Anonychia congenita in different generations of a single Saudi family.

Anonychia refers to the absence of nail plates owing to an autosomal dominant or recessive inheritance. Congenital anonychia is a rare condition that may be associated with other ectodermal or mesodermal malformations like epidermolysis bullosa, (deafness, onychodystrophy, osteodystrophy, and mental retardation) syndrome and Iso-Kikuchi syndrome. Here, we report 3 cases with anonychia congenita appearing in different generations of a single family in Kingdom of Saudi Arabia.

Herpes zoster in eastern Saudi Arabia: clinical presentation and management.

BACKGROUND: Herpes zoster (HZ), caused by varicella zoster virus (VZV), initially produces chicken-pox, then the virus lies dormant in the dorsal root ganglia. The virus can reactivate after many years and results in HZ along ganglion's distribution. Old age, trauma, stress, diabetes mellitus, and immune suppression are important risk factors for the reactivation. Herpes zoster is characterized by unilateral radicular pain and vesicular eruption that is generally limited to the dermatome innervated by the affected ganglion. In immunocompromised individuals, disseminated zoster may develop. The aims of therapy in HZ are to control pain or reduce its severity by the use of analgesics, reduce the duration and eruption of new lesions, and prevent complications, particularly postherpetic neuralgia (PHN) by appropriate antiviral therapy. METHODS: All cases of HZ seen in the dermatology clinic at King Fahd Hospital of the University (KFHU) from 1988 to 2006 were included in the study. Their diagnoses were based on the clinical presentation. The following parameters were collected and analyzed: age, sex, nationality, symptoms, dermatomal distribution, complications, coexisting diseases, and disease management. RESULTS: Of 22 749 new cases seen in the dermatology clinic over 18 years, 141 were HZ, with an occurrence of 0.62%. Male to female ratio was 2:1 and the age ranged from 14 months to 80 years. The thoracic dermatomes were the most commonly involved. The most frequent coexisting disease was diabetes mellitus, and the most common complication of HZ was PHN. Most patients with HZ ophthalmicus developed eye complications. CONCLUSION: The occurrence of HZ is 0.62% in patients reporting to the dermatology clinic of the hospital. Males are little more affected than females. The thoracic dermatomes are the most frequently involved. Diabetes mellitus is the most frequent coexisting disease. Postherpetic neuralgia is the most common complication of HZ.

Multiple side effects of Efalizumab in a Saudi female with chronic persistent psoriasis followed by severe rebound after Efalizumab discontinuation.

We are reporting a case of 23-year-old Saudi female with persistent chronic plaque psoriasis who was given subcutaneous Efalizumab 0.8 mg/kg/week for 14 weeks. During that period the patient developed multiple adverse reactions followed by severe rebound. The case is presented to highlight the importance of managing patients on Efalizumab carefully and closely.

Malignant peripheral nerve sheath tumor of the skin: case report.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors derived from Schwann cells or pluripotent cells of the neural crest. In this report, we describe a 21-year-old Saudi woman who presented with an asymptomatic, solid, round, protruding tumor on the right upper back which was diagnosed as an MPNST with no stigmata of neurofibromatosis. It was completely excised with a wide surgical margin. The patient was followed up for 9 months with no evidence of recurrence.

Poiklodermatous variant of mycosis fungoides.

Mycosis Fungoides (MF) is a T-cell lymphoma initially presenting in the skin. Many clinical variants exist including follicular, vesicular, poikilodermic, hypo- and hyperpigmented, ichthyosiform and pigmented purpura-like lesions. We present an unusual variant in a case of poikilodermic MF.

Vulvar basal cell carcinoma misdiagnosed for 4 years.

Vulvar basal cell carcinoma is a rare cutaneous neoplasm occurring mainly in white postmenopausal females. It can be misdiagnosed due to its nonspecific physical appearance. Here, we report a 59-year-old white female who had vulvar basal cell carcinoma misdiagnosed for 4 years.

Childhood solitary collagenoma.

Familial cutaneous collagenoma is an inherited connective tissue nevus, which presents with asymptomatic symmetrically distributed skin nodules on the trunk or upper limbs. Here, we describe a case of a 12-year-old girl with collagenoma affecting the lower back.

Cutaneous hyperpigmentation following nonpermanent henna tattoo.

Decorative henna skin painting can provoke severe reactions in the skin. We report 3 cases of unusual and persistent hyperpigmentation following nonpermanent henna tattoo, which alerted us to identify an additional side effect.

Undifferentiated connective tissue disease with various pigmentary patterns: a study of three patients.

Undifferentiated connective tissue disease (UCTD) is a condition characterized by the presence of clinical manifestations suggestive of a connective tissue disease and at least one non-organ specific autoantibody. In this report I am presenting three types of cutaneous pigmentary changes are presented in three patients which were the clue to the diagnosis of UCTD.

Impetigo herpetiformis in a primigravida: successful treatment with etretinate.

Impetigo herpetiformis (IH) is a rare dermatosis which usually occurs during the third trimester of pregnancy. It is characterized by acute erythematosquamous plaques covered with tiny superficial pustules in a herpetiform distribution with less likely mucus membranes involvement. It can be associated with constitutional symptom s such as fever, chills, nausea, vomiting and diarrhea. Impetigo herpetiformis can cause serious complications to the mother and fetus which include: maternal hypocalcemia leading to delirium, convulsions, and tetany in the mother, and placental insufficiency leading to still birth, neonatal death or fetal abnormalities. Lesions are expected to disappear after birth but may recur during subsequent pregnancies at an earlier gestational age. Presented here is a case of IH occurring during the 37th week of gestation in a primigravida who failed to respond to oral steroid but successfully cleared with oral etretinate.

Solitary mastocytoma successfully treated with a moderate potency topical steroid.

We report a case of solitary mastocytoma in a child which was successfully treated with a topical steroid of moderate potency. The patient was an 18-month-old girl who presented with localized oval shape yellowish to hyperpigmented lesion on the medial aspect of her right forearm noticed accidentally by the parents since the age of 6 months. The lesion was observed to get urticated mainly after bathing, toweling, and scratching of the area, associated with reddening and itching confined to the lesion (Figure 1). No other area of the body was affected with any similar lesion. Examination of the skin revealed a yellow-tan oval shape patch 1 x 3 cm in diameter which was firm to the touch with intact overlying skin. The lesion became swollen and itchy when it was rubbed vigorously (positive Darier's sign). Systemic examination was unremarkable. The patient investigations including complete blood count, routine biochemical data, plasma histamine level, and urinalysis were within normal levels. Skin biopsy was cancelled because the parents refused, so our clinical diagnosis was solitary mastocytoma even though it was not confirmed histologically. We started the patient on a moderate potency corticosteroid (betamethasone valerate 0.1% cream) twice a day for six weeks after which the lesion became softer with a weak Darier's sign. This treatment was continued for another four months which led to resolution of the lesion with residual hyperpigmentation, negative Darier's sign, and no signs of atrophy (Figure 2). Follow up of the patient for another 8 weeks without treatment did not reveal any recurrence of the lesion.

Subungual epidermoid inclusions.

Subungual epidermoid inclusions are protrusions of the nail bed epithelium into the upper most bed stroma. They may be associated with hyperplasia of the bed epithelium resulting in subungual keratosis, onycholysis or even clubbing. An 11-year-old girl presented to our dermatology clinic with a one year history of progressive increase in the size of the distal part of her left thumb associated with mild pain on pressure. The diagnosis became evident perioperatively, which showed multiple subungual epidermoid inclusions.

Erythrokeratoderma variabilis.

Erythrokeratoderma variabilis is a rare autosomal dominant genodermatosis of variable expressivity. In this report, we describe the clinical features and microscopic findings in one of our patients born to unaffected parents. We also briefly review the literature on this disorder.

Naxos disease in an Arab family is not caused by the Pk2157del2 mutation. Evidence for exclusion of the plakoglobin gene.

OBJECTIVE: Naxos disease is a rare hereditary disorder characterized by palmoplantar keratoderma, woolly hair and cardiomyopathy. This study aims to determine whether Naxos disease in a Saudi Arab family is caused by the Pk2157del2 mutation that was identified in Greek families from Naxos Island where the disease had originally been described. METHODS: This study was undertaken at King Fahad Hospital of the University, Al-Khobar, and the Medical University of Hannover, in the spring of 2003. Naxos disease has been encountered in a 2-year-old girl and her 30-year-old aunt of a Saudi Arab family. Deoxyribonucleic acid samples of this family were analyzed by polymerase chain-reaction (PCR) amplification of the respective region of the plakoglobin gene, and direct nucleotide sequencing of the PCR-products. Segregation analysis was performed employing the newly detected IVS11+22G/A polymorphism. RESULTS: Molecular genetic analysis of the DNA sample of the child diagnosed with Naxos disease showed absence of the Pk2157del2 mutation. In addition, the segregation analysis revealed heterozygosity for IVS11+22G/A in the affected girl. CONCLUSION: Absence of the Pk2157del2 frameshift in the affected child proved that Naxos disease in this Saudi Arab family is not caused by the same mutation that was identified in the Greek families. Furthermore, heterozygosity for the IVS11+22G/A polymorphism provided evidence for exclusion of the plakoglobin gene in this consanguineous family.