duration. One year ago Generalized Lymphadenopathy & hepatosplenomegaly Castleman’s disease LN biopsy. 3 months ago

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Dermatology department

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  1. Dermatology department

  2. CASE ( 1 ) By Yomna Mazid El-Hamd Assist. Lecturer

  3. A -42- y old male Multiple skin lesions scattered allover the body One month duration

  4. One year ago Generalized Lymphadenopathy & hepatosplenomegaly Castleman’s disease LN biopsy

  5. 3 months ago Admission in internal medicine department Persistant unexplained fever & progressive weight loss

  6. Trunk Back Multiple scattered firm tenderless violaceous papules.

  7. Upper extremities Neck

  8. Lower extremeties

  9. Multiple scattered firm tenderless violaceous papules.

  10. Ulcer at the under surface of the tongue Oral hairy leukoplakia

  11. Solitary Tender Superficial Well defined Irregular edge Red clean floor Soft base +ve LN

  12. General examination Generalized lymphadenopathy. Hepatosplenomegaly.

  13. Differential Diagnosis Kaposi’s sarcoma. Bacillary angiomatosis. Angiosarcoma.

  14. Investigations CBC: normocytic normochromic anemia, thrombo-cytopenia, normal total leukocytic count with relative lymphopenia. U/S: periportal fibrosis, portal hypertension and hepatosplenomegaly. LN biopsy: Castleman’s disease. ELISA forHIV: +ve.

  15. Investigations

  16. Investigations

  17. Investigations Skin biopsy:

  18. Multiple new blood vessel formation. The capillaries are dilated & increased in number, their endothelial cell lining are large & protrude into the lumen.

  19. Pre-existing blood vessels protruded into the newly formed BL vessels (promontory sign)

  20. Many spindle cell formations containing vascular slits.

  21. Kaposi’s sarcoma

  22. Generalized Lymphadenopathy. • LN biopsy. Multicentric Castleman’s disease

  23. HIV positive

  24. Kaposi's sarcoma & Castleman's disease in HIV +ve patient

  25. The patient was referred to: EL ABASSIA hospital.

  26. Castleman Disease • CD disease is a benign disorder first described by Dr. Benjamin Castleman in 1956. • CD is also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia.

  27. Types of Castleman Disease There are several variants of CD: Unicentric CD involves single LN group. It usually has few or no symptoms. Multicentric CD shows widespread lymphadenopathy & even hepatosplenomegaly. “B” symptoms including severe fatigue, night sweats, fever, wt-loss, anorexiaare usually present.

  28. CD frequently coexist with KS , an AIDS defining condition. • CD & KS are rare conditions, but in recent years their incidence have been increased due to their association with HIV infection.

  29. Human herpesvirus 8 ( Kaposi's sarcoma-associated herpesvirus) infection is present in nearly: • 100% of MCD cases associated with HIV-1 infection. • 50% of cases that are HIV-1 negative.

  30. In all cases, CD is likely due to hypersecretion of the cytokine IL6. In tumors that are positive for KSHV, this is most likely due to expression of the a virus-encoded cytokine vIL6. KSHV negative tumors appear to be the result of over-secretion of human IL6.

  31. Thanks

  32. CASE ( 2 ) By Yomna Mazid El-Hamd Assist. Lecturer

  33. A -24- y old female. Multiple skin lesions in the left axilla, arm & forarm. Neither itchy nor painful. One year duration. .

  34. Multiple hyperpigmented nodules. • Areas of skin atrophy. • in left axilla, arm & forarm with linear configuration.

  35. The lesions extened to the lateral aspect of the trunk

  36. Differential Diagnosis • Pilomatricoma. • Organoid Nevus.

  37. Investigations • Routine lab: NAD • Skin biopsy:

  38. Punch skin biopsy was taken from lesion in left axilla.

  39. Large number of apocrine glands extending from upper dermis into SC fat

  40. Hair follicles are seen beside apocrine glands

  41. Apocrine Hamartoma

  42. Apocrine hamartomas • Apocrine hamartomas are extremely rare benign tumors of the skin composed of mature apocrine glands in the papillary and reticular dermis. • The overlying epidermis is often hyperplastic.

  43. Apocrine hamartomas • Congenital apocrine hamartoma is an unusual clinical variant of organoid nevus with pure apocrine differentiation. • Several cases have been reported in the literature but few were of uniform clinical appearance.

  44. Differential Diagnosis • Eccrine nevus: There is increase innumber & size of normalappearing eccrine coils. • Eccrine Angiomatous Hammartoma: There is increase in number of eccrine glands together with small BVs, Ns, mucin or fat. • Porokeratotic eccrine ostial nevus: There are cornoid lamellae associated with eccrine ducts.

  45. Treatment • Laser. • Surgical resection.

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