Sir,
A 15-year-old male child presented with dark brown verrucous lesions over the trunk, right axilla, right elbow, and dorsum of right hand since birth. Thick white scaling over the scalp and asymptomatic linear, red, scaly plaques involving the right side of the body developed since the last 6 months. Multiple, small itchy papulosquamous lesions over the face, neck, and trunk preceded by an episode of sore throat with fever erupted for last 7 days. Past history and family history was noncontributory.
On examination, he had multiple, well-defined, discrete to confluent, erythematous scaly plaques arranged in transverse S-shaped lines on the right side of the trunk and as longitudinal lines on the genitals and right upper extremity [Figure 1]. Several tiny, erythematous, scaly plaques were scattered over the face, neck, and trunk. Auspitz's sign could be elicited in both nevoid as well as guttate lesions. Well-defined hyperpigmented, verrucous papules, and plaques were observed over the abdomen near umbilicus, right axilla, right elbow, and dorsum of right hand [Figure 1]. Scalp examination revealed plaques of silvery white adherent scaling. Mucosa and nail examination was normal. Routine blood investigations were within normal limits. The clinical differential diagnoses included linear or nevoid psoriasis with verrucous epidermal nevus, inflammatory linear verrucous epidermal nevus (ILVEN), and psoriasiform epidermal nevus.
Figure 1.
Typical psoriatic plaques along blaschko's lines (transverse S-shaped) over right side of the trunk with scattered guttate lesions (marked) over the trunk and neck and verrucous plaques over right axilla
Histopathological examination of biopsy specimen from erythematous scaly lesions showed psoriasiform epidermal hyperplasia, a diminished granular layer, and thick laminated horny layer with mounds of layered parakeratosis with neutrophils, which were consistent with psoriasis vulgaris [Figure 2]. Specimen from verrucous lesions showed epidermal hyperplasia with thickened granular layer and epidermolysis in the upper/mid spinous zone. The features were suggestive of epidermolytic hyperkeratosis [Figure 3].
Figure 2.
Histopathological features of biopsy from erythematous plaques showing psoriasiform epidermal hyperplasia, a diminished granular layer, and thick laminated horny layer with mounds of layered parakeratosis (hematoxylin and eosin; ×40)
Figure 3.
Histopathological features of biopsy from verrucous plaques showing epidermal hyperplasia with thickened granular layer and epidermolysis in the upper/mid spinous zone (hematoxylin and eosin; ×40)
The patient was put on weekly 7.5 mg methotrexate and was advised to apply clobetasol with calcipotriol ointment twice daily. The psoriatic lesions started healing after 1 month with subsidence of guttate lesions and there was a decrease in erythema and scaling over larger plaques [Figure 4]. After 2 months of follow up, the larger plaques also healed with post-inflammatory hypopigmentation. However, verrucous lesions remained unaffected [Figure 5a and b].
Figure 4.
Treatment response after 1 month shows significant improvement in psoriasis with subsidence of guttate lesions, with verrucous lesions persistent over right axilla and right upper side of umbilicus
Figure 5.
Treatment response after 2 months shows psoriasiform lesions subsided with hypopigmentation, while verrucous lesions are unaffected. (a) Anterior view; (b) lateral view
Psoriasis is a common genetically determined disease with several atypical forms. Nevoid form of psoriasis is a rare entity. Considerable diagnostic confusion exists and the very existence of linear or nevoid psoriasis has been questioned.[1,2] The main differential diagnosis of nevoid psoriasis is inflammatory linear verrucous epidermal nevus (ILVEN), which differs by early age of onset, female preponderance, predisposition to left lower extremity, pruritus, distinct inflammatory appearance, and refractoriness to treatment.[3,4] Nevoid psoriasis might coexist with or develop as an isomorphic phenomenon over a pre-existing ILVEN.[2] Some authors feel that linear psoriasis is merely an epidermal nevus with psoriasiform features whereas others feel that ILVEN is nothing but a mosaic form of psoriasis. Segmental manifestation of psoriasis superimposed on a symmetrical involvement has also been documented and loss of heterozygosity has been proposed as the underlying cause.[4]
In our patient, the verrucous plaques were present since birth, which although confined to the right side of the body, did not form any specific pattern. Psoriasiform lesions developed in continuity with these lesions along the Blaschko's lines since 6 months making them appear as part of the same entity. The absence of pruritus, presence of scalp scaling, and the association of acute pharyngitis with guttate lesions favored psoriasis over ILVEN. The lesions also differ on the basis of their histopathological features and treatment response. We believe that they represent two distinct entities which were coexistent in a patient with tendency toward mosaicism.
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References
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