D-penicillamine-induced pseudo-pseudoxanthoma elasticum and extensive elastosis perforans serpiginosa with excellent response to acitretin.

D-penicillamine (DPA)-induced pseudo-pseudoxanthoma elasticum (PXE) and elastosis perforans serpiginosa (EPS) has been reported in the past, but most of the treatment modalities used before have a sub.optimal response. We report a case of DPA-induced pseudo-PXE with extensive EPS who had an excellent rapid response to acitretin.

Rituximab/IVIG in pemphigus - a 10-year study with a long follow-up.

Background: Pemphigus is a chronic potentially life-threatening autoimmune blistering disease affecting the skin and/or mucous membranes. Rituximab is being increasingly used and found efficacious in the treatment of pemphigus.

Objective: To present the Middle-Eastern experience with the use of rituximab in pemphigus.

Pallor sign: an indicator of hemangioma in evolution.

Background: The typical presentation of infantile hemangioma is well known and is easily recognizable. However, it may have many atypical presentations, as reported in the literature. Most of the hemangiomas are not visible at birth and become apparent at about 3 to 4 weeks of age.

T-cell lymphoma/Sézary syndrome in a liver transplant recipient.

Background: Cutaneous T-cell lymphoma is a rare subtype of posttransplantation lymphoproliferative disease, with only 29 cases reported in the literature to date, the majority of which are post-renal transplantation. Only a few were reported after pancreas, heart, and bone marrow transplantation. There have been no reports of cutaneous T-cell lymphoma following liver transplantation to date.

Lupus erythematosus-associated primary and secondary anetoderma.

Background: Anetoderma (focal loss of dermal elastic tissue) can either be primary, which is an idiopathic occurrence of anetoderma in normal areas of the skin, or secondary, which is preceded by an inflammatory dermatosis in the same location.

Objective: Sporadic reports of lupus erythematosus-associated anetoderma have been described in the literature. All reported cases were positive for antiphospholipid antibodies.

Acquired cutis laxa type II (Marshall syndrome) in an 18-month-old child: a case report.

Cutis laxa is a rare disorder resulting from degradation and clumping of elastic fibers in dermis. Type II acquired cutis laxa, shows only cutaneous changes without any systemic involvement. We describe an infant with acquired cutis laxa type II following a generalized inflammatory dermatitis.

Skin-homing CD8+ T lymphocytes show preferential growth in vitro and suppress CD4+ T-cell proliferation in patients with early stages of cutaneous T-cell lymphoma.

A total of 27 T-lymphocyte cell strains were established from skin biopsies of 24 patients with various stages of cutaneous T-cell lymphoma (CTCL) by addition of the T-cell growth factors interleukin (IL)-2 and IL-4. Cellular proliferation and phenotypic changes were measured over 3 months in culture, and T-cell clones were studied using T-cell receptor-? re-arrangement techniques. An average outgrowth of 134 million T-lymphocytes from a 4-mm skin biopsy was observed over 2 months.

Phenotypic variation and allelic heterogeneity in young patients with Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome is an autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressive periodontitis. The aim of the study was to identify underlying cathepsin C mutations in 39 subjects with Papillon-Lefèvre syndrome and to explore any phenotypic associations. Genotyping and mutation analyses were performed using standard molecular techniques, and dermatological and oral characteristics were assessed with a semiquantitative clinical score.

Becker's melanosis: a report of 12 cases with atypical presentation.

Becker's melanosis is a distinct clinical entity. It usually presents in adolescence as a unilateral, hyperpigmented, hairy cutaneous hamartoma. We report herein 12 cases of Becker's melanosis that differ in their presentation from classical Becker's melanosis, and we believe that such presentations are not uncommon.

Phakomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome.

We describe a 23-month-old child with phakomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome. Phakomatosis pigmentovascularis type IIb is a rare cutaneous malformation characterized by the simultaneous occurrence of nevus flammeus and melanocytic nevi. A brief review of the literature is presented and classification of this rare disease is discussed.

Extensive Darier's disease with esophageal involvement.

Although the involvement of mucous membranes in Darier's disease is relatively uncommon, Darier's disease has been associated with mucosal lesions, especially in the oral mucosa. In the English literature there is only one report describing the involvement of esophageal mucous membranes in a patient with Darier's disease. Herein, we report the second case of Darier's disease with esophageal involvement.

Lichenoid drug eruption probably associated with rofecoxib.

Objective: To report a case of lichenoid drug eruption (LDE) probably induced by rofecoxib.

Case Summary: A 73-year-old woman was prescribed rofecoxib 25 mg/day for rheumatoid arthritis in addition to other medications on which the patient had been stabilized. Six months after initiation of rofecoxib, linear plaques over the infra-orbital and bitemporal areas of both eyes were observed.

Papular-purpuric "gloves and socks" syndrome in a mother and daughter.

The papular-purpuric "gloves and socks" syndrome (PPGSS) is a unique exanthem characterized by petechiae with painful edema of the hands and feet extending proximally with less severity. Constitutional symptoms of fever, lethargy, and arthralgia have also been described. Human parvovirus B19 has been implicated in most cases as the causative agent.

Thalidomide for treatment of severe generalized discoid lupus lesions in two patients with systemic lupus erythematosus.

We describe 2 patients with systemic lupus erythematous whose widespread discoid lupus erythematosus was unresponsive to systemic steroids and antimalarial agents. They showed dramatic improvement to thalidomide at a dose of 300 mg/d, with maximum benefit achieved within 15 weeks of therapy. Dosages of 50 to 100 mg/d were effective in maintaining remission for 1 year.

Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome is an autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early development of aggressive periodontal infection. The aims of this study were to rank the severity of dermatologic and oral affections using a semiquantitative scoring system, and to evaluate whether the severity of the dermatologic changes were correlated to age, degree of periodontal infection, or both. The study included 47 patients with Papillon-Lefèvre syndrome.

Ichthyosis follicularis: a case report and review of the literature.

Ichthyosis follicularis (IF) is a very rare neurocutaneous, X-linked recessive condition affecting the skin, hair, eyes, and central nervous system (CNS). This report describes a child with facial dysmorphism, mental retardation, psychomotor delay, congenital alopecia of the scalp, eyebrows, and eyelashes, and extensive spiny follicular papules. A skin biopsy specimen showed the characteristic absence of sebaceous glands.

Two brothers with keratosis follicularis spinulosa decalvans.

Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder affecting both the skin and eyes. There are few reports about this entity. The aim of this report is to describe 2 brothers with progressive scarring alopecia of the scalp, hypotrichosis with follicular prominence of the eyelashes, and extensive keratosis pilaris.

Two siblings born preterm with large ears and hypopigmented hair who developed palmoplantar keratoderma and frontal skull bossing: a new syndrome?

This article discusses two children with an unknown syndrome characterized by palmoplantar keratoderma (PPK), hypopigmented hair, large ears, and frontal bossing. The children were both born preterm, with low birthweights, to consanguineous parents. They developed PPK shortly after birth.

Transfer of vitiligo after allogeneic bone marrow transplantation.

Adoptive transfer of donor immunity has been demonstrated in animals after bone marrow transplantation (BMT). In humans, several autoimmune diseases have been similarly transferred. Although BMT may, per se, be associated with a modulation of the recipient's immune system, which could trigger or even cause autoimmune diseases, both animal experiments and experience with humans show the likeliness of adoptive transfer of donor immunity to the recipient.