Clinic Hours

History: A 9 yr child with pruritic and painful rashes on the lower extremities and hands. Arthritis and abdominal pain were also noted. What’s the diagnosis?

1. IgA Vasculitis
2. Hypersensitivity vasculitis
3. Leukocytoclastic vasculitis
4. Meningococcemia
5. Rickettsial diseases

Answer: Henoch Schonlein purpura (HSP) also known as IgA vasculitis is an acute IgA mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. IgAV is the most common vasculitis in childhood. The disease is more benign in children than in adults.

Signs & Symptoms: Purpura, arthritis, and abdominal pain are known as the “classic triad” of Henoch–Schönlein purpura.

Headache, Anorexia, Fever, Rash (95-100% of cases), especially involving the legs; this is the hallmark of the disease, Abdominal pain and vomiting (35-85%), Joint pain (60-84%), especially involving the knees and ankles, Subcutaneous edema (20-50%), Scrotal edema (2-35%) or Bloody stools.

Diagnosis: Urinalysis, KFT, CBC, PT-INR, aPTT, Biopsy

DDx: IgAV may mimic an abdominal emergency, Papular urticaria, SLE, meningococcemia, dermatitis herpetiformis.

Treatment: Combination of IV methylprednisolone (steroid), cyclophosphamide and dipyridamole followed by prednisone. IVIG.

Clinical Rounds by ClinicHours

History: A 33 yr female presented with fatigue & flat painless purple lesions on the nose. She had a history of HIV infection and a low CD4 cell count. What’s the diagnosis?
1. Hemangioma
2. Kaposi Sarcoma
3. Bacillary Angiomatosis
4. Purpura

Answer: Kaposi sarcoma (KS) is an indolent angio-proliferative spindle-cell tumor derived from endothelial and immune cells infected with human herpes virus type 8 (HHV-8).

Four sub-types are described:

1. Classic KS tends to affect older men in regions where KSHV is highly prevalent (Mediterranean, Eastern Europe, Middle East), is usually slow-growing, and most often affects only the legs.
2. Endemic KS is MC in Sub-Saharan Africa and is more aggressive in children, while older adults present similarly to classic KS.
3. Immunosuppression therapy-related KS generally occurs in people following organ transplantation and mostly affects the skin.
4. Epidemic KS occurs in people with AIDS and many parts of the body can be affected.

Symptoms: Lesions are nodules or blotches that may be red, purple, brown, or black, and are usually papular. They are typically found on the skin, mouth, gastrointestinal tract, and respiratory tract. Growth can range from very slow to explosively fast, and is associated with significant mortality and morbidity. The lesions are painless, but become cosmetically disfiguring or interruptive to organs.

Diagnosis: KS is diagnosed by tissue biopsy, CD4 count

Treatment: HAART, Combination therapy with regimens such as ABV (actinomycin D, bleomycin, vincristine) or single-agent therapy such as doxorubicin. Cytotoxic agents like doxorubicin, daunorubicin, Paclitaxel or oral etoposide. Radiation therapy, cryotherapy, laser therapy or surgical excision.

Clinical Rounds by ClinicHours

History: A healthy 10-year-old boy had a 12 months history of an itchy right eye with frequent tearing. Small masses that gradually developed in the right caruncula lacrimal area and multifocal small nodules with papillomatous growth and vascular fronds.

What’s the diagnosis?

1. Chalazion
2. Papilloma
3. Pterygium
4. Pinguecula
5. Coloboma

Answer: Conjunctival papilloma is an acquired benign epithelial tumor of the conjunctiva and may manifest as a sessile or pedunculated conjunctival mass.

Cause: Human papillomavirus (HPV) 6 and HPV 11

Signs:

• This lesion is typically benign.
• It is seen commonly in older adults.
• Anatomically, the lesion commonly occurs at the limbus or the bulbar conjunctiva.
• These lesions may spread centrally toward the cornea or laterally toward the conjunctiva.
• Visual acuity may be affected if the lesion grows centrally.
• These lesions almost always are unilateral and single.
• They tend to have variable proliferation potential with a tendency to slowly enlarge in size.

Differential diagnosis: Ichthyosis, Sebaceous Gland Carcinoma, Conjunctival Squamous Cell Carcinoma

Treatment: Surgical excision, Erilesional cryotherapy, intralesional or topical interferon-α injection, carbon dioxide (CO2) laser, topical mitomycin-C (MMC), and oral cimetidine have been reported to be adjunctive treatments. Recurrences are not uncommon.

Reference: AAO, NEJM

Clinical Rounds by ClinicHours

The Fugates, a family who lived in the hills of Kentucky, commonly known as the “Blue Fugates” or the “Blue People of Kentucky”, are notable for having been carriers of a genetic trait that led to methemoglobinemia, which causes the appearance of blue-tinged skin.

Martin Fugate and Elizabeth Smith who had married and settled near Hazard, Kentucky, around 1820, were both carriers of the recessive methemoglobinemia (met-H) gene. As a result, four of their seven children exhibited blue skin, and continued progenation within the very limited local gene pool ensured that many descendants of the Fugates were born with met-H.

A hematologist Madison Cawein treated the family with methylene blue, which eased their symptoms and reduced the blue coloring of their skin.

Reason: The underlying mechanism involves some of the iron in hemoglobin is converted from the ferrous [Fe2+] to the ferric [Fe3+] form.

Signs and symptoms: (methemoglobin level above 10%) include shortness of breath, cyanosis, mental status changes (~50%), headache, fatigue, exercise intolerance, dizziness, and loss of consciousness. People with severe methemoglobinemia (methemoglobin level above 50%) may exhibit seizures, coma, and death (level above 70%).

Causes:

1. Genetic: Due to a deficiency of the enzyme diaphorase I (cytochrome b5 reductase), Hereditary met-Hb, HbM, or HbH.
2. Acquired: Drugs – trimethoprim, sulfonamides, and dapsone, local anesthetics especially articaine, benzocaine, prilocaine, lidocaine, and aniline dyes, metoclopramide, rasburicase, umbellulone, chlorates, bromates, and nitrites.

Differential diagnosis: Argyria, sulfhemoglobinemia, heart failure

Treatment: Supplemental oxygen and methylene blue given as a 1% solution (10 mg/ml) 1 to 2 mg/kg administered IV slowly over five minutes.

Note: The image shown above is of Mr. Paul Karason who consumed silver as a home remedy for severe dermatitis and later on developed argyria.