A CASE OF KERATOSIS LICHENOIDES CHRONICA (NEKAM’S DISEASE): Dermoscopic and in vivo reflectance confocal microscopy findings

Ayris ÖZTÜRK; Ayda ACAR; Banu YAMAN; Işıl TUNA

A CASE OF KERATOSIS LICHENOIDES CHRONICA (NEKAM’S DISEASE): Dermoscopic and in vivo reflectance confocal microscopy findings

Bildiriler > Tam Metin Bildiri · 2023

Yazarlar: Ayris ÖZTÜRK, Ayda ACAR, Banu YAMAN, Işıl TUNA

32nd Congress of the European Academy of Dermatology and Venereology, Almanya, Berlin, Uluslararası, Poster, Tam Metin Bildiri, 2023-2023, cilt -, ss ---

Özet

Introduction & Objectives:Keratosis lichenoides chronica (KLC) is a rare chronic inflammatory disorder of keratinization, also known as Nekam’s disease, characterized by asymptomatic hyperkeratotic papules in a linear or reticular pattern on the trunk and the extremities symmetrically. Histopathology shows features of lichenoid dermatitis. The course of the disease is chronic, progressive, and the treatment is challenging. Herein, we report a case of KLC with dermoscopy and reflectance confocal microscopy (RCM) findings.Materials & Methods:A 39-year-old man presented with 3 years of reticular lichenoid lesions on the trunk and extremities, along with erythroderma. He was taking 100 milligrams (mg) of azathioprine and 4 mg of prednisolone for idiopathic pulmonary fibrosis. There was no family history of similar eruption, nor any skin disorders diagnosed.Results:A physical examination revealed erythroderma and generalized pink-brown hyperkeratotic and infiltrated papules with reticular coalescence on the face, trunk, and extremities. Oral mucosa and nails were unaffected. In dermoscopy, globular Wickham Stria with dotted and linear vessels, diffuse brown-gray granules, and perifollicular granules and dots were seen.RCM demonstrated a regular honeycomb pattern on the epidermal level, dense inflammatory infiltration with dendritic cells, and single-cell necrosis on basal layers. Dermo-epidermal junction (DEJ) was irregular with dense melanophage infiltration. Bright stellate spots in the superficial dermis, consisting of inflammatory cells and plump-bright cells including melanophages were present (Figure 1).Histopathology revealed hypergranulosis, epidermal basal necrotic keratinocytes, and basal lymphoid infiltration. A dense band of mononuclear cell infiltration with some melanophages and dilated vessels on the superficial dermis was present (Figure 1).The patient was diagnosed with Nekam’s disease with clinicopathologic correlation. Systemic prednisolone at a dose of 0.5 mg/kg was combined with 10 mg acitretin. The prednisolone dose was gradually reduced, while acitretin therapy was increased to 35 mg daily. The patient had a dramatic response to acitretin therapy with no recurrence in two years.Conclusion:Dermoscopy and RCM are well-known in vivo techniques significant in the diagnosis of lesions without a histopathologic examination. Epidermis, DEJ, and upper dermal morphological features can be analyzed using RCM. RCM findings of KLC have not been described in the literature.*RCM findings of the patient were in correlation with histopathologic examination. Bright stellate spots in DEJ are correlated with dense inflammatory infiltration with dendritic cells. In some areas, single-cell necrosis was visible, which is typical of Civatte bodies in lichen. Irregular DEJ is correlated with interface dermatitis. The plump-bright cells on DEJ and superficial dermis are correlated with melanophages.This case report illustrates the dermoscopy and RCM features of KLC with histopathological correlation. RCM and dermoscopy features, combined with the clinical findings, may be helpful to the diagnosis of KLC.

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