One of the benefits of being the only Dermatologist in a country of 2 million people is that all the weird skin stuff eventually makes its way to you. During any one rotation here in Botswana it's possible to see about 1/12th of every rare skin disease that exists in the entire country. So you get to see some very rare conditions, indeed. The majority of these conditions I've only read about and seen pictures of prior to his trip, but it was still gratifying to know that I could recognize them based on their description and pathology.
Kicking things off with a bang:
Leprosy!
This gentleman is a refugee from the Democratic Republic of The Congo which has some of the highest rates of Leprosy in the world. While Botswana itself has fairly low rates, it is surrounded by countries that are rife with Leprosy and due it's relative political and economic stability, sees frequent refugees from these areas.
This guy fits into the WHO classification of 'multi-bacillary' leprosy and, as such, will require long-term therapy with Dapsone, Rifampin, and Clofazamine -- over a year's worth. Fortunately the Ministry of Health in Botswana has a program set up for treatment of leprosy patients and there are blister-packs available for both multi- and pauci-bacillary forms of leprosy so it is a fairly simple matter to refer them to the leprosy clinic at the MOH and get them started on the correct therapy. Unfortunately the dermatologist who initially saw him was unaware of this fact and tried to get Clofazamine through the normal hospital channels, which is nearly impossible.
As you're probably aware, Leprosy can attack the peripheral nerves and this gentleman had a palsy of the ulnar nerve on the left side which was causing him considerable distress. Hopefully now that he is on the proper treatment he will not have any further nerve damage.
Job's Syndrome
I've already mentioned the patient I saw with Hyper-IgE syndrome (Job's syndrome) in a previous post, but here is a quick update on the patient who came in for follow-up this past week.
She was started on oral Ketoconazole and Cephepime for prophylaxis and was much improved.
I saw a family of four this past week, all of them with Ichthyosis Bullosa of Siemens (Congential Bullous Ichthyosiform Erythroderma) which is caused by an autosomal dominant mutation in Keratin 2e. I doubt I would have picked up this diagnosis based on their presentation, as they didn't quite look like the pictures I had seen in texts, but 2 of the family members had been biopsied which had confirmed the diagnosis.
Pemphigus Foliaceus. Not exceedingly rare, but this is certainly the worst case I have ever seen. This patient had about 100% body-surface-area involvement and was miserable. He was sent to me from Maun, in the far Northwest of the country and the call I got originally stated that he had pemphigus vulgaris. With the referring doctors description and diagnosis of PV I was pretty worried about this guy's survival chances but, as it turns out, he mostly just extremely uncomfortable with pemphigus foliaceus.
I admitted him to the male medical ward and started him on oral steroids and antibiotics. The plan is to start Dapsone in a week after checking a G6PD screen.
Again, not exceedingly rare, but this is the worst case of
Vitiligo that I have ever seen. In an area with less sun she would be a candidate for depigmentation therapy, where the patient is treated with a chemical that completely depigments the skin in order to have a consistent color. Here in Botswana that would place her at too high of a risk for skin cancer so it wasn't considered.
Worst case of
Psoriasis I have ever seen. She had almost 100% body suface area involvement with some interesting verrucous changes on the legs. These pictures are after admission and three weeks of therapy with Cyclosporine. She is about 50%
improved. Prior to therapy she was in so much pain that she couldn't walk. Her family carried her in suspended in a blanket. She looks pretty bad still, but she was able to walk in and feels much better.
Juvenile Dermatomyositis. This little girl had been diagnosed via biopsy.
This guy had pretty impressive
Hypertrophic Lichen Planus.
You guys seem to really like my
keloid pictures. This guy had even more extensive keloids than the one I posted previously. He was a former mine worker, and described getting hit with sparks all over his body which gave him tons of small burns. I gladly wrote a letter to the mining concern in an attempt to get him an increased pension. He had significant decrease mobility and was in considerable pain.
This guy was already treated, but still pretty interesting -
Pellagra, or 'Casale's Necklace'. This is caused by a deficiency in Niacin.
Signing off, from Cleveland.
