cutis laxa is an acquired or inherited condition characterized by redundant, sagging and inelastic skin.  the inherited form is heterogeneous condition with autosomal dominant, autosomal recessive and x-linked inheritance.  autosomal dominant cutis laxa is divided into three types, type i, ii and iii and the responsible genes are eln, fbln5 and aldh18a1 respectively. an x-linked form of cutis l...

Amyloidosis cutis dyschromica is a rare type of primary cutaneous amyloidosis characterized by reticulate hyper-pigmentation with discrete hypopigmented macules. Up to date, about 50 cases of amyloidosis cutis dyschromica have been reported and the majority are familial cases of Asian ethnicity. Various diseases, particularly autoimmune diseases such as systemic sclerosis and systemic lupus ery...

Ehlers-Danlos syndrome (EDS) is a heterogenous group of inherited disorders of connective tissue characterized by fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility. Cutis laxa is characteized clinically by lax, pendulous skin and histologically by loss of elastic tissue in the dermis. There are some reports of coexistence of cutis laxa with ot...

Pachydermoperiostosis (PDP) is a rare hereditary disease characterized by folded coarse skin, hyperostosis, clubbing of fingers and abnormalities in other organs such as gastrointestinal tract. Because of safety parents guides us to familial incidence and penetration of gene in 50% of cases. Diagnosis is based on clinical and radiological findings. Males are more susceptible than females to thi...

the authors describe two interesting and seldom cases of atrophia gyrata j and believe it to be a congenital hereditary disease with a recessive character. like retinitis pigmentosa, it does not affect the central posterior parts of the retina but the lesions are around the periphery. the visual fields are narrowed. they discuss; the similarity of this disease with atrophia gyrata described by ...

Plasma cell leukemia is an uncommon, aggressive variant of leukemia that may occur de novo or in association with multiple myeloma. Leukemia cutis is the cutaneous manifestation of leukemia, and indicates an infiltration of the skin by malignant leukocytes or their precursors. Plasma cell leukemia cutis is a rare clinical presentation of leukemia. We present a man who developed plasma cell leuk...

Aleukemic leukemia cutis is a rare condition characterized by the invasion of leukemic blasts into the skin before their appearance in the peripheral blood. Leukemia cutis usually occurs in patients with myeloid leukemia, especially the myelomonocytic and monocytic types of acute myeloblastic leukemia. We describe the case of a 62-year-old woman with aleukemic leukemia cutis who developed Phila...

Admas-Oliver syndrome is a rare autosomal dominant disease of congenital development defects association with aplasia cutis. All of 81 reported cases were of 32 group families. These two cases had congenital scalp cutis aplasia with developmental defect and hypoplasia of left foot digits in case 1.Congenital deaf-mute in both can added to the past known findings in this syndrome. Thoracic defor...

A 1-day-old boy with the characteristics of Adams-Oliver syndrome was presented. Adams-Oliver syndrome has a wide spectrum of anomalies ranging from aplasia cutis congenita, cutis marmorata telangiectatica congenita and transverse limb defects to lethal anomalies. Our patient had aplasia cutis congenita with scalp, skull and dura defect. He had also a large dura defect with herniation of brain ...