Clinic Hours

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.

History: A 50-year-old woman presented with asymptomatic multiple cutaneous nodules all over the body of 4 months duration. Cutaneous examination showed multiple hyperpigmented nodules and plaques involving face, trunk, and extremities. Peripheral smear showed abnormally elevated leucocyte count (TLC-70,000) with abnormal cells: myeloblasts 40%, promyelocytes 8% and myelocytes 39%. Auer rods were present in few myeloblasts. What is the most likely diagnosis?

1. Gottron’s papules
2. Guttate psoriasis
3. Immune thrombocytopenia purpura (ITP)
4. Leukemia cutis
5. Solar purpura

Answer:

Leukemia cutis is the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutis, resulting in clinically identifiable cutaneous lesions. Leukemia cutis may follow, precede or occur concomitantly with the diagnosis of systemic leukemia.

The clinical appearance of leukemia cutis is variable with erythematous to violaceous papules or nodules being the most frequent lesions (60%), followed by infiltrated plaques, generalised cutaneous eruption and erythroderma. They are frequently asymptomatic. The nodules are typically firm or rubbery in consistency, and can become purpuric if the patient is thrombocytopenic.

Source: PMC

Clinical Rounds by ClinicHours

History: A 30 yr old obese man presents with an asymptomatic rash on his back, arms, and hands that had developed 2 week earlier. On physical examination, scattered pink-yellow papules were present on the upper back, extensor surfaces of the upper arms, and dorsa of the hands. A fasting blood sample was grossly lipemic. Which of the following is the most likely diagnosis?

1. Eruptive xanthomas
2. Sebaceous hyperplasia
3. Granuloma annulare
4. Molluscum contagiosum

Answer: Eruptive xanthomas typically present as crops of 2–5 mm yellow papules with a red rim over extensor surfaces such as the buttocks or shoulders, but can be widespread including inside the mouth. The papules may be tender and are usually itchy. They may demonstrate köbnerisation. Eruptive xanthomas are due to hypertriglyceridaemia (triglyceride >11.2 mmol/L) of any cause.

Source: Dermanetz