A Case of Ichthyosis vulgaris IV鱼鳞癣

We present a 9-year-old girl, who no records of atopy,  with persistent asymptomatic discoloured scales of the lower limbs and body for 5 years. On physical examination, the skin was xerotic with gross hyperpigmentation and light grey scales on her trunk and limbs. The surface of the lower limbs were involved predominantly, with sparing of the flexural creases (Figures 1-A).

Ichthyosis3

The light grey scales with fine scaling developed at age 4 and reduced during the summer, lesion were found mainly on the extensor surfaces of her extremities and trunk. A biopsy was performed at a local hospital, and she was diagnosed with Ichthyosis vulgaris IV. The patient was prescribed with 5% glycerine in aqueous cream and 5% salicylic acid cream thereafter. However the grey scales persisted, fine scales are minimized with moisturizer applicaiton. Patient reported no itch but she could not perspire.

Pateint was referred to our TCM clinic and was subsequently prescribed with herbal oral medication two times every day. She was followed up by phone calls and WhatsApp’s messages on a monthly basis. At the five-month review, the patient acknowledged improvement in the showing moisture retention and a slight decrease in pigmentation in the affected areas. Heral oral medicine is still continued with the same dosage, additionally herbal yufu topical cream was incorporated into the procedure, in the 4 years review post-procedure, the lower limbs was noted to have significant improvement (Figure 1-B), skin on trunk and limb area has returned to the natural consistency and colour tone. Patient reported perspiration returned to norm.

IV overview

The ichthyoses, also recognized as the disorders of keratinization or disorders of cornification, are characterized by a generalized scaling of the skin to severities of varying hierarchies. Majority of ichthyoses are inherited, but acquired forms can develop in the setting of malignancy, autoimmune or infectious disease, and nutritional deficiency. A considerable number of patients indicate that they suffered from hypohidrosis and cannot perspire well [1]. IV is associated with an increased risk for atopy, including asthma, allergies, and atopic dermatitis [2,3].

IV is a rare, without any standard treatment procedures currently. On the flip side, IV patients benefit from ointments that hydrate the stratum corneum [4], and excellent experience, e.g. with creams containing glycerol [5], has been reported. We report a IV case successfully managed with herbal oral medication and herbal yufu cream.

 

Reference

  1. Oji V, Seller N, Sandilands A, et al. Ichthyosis vulgaris: novel FLG mutations in the German population and high presence of CD1a+ cells in the epidermis of the atopic subgroup. Br J Dermatol 2009;160:771–81.
  2. Sandilands A, Terron-Kwiatkowski A, Hull PR, et al. Comprehensive analysis of the gene encoding filaggrin uncovers prevalent and rare mutations in ichthyosis vulgaris and atopic eczema. Nat Genet 2007; 39:650.
  3. Osawa R, Akiyama M, Shimizu H. Filaggrin gene defects and the risk of developing allergic disorders. Allergol Int 2011; 60:1.
  4. Oji V, Traupe H. Ichthyosis: clinical manifestations and practical treatment options. Am J Clin Dermatol 2009;10:351–64.
  5. Blanchet‐Bardon C, Tadini G, Machado Matos M, Delarue A. Association of glycerol and paraffin in the treatment of ichthyosis in children: an international, multicentric, randomized, controlled, double‐blind study. J Eur Acad Dermatol Venereol2012;26:1014–19.