INTRODUCTION: Lamellar ichthyosis is a rare autosomal recessive disorder apparent at birth. Although not life threatening, it is disfiguring and causes considerable psychological stress.
CASE: We report a case of 15-year-old male with generalized, thick, dry, plate-like scales associated with ectropion and eclabium. At birth, he was enclosed in a transparent membrane that spontaneously shed revealing generalized pinkish skin. Non-pruritic plaques developed that evolved into plate-like scales. There was accompanying heat intolerance and tearing of eyes. Two siblings were similarly affected. Histopathologic findings were consistent with lamellar ichthyosis. The patient was treated with retinoic acid lotion and petroleum jelly. After three weeks, 50 percent improvement was noted with decrease in number and thickness of lesions.
CONCLUSION: This lifetime cutaneous disease affects the physical, mental and emotional state ofthe patient. It has no cure and treatment is directed symptomatically. We plan to continue topical treatment in this patient until significant improvement is attained.

Human ; Male ; Adolescent ; Administration, Topical ; Ectropion ; Emotions ; Eye ; Hot Temperature ; Ichthyosis, Lamellar ; Petrolatum ; Physical Examination ; Stress, Psychological ; Tretinoin

: Pachydermoperiostosis (PDP) or primary hypertrophic osteoarthropathy (PHO) is a rare genetic disorder that affects mainly skin and bones. Its main clinical features are pachyderma (thickening of the skin), periostosis ~excessiv~ bone formation) and clubbing of fingernails. The d1sea~e is more common among males with a 7:1 ratio, starts during adolescence and stabilize and cease progression after 5 - 20 years. OBJECTIVE: To discuss the summary of the case, new management options, and outcomes of the management options. CASE SUMMARY (METHODS): A 28-year-old male presents with a 9-year history of wrinkling of his facial skin. Other associated symptoms were seborrhea, acne, clubbed fingers, and occasional minimal knee joint pain. Histopathology results showed thickened dermis, fibrosis of the papillary dermis and around the folliculosebaceous units, hyalinized collagen bundles involving the fibrous trabeculae of the subcutis, and prominence of sebaceous and eccrine glands. The patient was given oral isotretinoin at 0.5 mg/kg/day, underwent fractional CO~ laser f?r rhytides and large pores, and given botuhnum toxin A injection (total of 16 U) on 5 sites at the glabellar region. The patient was referred to plastic surgery for frontal rhytidectomy, and orthopedic surgery for management of joint pains. RESULTS: The patient noted 80% improvement from baseline. CONCLUSION Treatment of pachydermoperiostosis is mainly symptomatic and requires a multi-spec~alty approach. Because of its rarity, treatment options for pachydermoperiostosis have yet to_ be standardized. In this particular case, all available options in the institution were utilized which led to satisfaction of the patient of the outcome.

Introduction: Nevus lipomatosus superficialis is a rare benign hamartomatous skin lesion. It is classified into 2 types: solitary and multiple. Lesions consist of multiple, flesh-colored or yellow papules and/or nodules in a segmental pattern, with a linear, zosteriform, or along the lines of skin folds distribution. Surfaces are smooth, but may also have verrucous or cerebri-form appearance. Pathogenesis is unknown, but there is speculation that precursor cells around dermal blood vessels give rise to mature fat cells in a mosaic pattern. Incidence is presently unknown, but this is the first reported case in this institution. Histopathology reveals presence of aggregates of mature adipose tissue among the collagen bundles of the dermis. Case Summary: DF is a 14-year-old, Filipino, female, who consulted due to multiple, pedun-culated, soft papules over the right lower back, and extending to the right flank, with a clustered arrangement. There was no history of trauma or manipulation, nor was there pain, pruri-tus, or other associated symptoms. Past medical, family, and social history were non-contributory. Initial impression was acrochordon. Excision biopsy revealed mature adipose tissue in the dermis, which is diagnostic for nevus lipomatosus superficialis. Serial excision was done. Conclusion Excision is the treatment of choice. Other treatment options that can be ex-ploredare COz laser, cryotherapy, and intralesional injection of phosphatidylcholine, which yield promising results recommended by other studies. Dermatopathology plays a vital role in the diagnosis of this condition. A high index of suspicion, a good clinical eye, and dermatopa-thologic analysis are essential tools in clinching the diagnosis.
Nevus ; Adipose Tissue