Farber disease overlapping with stiff skin syndrome: Expanding the spectrum

Background: Farber Disease (MIM 228000)1 is a rare AR disorder fi rst described by Sidney Farber in 19522. Farber disease is usually recognized by the presence of three symptoms: Painful and progressively deformed joints, nodules under the skin and progressive hoarseness. Other organ systems may also be involved. As with most lysosomal storage diseases, the course of Farber’s Disease is progressive and death typically occurs in infancy. Stiff skin syndrome (SSS) (MIM %184900)1 was fi rst described by Esterly and McKusick as a disorder characterized by thickened and indurated skin of the entire body and limitation of joint mobility with fl exion contractures.Aim of the Study: Diagnosis and clarifi cation of overlapping in the clinicalpresentation of the studied case.Patients and Methods: Clinical report of an atypically presenting Farber case and analyzing the overlapping manifestations between the two syndromes.Results: Histopathological study was the conclusive diagnostic key in ourcase. Conclusion: Recognition of atypical or abortive cases is of practical importance as it may affect counseling or therapeutic decision making. Orodental manifestations were not previously considered but they may be of future diagnostic help.Keywords: Farber, stiff skin, lipogranulomatosis

Crude Coal Tar and Ultraviolet (UV) A radiation (Modified Goeckerman Technique) in Treatment of Psoriasis

Background: Psoriasis is a chronic inflammatory dermatosis that has a substantial impact on the quality of life. Goeckerman\u27s technique (GT) has been recruited for the treatment of psoriasis with high clearance rates and long periods of remission.Psoriasis is a chronic inflammatory dermatosis that has a substantial impact on the quality of life. Goeckerman’s technique (GT) has been implemented for the treatment of psoriasis with high clearance rates and long periods of remission. The objective of this article was to evaluate the efficacy and safety of modified GT (crude coal tar 2.5% plus UVA) as an alternative therapeutic modality for psoriatic patients with skin types III-V. Twenty two patients with moderate, severe, and erythrodermic psoriasis were included in this study. All patients received modified GT (crude coal tar 2.5% plus UVA) six days per week for a period of 3 months. Assessment of the rate of reduction of psoriasis area severity index (PASI) was performed, as well as photographic documentation of each patient at baseline and after completion of therapy. There was a significant reduction in PASI scores after therapy in all patients (P=0.001). The rate of PASI reduction after therapy was &gt;50% in 63.6% of patients; 27.3% of patients achieved &gt;75% reduction and 9.1% of patients achieved 26-50% reduction. No serious side effects were reported in any of the patients. Modified GT is a safe and effective therapeutic option for patients with moderate and severe psoriasis.</p

Impetigo herpetiformis during the puerperium triggered by secondary hypoparathyroidism: a case report

A 38-year-old multiparous woman with post thyroidectomy hypoparathyroidism developed pruritic erythematous patches with multiple pustules on its margins on her thighs and groin accompanied by fever few days after delivery by caesarean section. Impetigo herpetiformis was diagnosed based on the typical clinicopathological findings. The patient was treated with intravenous fluids, calcium, Calcitrol and corticosteroids. The correction of hypocalcaemia was accompanied with rapid improvement of her skin disease and general condition. Our case is the fourth case of impetigo herpetiformis initially presented during puerperium and the first case of puerperal impetigo herpetiformis that is precipitated by secondary hypoparathyroidism. The awareness of the possible occurrence of impetigo herpetiformis during the puerperium allows early diagnosis, treatment and prevention of maternal complications

Childhood Granulomatous Periorificial Dermatitis

Childhood granulomatous periorificial dermatitis (CGPD), also known as facial Afro-Caribbean childhood eruption (FACE), is a distinctive granulomatous form of perioral dermatitis. It is a condition of unknown etiology, characterized by monomorphous, small, papular eruptions around the mouth, nose and eyes that histopathologically show a granulomatous pattern. It affects prepubescent children of both sexes and typically persists for several months but resolved without scarring. We report a 9 year-old girl with multiple, discrete, monomorphic, papular eruptions of 2-months duration on the perioral and periocular areas. Histopathological examination demonstrated upper dermal and perifollicular granulomatous infiltrate