Cutaneous lymphadenoma in a young Saudi female: a case report.

Background: Cutaneous lymphadenoma (CL) is a rare benign epithelial neoplasm. It presents as skin-colored nodule in the head and neck area. CL is an uncommon condition with less than 60 cases reported in the literature and very rare occurrence in the Asian population; it has never been previously reported in the Arabian gulf countries. We report the first case of CL in a young Saudi female. Case Description: A 28-year-old Saudi female presented with an asymptomatic skin lesion on her forehead that first appeared eight years ago. Physical examination showed a 3 mm × 3.4 mm solitary, nontender, firm, skin-colored to pink nodule with overlying telangiectasia. A biopsy was taken, and histopathology findings revealed epithelial nests of uniform, small basaloid cells with some peripheral palisading; a fibrotic stroma; and Lympho-histiocytic inflammatory infiltrates. No retraction artifacts, atypia or necrosis were observed. These findings are consistent with CL. The lesion was fully excised by skin punch biopsy without any residual tumor. No recurrence was observed after 1 year follow-up. Conclusions: CL presents a diagnostic pitfall, and many dermatologists fail to recognize it in the initial clinical impression. Moreover, although CL is a rare and benign tumor, it must be in the differential diagnosis of any asymptomatic skin nodule in the head and neck area.

ARL6IP6, a susceptibility locus for ischemic stroke, is mutated in a patient with syndromic Cutis Marmorata Telangiectatica Congenita.

Cutis Marmorata Telangiectatica Congenita (CMTC) is a congenital localized or generalized vascular anomaly, usually sporadic in occurrence. It can be associated with other cutaneous or systemic manifestations. About 300 cases have been reported. The molecular etiology remains largely unknown. The main purpose of this study is to delineate the molecular basis for a syndromic CMTC phenotype in a consanguineous Saudi family. Clinical phenotyping including detailed neurological imaging, followed by autozygosity mapping and trio whole exome sequencing (WES) are also studied. We have identified a homozygous truncating mutation in ARL6IP6 as the likely cause of a syndromic form of CMTC associated with major dysmorphism, developmental delay, transient ischemic attacks and cerebral vascular malformations. This gene was previously implicated by genome wide association study (GWAS) as a susceptibility locus to ischemic stroke in young adults. We identify ARL6IP6 as a novel candidate gene for a syndromic form of CMTC. This suggests that ischemic stroke or transient ischemic attacks (TIA) may represent, at least in some cases, the mild end of a phenotypic spectrum that has at its severe end autosomal recessive CMTC. This finding contributes to a growing appreciation of the continuum of Mendelian and common complex diseases.

Primary systemic amyloidosis presenting as extensive cutaneous ulceration.

BACKGROUND: We report a case of primary systemic amyloidosis in a 78-year-old Caucasian woman presented as a nonhealing ulcer on the right thigh for 3 months. Histopathology of the skin revealed widely thickened walls of middermal and subcutaneous vessels from deposition of amorphous eosinophilic material that stained positively with Congo red and crystal violet. OBJECTIVE: This case represents a very unusual presentation of primary systemic amyloidosis, one in which the cutaneous manifestations provided the first signs of a devastatingly widespread multiorgan infiltration of amyloid protein. CONCLUSION: This presentation of the disease may signify an advanced stage with a grave prognosis as our patient passed away 3 months after development of the cutaneous ulceration.

Effect of graft size, angle, and intergraft distance on dense packing in hair transplant.

BACKGROUND: The maximum number of hair grafts that can be safely implanted in 1 cm2 is still debatable. To our knowledge, no previous report has addressed this issue in three dimensions, taking into account the size, the angle of the graft, and the intergraft distance. OBJECTIVES: To study the effect of the size and angle of the graft and the intergraft distance on dense packing. METHODS: Using a mathematical formula (the maximum number of hair grafts in 1 cm2 = 33 * cosine), the volume of the recipient area and the volume of the hair graft are calculated, assuming that the surface area of the recipient area is 1 cm2, the diameter of the hair graft is 1 mm, and the intergraft distance is 1.5 mm laterally and 1 mm anteriorly and posteriorly. RESULTS: The maximum number of hair grafts that could be implanted in 1 cm2 at a 90 angle in relation to the skin surface is 33 grafts, at a 60 angle is 28 grafts, and at a 30 angle is 16 grafts. CONCLUSION: The maximum number of hair grafts that can be implanted in any given recipient area depends on the graft size, the angle or direction of these grafts, and the intergraft distance. Where more space is allowed between the grafts, and the more acute the angle, the fewer hair grafts that can be implanted.