Indian Journal of Dermatology
CORRESPONDENCE
Year
: 2011  |  Volume : 56  |  Issue : 2  |  Page : 251--252

Authors' reply


Saumya Panda1, Koushik Lahiri2,  
1 Department of Dermatology, KPC Medical College, Kolkata, India
2 Department of Dermatology, Apollo Gleneagles Hospital, Kolkata, India

Correspondence Address:
Saumya Panda
Department of Dermatology, KPC Medical College, Kolkata
India




How to cite this article:
Panda S, Lahiri K. Authors' reply.Indian J Dermatol 2011;56:251-252


How to cite this URL:
Panda S, Lahiri K. Authors' reply. Indian J Dermatol [serial online] 2011 [cited 2020 Oct 29 ];56:251-252
Available from: https://www.e-ijd.org/text.asp?2011/56/2/251/80449


Full Text

Sir,

We thank the correspondent, Dr. Jayanta Kumar Das, for the prompt, thoughtful, painstakingly detailed reply [1] to our case report. [2] He makes his view abundantly clear from the outset, right from choosing a provocative title to the effect that he does not agree with the diagnosis. He weakens his own case when, in the concluding sentence, he avers, a little patronizingly, "The case reported by Panda et al. is compatible with the diagnosis of necrolytic acral erythema". However, he is quick to add, "Enough evidence has not been produced by the authors to label it with that diagnosis". As ordinary readers, we are left confused. Is it not like saying that it is justifiable to say that smoking causes cancer and, at the same time, adding that there is not enough evidence to say that smoking causes cancer?

The correspondent's objections mainly revolve around the interpretation of the histopathology. His contention is that the photomicrograph as well as the histopathologic description resembles eczema rather than NAE. In 2004, Abdallah et al. described the histopathologic features of lesions from 30 patients with NAE. [3] Until now, this has been the largest series of NAE focusing on microscopic features. Early stage lesions in that study demonstrated moderate regular acanthosis with variable spongiosis and inflammatory infiltrates resembling nummular dermatitis. It would not be illogical to say that our case has had a strong similarity to this description. Also, we would like to remind the correspondent that the duration in our case was of only 2 months.

In 2005, the same group further widened the ambit of their study and described the natural history of progression of NAE and evolution of the skin lesion in three stages. [4] This remains a path-breaking study and is a must-read for anyone working on NAE. Anyone following these two studies in tandem would concur that any particular histopathologic characteristic is not pathognomonic in NAE.

The presence of pale, vacuolated keratinocytes in the upper epidermis, leading to focal or confluent necrosis, a process termed "necrolysis", [5] is thus neither the sine qua non nor an absolute necessity for the diagnosis of NAE. Necrotic keratinocytes are just one of several characteristic findings that one comes across depending on the stage of disease that has been encountered. The absence of necrolysis in our case is adequately explained by the fact that there were neither any blisters nor any pustules in our case, which macroscopically represent most reliably the process of necrolysis. Our case was simply in too early a stage of disease progression to demonstrate these features.

The correspondent goes on to cite the histopathologic characteristics of the seronegative cases cited in our references in an attempt to demonstrate the unsuitability of the present case to be labeled as one of them. The first case that he cites, that of Liu et al., [6] is obviously a well-established case, if the staging of Abdallah et al.[4] is followed. There was nothing "unique" about the microscopy, as the correspondent suggests, but it correlates fully with the morphologic evolution.

The second case, that of Wu et al., [7] is more interesting. The correspondent forgets to mention that the case was diagnosed as one of systemic lupus erythematosus (SLE) 12 years prior to being diagnosed as having NAE. The psoriasiform lesions in this case were present from the very beginning. Whether the dyskeratotic cells here represent lichenoid change of lupus erythematosus is anybody's guess. Even if one ascribes the apoptosis to NAE, this case should be treated as a very special one, given its clinical background.

Regarding histopathology, the correspondent misses the wood for the trees by failing to correlate microscopic findings with the disease dynamics, and also misses out on the simple fact that NAE is always to be diagnosed on the basis of clinicopathologic correlation, rather than depending on any histopathologic straitjacket.

Now, let us take a look at the correspondent's objections regarding what he chooses to call "purportedly characteristic" clinical features of this case. As he opines, "… the morphology was not distinctive enough to differentiate it from psoriasis, eczema, and tinea". Now, which case of NAE is distinctive enough on purely clinical grounds from psoriasis? Hardly any, we presume. Just take a look at the clinical photos of previously published NAE cases and that of ours. The resemblance is nothing less than striking. That is why, we chose to describe the case as having "classic morphology", and we stand by that description.

The correspondent seems fixated on this case being one of eczema. He chooses to ascribe the clinical improvement to topical clobetasol rather than oral zinc. Besides the obvious question as to what could possibly be the etiology of such a well-circumscribed "eczema" of such short duration other than contact dermatitis (just to recount, patch test was non-contributory in this case), he chooses to disregard the crucial fact that the initial pre-treatment photograph was taken after the patient had already been administered topical and systemic steroids. So, the only difference in therapy which could conceivably result in such visible difference in outcome was oral zinc. And then, in our limited combined clinical experience, both the authors are yet to encounter a single case of eczema, of whatever variety, with such severe inflammation, that is absolutely devoid of itch!

Another point is that Wu et al., [7] who the correspondent has quoted quite extensively from, had an interesting sidelight to offer. They noticed that discontinuation of oral steroids preceded the most striking improvement in skin lesions of their patient. They failed to offer any cogent reason behind this, but suggested that this phenomenon suggests the role of some immune imbalance in the causation of NAE. We venture to add that our patient too perhaps demonstrated a similar pattern of post-systemic steroid flare, as the history and the first photograph in our report would suggest when taken together.

The correspondent writes, "Low serum albumin and low-normal zinc levels are common enough to be found as non-significant incidental finding". We would humbly maintain that this may not be necessarily so. A careful perusal of the NAE literature would inform us that el Darouti and Abu el Ela, [8] who described the entity in the first place, postulated that hypoalbuminemia and resultant hypoaminoacidemia may lead to epidermal protein depletion, resulting in necrolysis of epidermal cells. Later, Nofal et al.[9] in 2005 hypothesized that decreased serum albumin levels could lead to high levels of prostaglandins, thereby inducing inflammatory changes of NAE; decreased serum albumin levels also might cause a transitory deficiency in zinc or essential fatty acids in plasma.

In essence, we find the concerns raised by the correspondent to be based on labored presumptions rather than scientific rationale. One makes a clinical diagnosis based on holistic reasoning, particularly when the entity is such a polymorphic one as NAE. Any objection about such a diagnosis should also be made bearing such multidimensionality in mind. Otherwise, one would commit the mistake as the correspondent does, picking and choosing random characteristics and ignoring, or failing to discern, the overall pattern. We labeled the case as NAE not merely because it was the most probable diagnosis, but because it was the only possible one.

A minor correction is to be made. The correspondent writes, "The authors have mentioned that absence of reticular degeneration of superficial keratinocytes with dyskeratosis and vacuolization of basal keratinocytes on microscopy are atypical histological features for NAE". On the contrary, what we had written was something very different: "Other than being HCV-negative, the other atypical features of the case were the involvement of soles and nails and absence of reticular degeneration of superficial keratinocytes with dyskeratosis and vacuolization of basal keratinocytes on microscopy, though there are cases in the literature that have been diagnosed as NAE with these atypical features". [10] On hindsight, this passage might have resulted in an error of communication. To the uninitiated, our statement perhaps suggests a far greater significance of dyskeratosis than is actually the case. A reference to standard dermatopathology textbooks (e.g., Weedon [11] ) to point out the fact that focal necrosis of keratinocytes is just another histopathologic characteristic could have obviated the need for this lengthy clarification. We quoted Bentley et al.[12] not in defense of our diagnosis, as the correspondent suggests, but to acquaint the reader with the multiplicity of microscopic features that NAE may present with. Our case, we may humbly add, does not need any defense; it stands firmly on its feet and may withstand any offence.

Once again, we record our note of appreciation and congratulate the correspondent for his erudite comments that have given us a chance to further clarify ourselves.

References

1Das JK. Diagnosing necrolytic acral erythema: Does anything go? Indian J Dermatol 2011;56:238-9.
2Panda S, Lahiri K. Seronegative necrolytic acral erythema: A distinct clinical subset? Indian J Dermatol 2010;55:259-61.
3Abdallah MA, Ghozzi MY, Monib HA, Hafez AM, Hiatt KM, Smoller BR, et al. Histological study of necrolytic acral erythema. J Ark Med Soc 2004;100:354-5.
4Abdallah MA, Ghozzi MY, Monib HA, Hafez AM, Hiatt KM, Smoller BR, et al. Necrolytic acral erythema: A cutaneous sign of hepatitis C virus infection. J Am Acad Dermatol 2005;53:247-51.
5Franchimont C, Pierard GE, Luyckx AS, Gerard J, Lapiere CM. Angioplastic necrolytic migratory erythema. Am J Dermatopathol 1982;4:485-95.
6Liu A, Erickson CP, Cockerell CJ, Hsu S. Necrolytic acral erythema: A case not associated with hepatitis C infection. Dermatol Online J 2008;14:10.
7Wu YH, Tu ME, Lee CS, Lin YC. Necrolytic acral erythema without hepatitis C infection. J Cutan Pathol 2009;36:355-8.
8el Darouti M, Abu el Ela M. Neorolytic acral erythema: a cutaneous marker of viral hepatitis C. Int J Dermatol 1996;35:252-6.
9Nofal AA, Nofal E, Attwa E, El-Assar O, Assaf M. Necrolytic acral erythema: a variant of necrolytic migratory erythema or a distinct entity? Int J Dermatol 2005;44:916-21.
10Panda S, Lahiri K. Seronegative necrolytic acral erythema: A distinct clinical subset? Indian J Dermatol 2010;55:259-61.
11Weedon D. Skin Pathology, 2 nd ed. London: Churchill Livingstone; 2002. p. 554.
12Bentley D, Andea A, Holzer A, Elewski B. Lack of classic histology should not prevent diagnosis of necrolytic acral erythema. J Am Acad Dermatol 2009;60:504-7.