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Elephantine Psoriasis with Papillomatosis And DERMATOPATHOLOGY ROUND Elephantine Psoriasis with Papillomatosis and Alternating Hypogranulosis and Hypergranulosis Sankha Koley, Rajesh Kumar Mandal1, Kingshuk Chatterjee, Sk Masud Hassan, Swapan Pathak2 Abstract From the Department of Psoriasis is a disease of considerable clinical and histopathological diversity. We report a rare Dermatology, B. S. Medical 1 case of elephantine psoriasis responding very well to methotrexate. Histopathology revealed College, Bankura, Department abnormal papillomatosis with finger-like projections in addition to alternating orthokeratosis of Dermatology, Dept of North Bengal Medical College, with overlying hypergranulosis and parakeratosis with overlying hypogranulosis. We believe Susrutanagar, Darjeeling, that this finding may represent an odd histopathologic type in elephantine psoriasis. 2Department of Pathology, BS Medical College, Bankura, Key Words: Elephantine psoriasis, hypergranulosis, hypogranulosis, ILVEN, papillomatosis West Bengal, India Address for correspondence: Dr. Sankha Koley, Department of dermatology, BS Medical College, Bankura, West Bengal, India. E-mail: [email protected] What was known? • Elephantine psoriasis is a rare variant of hyperkeratotic psoriasis • A PubMed search of articles indexed for MEDLINE revealed no articles on elephantine psoriasis and its histopathology • Psoriasiform changes in addition to alternate bands of hypergranulosis and hypogranulosis, an extremely rare presentation in psoriasis, are a characteristic feature in inflammatory linear epidermal nevus (ILVEN). Introduction with positive Auspitz sign and Grattage test. There was Psoriasis is a disease of substantial clinical and no history of a similar lesion in the family. He had no histopathological diversity. Elephantine psoriasis is a rare history of trauma and did not receive any drug known to variant of psoriasis. A PubMed search of articles indexed induce or exacerbate psoriasis. He had received treatment for MEDLINE did not reveal any reports of elephantine with topical corticosteroids with no improvement. psoriasis or its histopathology. We report a rare case of Routine investigations revealed no abnormalities other elephantine psoriasis and discuss the unique histological than mild leukocytosis (probably due to resolving upper features. respiratory infection). Human immunodeficiency virus and veneral disease research and laboratory (VDRL) Case Report tests were negative. Histopathologic examination A 55-year-old man presented in our hospital at of two punch biopsy specimens from hyperkeratotic out-patient department with a 6-month history of large, plaques from leg revealed hyperkeratosis, parakeratosis, itchy plaques over the legs. On cutaneous examination, microabscess of Munro, acanthosis, severe papillomatosis, symmetrical, large extensive thick scaly plaques were dermal vascular dilatation, and perivascular seen over extensor surface of both legs. The borders lymphohistocytic infiltration [Figures 2 and 3]. The were well defined and convex, but interrupted at certain striking histopathological features were abnormal parts. Few adjacent small circular plaques of different papillomatosis leading to finger-like projections sizes were present as satellite lesions. A circular thick and alternate thinning and thickening of stratum scaly plaque of similar consistency of 1.5 cm diameter granulosum [Figure 4]. Parakeratosis and orthokeratosis was present on the left thigh [Figure 1]. Rest of the body were noted overlying hypogranulosis and hypergranulosis, was not involved. The scales were silvery white in color respectively [Figure 5]. The clinical and histopathological features were consistent with elephantine psoriasis. We Access this article online started the treatment with methotrexate 10 mg per week Quick Response Code: and all lesions healed completely in 10 weeks [Figure 6]. Website: www.e‑ijd.org Discussion Psoriasis is classified morphologically into plaque, DOI: 10.4103/0019‑5154.156367 guttate, pustular and erythrodermic forms. Chronic plaque type of psoriasis includes few rare subtypes like Indian Journal of Dermatology 2015; 60(3) 264 Koley, et al.: Elephantine psorasis with alternating hypogranulosis and hypergranulosis Figure 1: Large thick scaly plaques on the extensor surface of bilateral legs Figure 2: Hyperkeratosis, parakeratosis, microabscess of Munro, acanthosis, severe papillomatosis, dermal vascular dilatation, perivascular lymphohistocytic infiltration in a scanner view (H and E, ×10) Figure 3: Microabscess of Munro (H and E, ×40) Figure 4: Papillomatosis with finger-like projections (H and E, ×40) Figure 5: Alternate hypogranulosis with overlying parakeratosis and hypergranulosis with overlying orthokeratosis (H and E, ×40) Figure 6: Lesions completely healed after 10 weeks annular (ring-shaped plaques with central clearing), lichenified and hyperkeratotic forms. The hyperkeratotic Lesions with firmly adhered thick scales, varying color form, rarely reported in the literature, is further classified and surface resembling an oyster shell are typical into ostraceous, rupioid and elephantine types.[1,2] features of ostraceous psoriasis.[3,4] The rupioid form is 265 Indian Journal of Dermatology 2015; 60(3) Koley, et al.: Elephantine psorasis with alternating hypogranulosis and hypergranulosis characterized by hyperkeratotic, concentric, circular and in the orthokeratotic epithelium, but is minimally limpet like cone-shaped plaques.[3-5] Elephantine psoriasis expressed within parakeratotic regions. By contrast, in lesions are defined as large, thick, flat long-standing psoriasis, involucrin is expressed in all layers of the plaques, typically found in the dorsal area, buttocks, epidermis except the basal layer.[12] Other authors have upper limbs[1,2] and often on lower limbs.[4] Some authors suggested that the behavior of other markers (elastin, do not distinguish between hyperkeratotic forms of antikeratin 10, antikeratin 16, Ki-67) may be useful psoriasis and use the terms interchangeably.[6] to differentiate between psoriasis and ILVEN.[13] In our patient immunohistochemistry was not considered as the In our patient, multiple enlarging circular plaques have facility was unavailable in our institute and he could not coalesced to form large extensive elephantine forms afford it from outside. on both legs. The interrupted convex margins of the plaques also suggested so. Most cases of hyperkeratotic There is a considerable overlap between the two entities, psoriasis described in the literature were resistant to that is, nevoid or linear psoriasis and ILVEN. In our topical treatment, possibly because of the overlying patient, the histopathological findings of psoriasiform thick scaly surfaces on lesions. There are reports of dermatitis along with alternate hypogranulosis and ostraceous psoriasis subsiding with immunosuppressive hypergranulosis were very typical of ILVEN. But and immunobiological drugs.[7-9] Our patient was a here distribution of lesions was not linear. Our poor farmer and so immunobiologic therapy was never patient presented with symmetrical, large extensive, considered. Our patient responded very well to 10 mg of long-standing thick scaly plaques over extensor surface weekly dose of methotrexate and all lesions completely of both legs. The morphological, histopathological and subsided in 10 weeks. topographical features of the lesions, predominantly in extensor areas, favored the diagnosis of elephantine Histologic picture of fully developed lesion of psoriasis psoriasis. is characterized by (a) acanthosis with regular elongation of rete ridges with thickening in lower Erkek and Bozdogan suggested that the presence of portions, (b) suprapapillary thinning of epidermis with finger-like projections due to exaggerated papillomatosis occasional spongiform pustules, (c) diminished to absent is an odd histologic characteristic, especially in granular layer, (d) confluent parakeratosis, (e) Munro verrucous and rupial histology.[14] Our case also showed microabscesses, (f) elongation and edema of dermal exaggerated papillomatosis in addition to parakeratosis papillae, and (g) dilated and tortuous capillaries. and orthokeratosis overlying hypogranulosis and Our patient’s histological findings were compatible hypergranulosis, respectively. Though the latter may be with diagnosis of psoriasis. It also had parakeratosis very rarely present in psoriasis, it is very characteristic and orthokeratosis overlying hypogranulosis and of ILVEN. So far we know that any case report of hypergranulosis respectively. Though the latter may elephantine psoriasis is very rare. As histopathology be very rarely present in psoriasis, it is the very report of elephantine psoriasis has never been characteristic of inflammatory linear verrucous epidermal documented before, it may be too early to conclude this nevus (ILVEN).[10] as classical histopathological feature. But we definitely propose this to be an unusual variant of histopathology ILVEN is a relatively rare, linear lesion presenting during in elephantine psoriasis. childhood, most of them arising in first 6 months of life. Scaly, erythematous papules coalesce to form linear What is new? psoriasiform plaques following the lines of Blaschko and • A case of elephantine psoriasis and its histology is reported are often associated with significant pruritus. In the • Excellent response of elephantine psoriasis to methotrexate • Histopathology revealed abnormal papillomatosis with finger‑like projections literature, there is
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