Clinical case study 1

Patient Presentation

A 55-year old HIV-infected male was referred to the Dermatology Outpatient Clinic with a four month history of a swollen face and multiple skin nodules.

Acknowledgement

  • Ranks Lehloenya, Dr. Khadija Shebe and Prof. Gail Todd from the Department of Dermatology.
  • Dr Sipho Dlamini from the Department of Infectious Diseases, Groote Schuur Hospital, Cape Town.
  • Dr Carol Hlela, Institute of Infectious Disease and Molecular Medicine, University of Cape Town

History

Three years prior to this presentation, the patient was treated at his local clinic for a persistent rash, diagnosed as eczema. There was minimal response to a standard therapy of topical steroids, he experienced only a minimal response.

Past Medical History

He was diagnosed with pulmonary tuberculosis (TB) and tested positive for HIV at the same time. He completed six months of standard TB treatment.

His CD4 count over three months showed a steady decline:

  • 1st measurement: CD4 count was 987 cells/ul
  • 3 months later his CD4 count was 603 cells/ul

HAART was initiated five months later when his CD4 count dropped below 350 cells/ul. He was started on antiretroviral therapy (ARV), consisting of lamivudine (3TC), stavudine (d4T) and efavirenz (EFV)

He defaulted treatment and was lost to follow-up for more than a year.

When he returned to the clinic he was re-initiated on ARV.  Four months after restarting treatment he developed facial swelling and bulky, non-tender nodules on the trunk and limbs.

A skin biopsy showed dermal infiltration of atypical lymphoid cells.

Following his biopsy results, he was referred to a specialist skin lymphoma clinic.

Past Surgical History

Previous laparotomy for small bowel obstruction, cause unknown.

Family History

Nothing of significance

Allergies

None known

Medication

Topical steroids

ARV: 3TC, d4T and EFV

Travel History

None noted

Social History

Non-smoker

No alcohol use

No illegal substance use

Differential Diagnosis

  • HIV-associated atypical cutaneous lympho-proliferative disorder (ACLD)
  • Leprosy
  • Mycoses fungoides
  • Sezary syndrome
  • Other lymphomas

Examination

Appearance: ambulatory, underweight, erythrodermic male, awake, alert and co-operative.

Vitals

  • Temperature: afebrile
  • Blood pressure: 124/76
  • Heart rate: 75
  • Respiratory rate: 16

General

  • Erythrodermic, skin, generally indurated
  • Palpable axillary and groin lymph nodes
  • No jaundice, pallor or oedema

Chest

  • Chest clear

Cardiovascular

  • Normotensive
  • No murmurs, no added heart sounds

Abdomen

  • Mild tenderness over the liver and the spleen with accompanying hepatosplenomegaly.

Neurological

  • No abnormalities detected

Dermatological

  • Erythrodermic, skin generally indurated
  • Diffusely indurated face with leonine features
  • Boggy non-tender multiple nodules and tumors on the face, of varying sizes
  • Similar tumourous nodules on thighs
  • Fine non-palpable purpuric rash on upper the trunk
  • Non-tender tumourous nodules on forearm
   
  On Admission  6 weeks 14 weeks Reference ranges
WCC +peripheral smear 50.51 with > 10%  circulating atypical lymphocytes 8.6 11.1 4-12×109/L
Hb 13.3 10.6 7.4 12.1-15.2g/L
MCV 105 79-98.9fL
MCH 33.4 27-32.0pg
MCHC 31.8 32-36.0g/dL
Platelets 383 93 130 140-450×109/L
Diff Count:
Neutrophils 6.57 2.0-7.50 x109/L
Lymphocytes 34.35 1-4×109/L
Monocytes 3.54 0.18 – 0.80 x109L
Eosinophils 1.01 0.0 – 0.45 x10 9 /L
CD4 count 94
Viral Load 1890

 

Discussion points to consider:

Our presenting case was a middle-aged man with severely indurated face presenting as a leonine facies as well as skin nodules and tumours on his trunk and limbs. He was previously diagnosed as HIV-infected and was initiated on ARV. It is uncertain whether the skin lesions were a result of drug toxicity following initiation of ARV, or possibly due to immune reconstitution inflammatory syndrome (IRIS). It is also possible this could be an aggressive progression of a pre-existing condition due to his immune-compromised state

What primary immune response to foreign antigen in the skin?

Homing of effector memory CD4+ T cells to the skin.

Circulation and homing of central memory CD4+ T cells.

How do effector memory CD4+ T cells migrate to the epidermis?

What is happening in our patient?

What happens in Sezary Syndrome?

What happens to these transformed malignant CD4+ T cells?

How are Sezary Cells Identified?

Final Outcome

Despite stopping HAART and administering chemotherapy along with radiotherapy, the disease continued to worsen and the patient’s skin lesions ulcerated and he developed progressive systemic disease. During the fourth month following admission he contracted pneumonia and died shortly thereafter.