Relapses and Remissions of Netherton Syndrome in a Preadolescent Child- Letter to Editor

Dear Editor, A 9-year-old male child presented to the dermatology outpatient department with intensely pruritic dermatosis, along with sparse eyebrow hair. The mother reported that he developed scalp lesions at birth and subsequently disseminated intermittent eczematous plaques. Physical examination revealed erythematous and squamous plaques with double-edged desquamation showing polycyclic and serpiginous borders on the abdomen and left thigh [Figures 1,2]. Also, a lack of the external portion of the eyebrows was noted with few short and brittle hair predominantly in the occipital region [Figure 3]. On dermoscopy, polarised mode, 10x magnification of serpiginous borders of the abdominal plaque, incomplete double edged scales were evident [Figure 4]. Trichoscopy of the eyebrows showed bamboo hairs, golf tee and matchstick hairs; trichorrhexis invaginata was observed predominantly in the occipital region [Figure 5]. Laboratory findings were remarkable for peripheral eosinophilia and increased immunoglobulin E levels. The patient was given topical mild-potency corticosteroids, oral antihistamines.Figure 1: On clinical examination multiple red, scaly, annular to polycyclic plaques with incomplete advancing double edged scale were seen on the left thighFigure 2: On clinical examination multiple red, scaly, annular to polycyclic plaques with incomplete advancing double edged scale were seen on the abdomenFigure 3: On clinical examination of the face, sparse lateral one thirds of bilateral eyebrows were seenFigure 4: On dermoscopy, polarised mode, 10x magnification incomplete double edged scales were evident.Figure 5: On Trichoscopy of eyebrow hair, polarised mode,10x magnification, findings of Trichorrehxis invaginata was notedWith an incidence of one instance per 200,000 births, Netherton syndrome (NS) is an uncommon and potentially fatal autosomal recessive genodermatosis.[1,2] One of the hallmarks of NS is icthyosis linearis circumflexa (ILC), first reported in 1949 and is characterized by itchy, polycyclic erythematous patches with a double-edged circinate or serpiginous scale.[3] The bamboo hair (trichorrhexis invaginata), a pathognomonic hair abnormality of NS that was first identified by Netherton in 1958, is another characteristic.[4] It consists of an invagination of the hair shaft that gives the hair a “ball in cup” appearance.[5] Finally, atopic diathesis and the first two observations fit the well-known trio that was originally identified.[6] NS is a complex condition that can be difficult to diagnose due to its similarities with other skin disorders, such as atopic dermatitis. Fortunately, trichoscopy has emerged as a valuable diagnostic tool in these cases. This noninvasive technique allows clinicians to examine hair and skin features in greater detail, facilitating a more accurate diagnosis. In our case, trichoscopy helped in diagnosing Netherton syndrome by the trichoscopic presence of trichorrhexis invaginata. Declaration of patient consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.