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They further suggested that CTSC protects degradation of serine proteinases only in mature immune cell subsets and not in progenitor immune cells. Loss of activity of cathepsin C has also been linked with lack of immunomodulatory and microbial function of LL-37 in infected periodontum [16]. In one of the study investigators desired to understand if proinflammatory cytokines levels are elevated in blood samples of PLS patients [17]. However, they could not find any statistically significant rise in the level of cytokines. Apart from the role of gene mutation and deregulation of several immunologic factors in the cause and development of PLS, low level of anti-inflammatory fatty acids has also been reported in the patients with PLS [18]. It is observed that various periodontologic pathogens are known to proliferate in PLS patients. Microbial flora in subgingival plaque is very complex. Glafenine Albandar conducted a detailed study of PLS microbiota and detected 12 bacterial species [19]. Robertson et al., found periodontologic pathogens like A. actinomycetemcomitans, Porphyromonas gingivalis, and Prevotella intermedia in the subgingival plaque of PLS patients [20]. Presence of A. actinomycetumcomitans in the periodontal pockets is known to act as triggering factor for PLS development [14]. In large number of cases of PLS, it has been described in the scientific literature that consanguinity is a potential risk factor in the development of PLS. Recently, a paper by Shah et al., described two cases of PLS who were born of consanguineously married parents [21]. Another paper by Valeshabad et al., described six cases of PLS in the same family [22]. Palmoplantar hyperkeratosis was detected in all of the cases, and two cases had painful lesions on the soles. The lesions and abscesses in the internal organs are frequently associated with PLS. For the first time, Kanthimathinathan et al reported presence of brain abscess in a child with PLS [23]. Presence of liver abscess has also been reported in one of the two siblings who were born of consanguineously married parents [24]. Morgan et al., described a case of 5-year-old patient of PLS who developed a renal mass [25]. Therefore, it may be concluded that PLS is often present with lesions in different internal body parts and the complications of PLS are frequently observed in first generation of consanguineously married couples. Provisional diagnoses of aggressive periodontitis type 1 and palmoplantar hyperkeratosis and differential diagnoses of Haim-Munk syndrome and hypophosphatasia were initially considered for the patient. We confirmed the diagnosis as PLS on the basis of fulfilling classical description of the syndrome. The familial history of the patient was consistent with consanguinity. On physical examination, the patient showed characteristic diffuse skin lesions in the knees, dorsum of hands and feet.