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Inflammatory Myopathies
Disease                  Poly Myositis             Dermatomyositis           Inclusion Body Myositis

Tissue involved          Inf. Of Skeletal Muscle   Inf. Of Skeletal Muscle   Inf. Of Skeletal Muscle
                         Fibre                     Fibre & Skin              Fibre
Age                      Adults                    Children & Adults         Adults>50 years

Clinical Presentations   Bilateral Proximal        Bilateral Proximal        Asymmetrical Distal
                         Muscle Weakness           Muscle Weakness.          Muscle Weakness

                                                   Skin Rash of Upper
                                                   Eyelid

                                                   Periorbital edema
Microscopic Features     Endomysial lymphocytic    Perimysial and vascular   Cytoplasmic Vacuoles
                         Infiltration( CD 8)       lymocytic infiltration    and basophilic granules
                                                                             and amyloid.
                         Skeletal muscle fibre     Perifasciular Fibre
                         regeneration and          Atrophy                   EM: Filamentous
                         degeneration                                        Inclusions
                                                   Skeletal muscle fibre
                                                   regeneration and
                                                   degeneration
Cancer Risk:                                       Increased Risk Of :
                                                   Lung , Stomach &
                                                   Ovarian Cancers.
Myasthenic Syndromes.
         Disease           Myasthenia Gravis            Eaton-Lambert           Gullian- Barre
                                                          Syndrome                Syndrome
Mechanism               Autoantibodies against    Autoantibodies against    Antibodies against
                        Ach. Receptors in NMJ     Ca. Ion Channels in NMJ   Schwann cells
                                                                            leading to
                                                                            Peripheral Nerve
                                                                            Demyelination &
                                                                            inflammation
Associated Diseases     Thymoma or Thymic         Small Cell Lung Cancer    Preceded by Viral
                        hyperplasia                                         illness

Gender                  Females > Males

Clinical Presentation   Muscular weakness of      Proximal Muscle           Muscle weakness
                        Body but predominantly    Weakness                  with an ascending
                        affecting:                                          paralysis.
                        Facial Muscles
                        Extraoccular Muscles with                           Loss of Deep
                        Ptosis                                              tendon reflexes


                        May Involve Respiratory                             May Involve
                        Muscles and cause Death                             Respiratory
                                                                            Muscles & cause
                                                                            death
Alleviating Factors     Weakness worsens with     Weakness improves with
                        repeated contractions.    repeated contractions

Diagnosis                                                                   Nerve Conduction
                                                                            Studies.
Muscular Dystrophy
             Disease       Duchene Muscular dystrophy             Becker Muscular Dystrophy

Incidence                 Most Common & Severe Form of          Less Common & Mild Form of
                          Muscular dystrophy                    Muscular dystrophy

Genetics                  X-linked Recessive                    X-linked Recessive

Mutations                 Mutations lead to Absence of          Mutations lead to Alteration of
                          Dystropin protein                     Dystropin protein
Clinical Presentations    Rapidly Progressive Muscular          Slowly Progressive Muscular
                          Weakness                              weakness.

                          Proximal weakness of Shoulder         Muscle weakness of Pelvis and
                          and pelvic Girdle                     Legs

                          Calf Pseudohypertrophy

                          Heart Failure and arrhythmia          Heart involvement is rare.

                          Respiratory insufficiency and
                          infections

                          Age 5- Onset of symptoms              Later onset with variable
                                                                progression and may lead a
                          Age 10- Wheel chaired                 normal life span

                          Age 20- Respiratory Failure and
                          death

Microscopic Features.     Fibrosis and Fatty infiltration and
                          Necrosis

                          Skeletal muscle degeneration          Skeletal muscle degeneration
                          and regeneration                      and regeneration
Diagnosis                 Muscle Biopsy                         Muscle Biopsy
                          DNA Analysis                          DNA Analysis
Benign Soft Tissue tumors
     Tumors                      Type of cells                     Locations

     Lipoma                     Adipose tissue                       Trunk
 ( Most Common)                                                      Neck
                                                              Proximal Extremities
 Dermatofibroma              Dermal Spindle cell                  Extremities
                        (Forms a red nodule that can be
                                   squeezed)
   Fibromatosis                  Fibrous tissue             Infiltrates Muscle tissue

  Rhabdomyoma                Striated Muscle cells                   Heart
                                                                    Tongue
                                                                    Vagina
   Leiomyoma                 Smooth muscle cells                    Uterus
                                                                   Stomach




                  Malignant Soft Tissue Cancers
     Cancers                     Type of cells                     Locations

   Liposarcoma                  Adipose tissue                      Thigh
 ( Most Common)                  & lipoblasts                  Retroperitoneum

  Fibrosarcoma                                                      Thigh
                                                                 Upper Limb
Malignant Fibrous             Pleomorphic cells                     Thigh
  Histocytoma                                                  Retroperitoneum

    Embryonal                Striated muscle cells         Male โ€“ Mass seen protruding
Rhabdomyosarcoma                                                    form penis
                                                          Female- Mass seen protruding
                                                                   from Vagina
 Leiomyosarcoma              Smooth Muscle cells                      Uterus
                                                                        GIT
Neurofibrosarcoma                 Nerve cells               Peripheral Nervous system

 Synovial Sarcoma          Glands and spindle cells              Around Joints

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Inflammatory Myopathies and Muscular Dystrophies

  • 1.
  • 2. Inflammatory Myopathies Disease Poly Myositis Dermatomyositis Inclusion Body Myositis Tissue involved Inf. Of Skeletal Muscle Inf. Of Skeletal Muscle Inf. Of Skeletal Muscle Fibre Fibre & Skin Fibre Age Adults Children & Adults Adults>50 years Clinical Presentations Bilateral Proximal Bilateral Proximal Asymmetrical Distal Muscle Weakness Muscle Weakness. Muscle Weakness Skin Rash of Upper Eyelid Periorbital edema Microscopic Features Endomysial lymphocytic Perimysial and vascular Cytoplasmic Vacuoles Infiltration( CD 8) lymocytic infiltration and basophilic granules and amyloid. Skeletal muscle fibre Perifasciular Fibre regeneration and Atrophy EM: Filamentous degeneration Inclusions Skeletal muscle fibre regeneration and degeneration Cancer Risk: Increased Risk Of : Lung , Stomach & Ovarian Cancers.
  • 3. Myasthenic Syndromes. Disease Myasthenia Gravis Eaton-Lambert Gullian- Barre Syndrome Syndrome Mechanism Autoantibodies against Autoantibodies against Antibodies against Ach. Receptors in NMJ Ca. Ion Channels in NMJ Schwann cells leading to Peripheral Nerve Demyelination & inflammation Associated Diseases Thymoma or Thymic Small Cell Lung Cancer Preceded by Viral hyperplasia illness Gender Females > Males Clinical Presentation Muscular weakness of Proximal Muscle Muscle weakness Body but predominantly Weakness with an ascending affecting: paralysis. Facial Muscles Extraoccular Muscles with Loss of Deep Ptosis tendon reflexes May Involve Respiratory May Involve Muscles and cause Death Respiratory Muscles & cause death Alleviating Factors Weakness worsens with Weakness improves with repeated contractions. repeated contractions Diagnosis Nerve Conduction Studies.
  • 4. Muscular Dystrophy Disease Duchene Muscular dystrophy Becker Muscular Dystrophy Incidence Most Common & Severe Form of Less Common & Mild Form of Muscular dystrophy Muscular dystrophy Genetics X-linked Recessive X-linked Recessive Mutations Mutations lead to Absence of Mutations lead to Alteration of Dystropin protein Dystropin protein Clinical Presentations Rapidly Progressive Muscular Slowly Progressive Muscular Weakness weakness. Proximal weakness of Shoulder Muscle weakness of Pelvis and and pelvic Girdle Legs Calf Pseudohypertrophy Heart Failure and arrhythmia Heart involvement is rare. Respiratory insufficiency and infections Age 5- Onset of symptoms Later onset with variable progression and may lead a Age 10- Wheel chaired normal life span Age 20- Respiratory Failure and death Microscopic Features. Fibrosis and Fatty infiltration and Necrosis Skeletal muscle degeneration Skeletal muscle degeneration and regeneration and regeneration Diagnosis Muscle Biopsy Muscle Biopsy DNA Analysis DNA Analysis
  • 5. Benign Soft Tissue tumors Tumors Type of cells Locations Lipoma Adipose tissue Trunk ( Most Common) Neck Proximal Extremities Dermatofibroma Dermal Spindle cell Extremities (Forms a red nodule that can be squeezed) Fibromatosis Fibrous tissue Infiltrates Muscle tissue Rhabdomyoma Striated Muscle cells Heart Tongue Vagina Leiomyoma Smooth muscle cells Uterus Stomach Malignant Soft Tissue Cancers Cancers Type of cells Locations Liposarcoma Adipose tissue Thigh ( Most Common) & lipoblasts Retroperitoneum Fibrosarcoma Thigh Upper Limb Malignant Fibrous Pleomorphic cells Thigh Histocytoma Retroperitoneum Embryonal Striated muscle cells Male โ€“ Mass seen protruding Rhabdomyosarcoma form penis Female- Mass seen protruding from Vagina Leiomyosarcoma Smooth Muscle cells Uterus GIT Neurofibrosarcoma Nerve cells Peripheral Nervous system Synovial Sarcoma Glands and spindle cells Around Joints