Dermatology Online Journal

Development of disseminated superficial porokeratosis in a patient with complicated acute pancreatitis
O Ferreira^1,2, AF Duarte^1,2, T Baudrier¹, A Mota^1,2, F Azevedo¹
Dermatology Online Journal 17 (3): 5

1. Department of Dermatology and Venereology, Hospital de São João EPE, Porto, Portugal
2. Faculty of Medicine of Porto, Porto, Portugal

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Abstract

Porokeratosis is a group of hereditary or acquired diseases with abnormal epidermal keratinization. A 71-year-old man was admitted to the Surgery Department after an attack of acute pancreatitis complicated with pancreatic necrosis, which required surgical resection. Three weeks after the admission, the patient was observed by our Dermatology department with sudden onset of a generalized eruption of asymptomatic flat papules with a hyperkeratotic rim, sparing the face, palms, soles, and mucous membranes. A skin biopsy was performed at the border of a leg lesion, which disclosed the presence of cornoid lamella, confirming the clinical diagnosis of disseminated superficial porokeratosis (DSP). The skin eruption spontaneously subsided about one month after pancreatic resection. The late onset of DSP in our patient may represent a type of immunossuppression-induced porokeratosis. Possibly, the pathologic clone of keratinocytes for porokeratosis was present, but remained latent until there was a decrease in the immunological status. It is possible that this relative and transient state of immunossupression was the result of the concurrent necrotizing pancreatitis. This case represents an unusually good outcome of DSP. To the authors best knowledge, this is the first case of DSP related to severe acute pancreatitis.

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Porokeratosis is a group of hereditary or acquired diseases with abnormal epidermal keratinization [1, 2]. Clinically, the condition is characterized by annular or gyrate papules and plaques, with central atrophy and sharply elevated hyperkeratotic borders. The typical histologic hallmark is the cornoid lamella. There are six clinical variants described, including Mibelli or plaque type, disseminated superficial, disseminated superficial actinic, linear, punctuate, and palmaris et plantaris disseminata. Recently some reports of disseminated superficial porokeratosis (DSP) have been described in association with immunossuppression.

Figure 1	Figure 2

Figure 3

A 71-year-old man was admitted to the Surgery Department because of acute pancreatitis complicated with pancreatic necrosis, which required surgical resection. Three weeks after admission the patient was observed by our Dermatology department because of the sudden onset of a generalized eruption of asymptomatic thin papules with a hyperkeratotic rim, sparing the face, palms, soles, and mucous membranes (Figures 1 through 3). A history of similar skin changes in his family or of excessive sun exposure was denied. A skin biopsy was performed at the border of a leg lesion, which disclosed the presence of a cornoid lamella, confirming the clinical diagnosis of DSP (Figure 4). The skin eruption spontaneously subsided about one month after pancreatic resection (Figure 5).

Figure 4	Figure 5

Porokeratosis is an uncommon disorder of unknown etiology. Nevertheless, in patients with a genetic predisposition it is possible that external triggering factors can activate an abnormal clone of epidermal keratinocytes. In this setting several predisposing conditions have been reported, namely bacterial and viral infections (group A streptococcus, herpes simplex virus, HIV), autoimmune diseases (dermatomyositis, Sjögren syndrome, rheumatoid arthritis), inflammatory bowel disease, organ transplantation, lymphoproliferative disorders, and solid malignant tumors (ovarian and colon cancer, cholangiocarcinoma) [1-6]. Although the mechanism of dissemination of porokeratosis remains unclear, several hypotheses have been proposed. For instance, the overexpression of the p53 gene has been implicated in the pathogenesis of porokeratosis associated with malignant solid organ tumors [6]. In the case of infections, autoimmune or inflammatory diseases, an immunological impairment is the most commonly proposed mechanism, In fact, the late onset of DSP in our patient may represent a type of immunossuppression-induced porokeratosis. The pathologic clone of keratinocytes for porokeratosis was present, but remained latent until there was a decrease in the immunological status. It is possible that this relative and transient state of immunossuppression was the result of the concurrent necrotizing pancreatitis in a elderly patient, sufficient enough to trigger the porokeratosis, which resolved upon improvement of the overall patient condition after the surgery. In the light of this view it is interesting that Ueda et al described in a series of patients significant immunological alterations occurring in the course of severe acute pancreatitis [7].

The treatment of DSP must be individualized, based on the number and size of the lesions, anatomical location, aesthetic considerations and patient preference. Although there are multiple treatments available, usually the response of porokeratosis to therapy is poor and the disease progresses. Occasional success has been reported with topical corticosteroids, retinoids, 5-fluorouracil (5-FU), imiquimod, vitamin D3 analogues, cryotherapy, and laser [5, 8]. Recently, a case of success was described with the aggressive combination of topical imiquimod and 5-FU [8]. Interestingly, in our patient no treatment was needed thanks to the spontaneous regression after the resection of the necrotic pancreas, which supports the hypothesis that necrotizing pancreatitis was the triggering event. Similarly Cannavó et al reported an unusual regression of an ovarian cancer-related porokeratosis after initiating the chemotherapy [1].

In conclusion, this case represents an unusually good outcome of DSP. To the authors’ best knowledge this is the first case of DSP related to severe acute pancreatitis.

References

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