Inflammatory Stage of Disseminated Superficial Porokeratosis

Abstract

Disseminated superficial porokeratosis (DSP) is a keratinization disorder characterized by multiple small lesions with a slightly elevated, sharply defined ridge over the whole body. Unusual DSP cases with acute exacerbation of their lesions accompanied by severe pruritus have been reported and designated as "eruptive pruritic papular porokeratosis" or "inflammatory DSP". Histologically, the pruritic lesions in the majority of these unusual DSP cases had a dense infiltration of eosinophils and lymphocytes in the vicinity of blood vessels in the upper dermis. In this report, we describe an additional case of DSP with a similar clinical course and histopathological findings. A review of the literature showed that the pruritic condition in these unusual DSP cases can be transient and is not necessarily related to spontaneous regression. We propose the term "Inflammatory stage of DSP" for describing this unusual variant of DSP.

Hyperkeratotic variant of inflammatory disseminated superficial porokeratosis with lichenoid reaction and extensive amyloid deposition

“…The present case was characterized by hyperkeratotic erythematous papules with severe itching. Verrucous papular and/or pruritic inflammatory lesions have been previously described as rare variants of porokeratosis 1–9 . In addition to the above‐noted macroscopic features, our case was histopathologically characterized by a prominent lichenoid tissue reaction.…”

Inflammatory disseminated superficial porokeratosis with an unusual clinical feature of the pruritic, erythematous papules preceding annular brownish pigmentation

“…Porokeratosis may be associated with inflammatory changes and is known as inflammatory porokeratosis. Inflammatory porokeratosis has been reported as various diagnostic terms; eruptive pruritic papular porokeratosis (EPPP), inflammatory stage of porokeratosis or porokeratosis accompanied by eosinophilic spongiosis 2–5 . Generally, most of the patients with inflammatory porokeratosis demonstrated that the pre‐existing annular pigmented lesions caused erythematous changes and inflammation.…”

“…Six cases and the present case demonstrated inflammatory porokeratosis without pre‐existing lesions. The inflammatory lesions had disappeared within several months, leaving brownish macular or annular lesions 3–5 . The average period of the inflammation was 3.3 months.…”

Eruptive pruritic papular porokeratosis

“…By analysing 17 cases of EPPP including the one reported here, we found that EPPP might be manifested with the following characteristics (Table 1 [1][2][3][4][5][6][7][8][9][10][11][12][13][14]): (1) history of pre-existing porokeratosis varied from months (> 2 months) to several years except 1 patient who had no pre-existing lesions and no tendency to spontaneous resolution in a 16-month follow-up [4]; (2) less than half of patients had other pre-existing diseases or suspicious triggering factors, including cardiovascular disease, medication history, autoimmune disease, cancer or others;…”