Tuesday, 17 November 2009

Answers to the November 'Spot Case'

Dear Bloggers

Thank you for waiting for the answers to the November short case which involved no history, but the sole use of observational skills.

Without further ado, here are the answers to the following questions:

Question 1: Please carefully examine the patient's arm (first photo). What physical signs can be seen here?

When we look at the picture we are immediately drawn to the skin abnormality which is diffuse, purplish and irregular. It is in fact some mild bruising rather than a melanoma, although the latter is indeed a good thought. However, this is in part, a pseudo- 'red herring'. It draws you away from the significant abnormality which is coiling of the hairs. Such a change is consistent with vitamin C deficiency (scurvy). One could indeed argue that the bruising is also caused by a vitamin C deficiency although there is not the typical peri-follicular hemorrhage that one sometimes sees in this condition.

If one increases the magnification of the picture (done by clicking on the picture) it soon becomes clear that there are whitish plug-like structures at some of the hair follicle bases which may represent lice infection.

Question 2: Please carefully examine the second photo. What physical signs can be seen here?

This photo tells a lot of information. The patient is unshaven which may signify he has not been looking after himself very well. The other clue to this is his dentition, which is very poor. There is also evidence of gingivitis, which supports the idea of poor dentition, but which may also be seen in vitamin C deficiency.

Question 3: Which ONE cause can link the main abnormalities seen below?

Vitamin C deficiency (scurvy) links both pictures.

Question 4: What is the most appropriate test(s) and subsequent treatment?

Vitamin C levels can be measured, but based on the examination findings, it would be reasonable to commence vitamin C replenishment therapy before the results are available.

The fact that this patient has a high probability of vitamin C deficiency, it should also alert the physician to consider other vitamin deficiencies and hence, replenishment of multi-vitamins would be an additional step in his treatment. The patient would also need general improvements in nutrition.

One has to also consider separate consults to dermatology, especially if there is a concern about melanoma and lice, and the dental surgeons and hygienists for optimising oral care. Lice eradication therapy would also be appropriate once confirmed by microscopic examination.

One should also consider the socioeconomic situation of the patient as well. If this patient has vitamin deficiency, possible malnutrition, and skin infestation, then he may require social help e.g. a carer for bathing and meals-on-wheels service, in addition to economic support if he has insufficient funds to cover the services required.

Both Professor Stein and Professor Dhaliwal correctly answered this month's case. Their comments are as follows:

Prof Stein:

Quesiton # 1 Problems in skin image:
1. 1) pigmented lesion of melenoma with irregular borders and 2 + different colors.And diffuse hyperkeratosis-like lesions
02)globules with tear drop forms and ? budding form suggest coiled hairs (looking like scurvy), doubt follicilitis (not looking like scurvy), yeast or parasite on skin

Question # 2
2. Front of oral cavity/lips: chronic gingivitis, candida gingivitis as seen in HIV pts, necrotizing gingivitis(trench mouth) also seen in HIV pts, and chronic periodonitis from Vit C deficiency (coiled hairs & hyperkeratosis), severe malnutrition causing necroziting periodonitis

3 & 4 For 1/1 link is CA melenoma>skin Bx; For 1/02 HIV test, ? vit C assay; For 2 xray teeth, dental consult, derm consult. Rx Vit C, multi-vit, general nutritional improvements

Prof Dhaliwal

Question 1: Please carefully examine the patient's arm (first photo). What physical signs can be seen here?

This is a wonderful exercise that asks two things of the clinician 1) observation/detection and 2) interpretation; success in #2 is intimately tied to #1. Stated otherwise, we cannot simply solve a problem (e.g., as in a presented case); we must detect and define the problem first.

The picture of the arm has a number of findings – although I cannot say with certainty which ones are decidedly abnormal. There is a dark pigmented macule that has some if not all of the ABCD characteristics of melanoma (http://www.melanomafoundation.org/prevention/abcd.htm) . One must always respect melanoma’s devastating metastatic potential when a second site of the body is being evaluated in the setting of a suspicious pigmented lesion. An alternative explanation for this skin finding would be a seborrheic keratosis, which is far more common, although typically favoring the trunk over the extremities.

The skin also seems to have punctuate yellow colored scale or buildup that is distributed throughout the skin. I regrettably don’t possess a large differential diagnosis for this finding, although things that come to mind include an exudation of excess lipid, a simple normal variant in skin pigmentation, or the accumulation of sebaceous material if regularly bathing has not been possible.

There are approximately 5-10 cystic appearing structures mostly in the middle portion of the photo. Although it is hard to tell whether they are attached to the hair shafts, I think they are at least compatible with lice. I’m hesitant to make this interpretation however, because tropism for the arm hair (in comparison to the head) would be atypical.

Finally, some of the hairs are linear, but there are a number of coiled hairs.

Question 2: Please carefully examine the second photo. What physical signs can be seen here?

This is a man, perhaps at least 30, inferred by some visible skin wrinkling, which smoking and sunexposure can accelerate. Otherwise, the external structures appear normal.

The left lower central incisor and the left lower lateral incisor are slightly discolored. The former has a grey hue, the latter, a ruddy off white appearance. Smoking may have caused a generally yellowing of the teeth. The gingival tissue associated with the lateral incisor appears to be inflamed, which may reflect a periodontal infection.

Question 3: Which ONE cause can link the main abnormalities seen below?

Since I do not recognize the dental abnormality outright, I have considered systemic diseases which may originate from periodontal disease (e.g., endocarditis) or can present as periodontal problems (drug effects like phenytoin, infiltrative processes such as leukemia, or connective tissue disturbances such as scurvy).

Given the previous observation of coiled hairs, a question I raised about hygiene (implying socioeconomic difficulties that could impair or radically modify access to food), I will hazard a guess of scurvy (vitamin C deficiency). Neither perifollicular hemorrhages nor gingival bleeding are seen however.

Question 4: What is the most appropriate test(s) and subsequent treatment?

In the case of scurvy, the diagnostic test is a serum ascorbic acid level and the treatment is the restoration of a proper diet.

Vitamin C (Ascorbic Acid) Deficiency

In developed countries the major groups affected by vitamin C deficiency include the severely malnourished, drug and alcohol misusers and the poor. In the UK, it is typically seen in the impoverished 'tea and toast' elderly.

Vitamin C deficiency (scurvy) can occur within just 3 months of deficiency resulting in various symptoms and signs largely due to impaired collagen production with impaired connective tissue organisation. Generalised symptoms include malaise, weakness, joint pain and / or swelling, depression, neuropathic symptoms, extremity swelling etc

Signs on examination include bruising of the skin, coiled hairs, bleeding gums, impaired wound healing, petechial hemorrhage, hyperkeratosis and Sjogren's syndrome.

The chronic gingivitis is thought to occur due to infection from oral bacteria in the gums of sufferers as a result of impaired neutrophil killing activity. Hemorrhage is a secondary phenomenon caused by infection disrupting fragile vessels within the gums.

Scurvy was described in the times of Hippocrates over 2,000 years ago! It was not until about 400 years ago that it was considered that 'fresh food' and if not available, citrus fruit, were the cure of the illness. About 250 years ago, the ever first medical trial took place by a Scottish doctor, James Lind, who treated sailors suffering with scurvy with various potential 'cures.' The sailors given vitamin C containing regimens recovered.

Take Home Message

  • When something does not look right then it is not right. This patient had coiled hairs, ecchymosis (bruising), poor dentition and gingivitis. The diagnosis of scurvy should be considered especially in the elderly with poor nutrition and socioeconomic problems.
  • When looking at clinical pictures look for the normal and the abnormal. Look at all of the anatomy rather than what you are initially drawn to, otherwise, the diagnosis may be missed entirely!
I would like to take the opportunity to thank Professor Stein and Professor Dhaliwal for their superb interpretations and correct answers to this difficult 'spot case'.