Nevoid Basal Cell Carcinoma Syndrome and Hairy Skin Patches

We report a case of an increasing number of discrete patches of darkly pigmented terminal hair in a patient with nevoid basal cell carcinoma syndrome. This case adds to a small case series of three patients which have previously reported this observation. We report this case to highlight hairy patches as an important clinical feature associated with nevoid basal cell carcinoma syndrome.     

Werner's syndrome and astrocytoma

Werner's syndrome, a relatively rare and autosomal recessive disorder, is well known to be characterized by a high frequency of malignant neoplasms. Werner's syndrome has not infrequently been associated with meningiomas. We report a case of Werner's syndrome and temporal astrocytoma in a 49-year-old male. As far as we are aware, this is the first report of the association of Werner's syndrome and astrocytoma.     

A Novel VPS33B Mutation in a Patient with Arthrogryposis‐Renal Dysfunction‐Cholestasis Syndrome

We report a case of arthrogryposis‐renal dysfunction‐cholestasis (ARC) syndrome in a girl with a novel VPS33B mutation. To our knowledge, this is the first reported case of ARC syndrome in the United States.     

Sweet's syndrome in association with non-Hodgkin's lymphoma

Sweet's syndrome is an acute neutrophilic dermatosis frequently found in association with other conditions, particularly inflammatory and neoplastic disease. We report here a patient who developed the condition 2 years after the diagnosis of non-Hodgkin's lymphoma (NHL), in this case affecting: a tonsil, the thirteenth report of such an association. We discuss the diagnosis, investigation and management of Sweet's syndrome in the context of the current case.     

Romberg's syndrome: a case report

A first case of Romberg's syndrome from Libya is reported. Romberg's syndrome is a rare disease causing progressive facial hemiatrophy. It was first described by Parry in 1825 and then by Romberg in 1846. Since then there have been sporadic case reports and reviews in the literature [1-5]. For the first time, we report a case from Libya.     

Trigeminal trophic syndrome

A case report of a 51-year-old woman who developed trigeminal trophic syndrome Following craniectomy is presented. This syndrome represents trophic ulceration by self-induced trauma to skin in the trigeminal area. Treatment of the present case was complicated by the patient's underlying psychological problems.     

Solitary Milialike Idiopathic Calcinosis Cutis: A Case Unassociated with Down Syndrome

We report a unique case of solitary milialike idiopathic calcinosis cutis (MICC) in a healthy Korean woman, which is not associated with Down syndrome. This case of MICC would be a form of idiopathic calcinosis cutis, which can be solitary or multiple, sporadic or associated with Down syndrome.     

Gianotti-Crosti syndrome preceded by oral polio vaccine and followed by varicella infection

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is considered a nonspecific cutaneous host response to a variety of infectious agents, particularly viruses. Herein we report a case of Gianotti- Crosti syndrome preceded by oral polio vaccine and followed by varicella infection. The present case may support the hypothesis of initial immunization and following viral infection as a possible mechanism for Gianotti-Crosti syndrome.     

Zinsser-Cole-Engmann syndrome

Zinser-Cole-Engmann syndrome is a rare syndrome characterized by atrophy and pigmentation of skin, nail dystrophy and oral leukoplakia. Here we report a case of this rare entity.     

A Case of CREST Syndrome Associated with Sick Sinus Syndrome

Cardiac manifestations of the CREST syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of CREST syndrome associated with sick sinus syndrome. Histopathology of cardiac muscle revealed fibrotic changes, suggesting that such changes may be pathogenetically related to CREST syndrome.     

A case of blue rubber bleb nevus syndrome with gastrointestinal and central nervous system involvement

Blue rubber bleb nevus syndrome, or Bean syndrome, is a rare sporadic disorder of venous malformations of the skin and internal viscera. We report a rare case of blue rubber bleb nevus syndrome with gastrointestinal and central nervous system involvement.     

Olmsted syndrome: report of a new case with unusual features

Olmsted syndrome is a rare disorder characterized by the combination of periorificial keratotic plaques and bilateral palmoplantar keratoderma. New associated features continue to be reported. Olmsted syndrome in a female patient is particularly rare, and we report such a case having a haemangioma in addition to other features of the syndrome.     

Maffucci'S syndrome associated with hyperparathyroidism

Maffucci's syndrome is a rare, congenital, nonhereditary, mesodermal dysplastic disease characterized by venous malformations and benign cartilaginous tumors. The occurrence of endocrine tumors in Maffucci's syndrome is very rare. We report a case of Maffucci's syndrome associated with hyperparathyroidism and multinodular goiter.     

Sweet's syndrome associated with ovarian carcinoma

Sweet's syndrome is sometimes associated with haematological malignancies and less frequently with solid tumors. We report a new case of association of a Sweet's syndrome with an ovarian carcinoma.     

Brittle nails and dyspareunia as first clues to recurrences of malignant glucagonoma

Glucagonoma syndrome is a paraneoplastic syndrome in which the occurrence and resolution of the characteristic necrolytic migratory erythema lesions parallel the course of the underlying glucagonoma. Nail abnormalities and dyspareunia are rarely reported in this syndrome. We describe a case of glucagonoma syndrome in which recurrent brittle nails and dyspareunia gave the patient the first clues of the recurrence of glucagonoma. It is possible that the significance of onychoschizia and dyspareunia has been overlooked in glucagonoma syndrome because patients might not report these problems to their doctors. Our case illustrates the importance of examining the nail and genital mucosa in patients with glucagonoma syndrome and including this syndrome in the differential diagnosis of onychoschizia and dyspareunia.     

Cardiofaciocutaneous syndrome: a rare entity

The cardiofaciocutaneous (CFC) syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.     

Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma, spiradenoma, and syringoma

Brooke-Spiegler syndrome is a rare, autosomally dominant disease characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas. We report herein a case of Brooke-Spiegler syndrome in a 55-year-old woman associated with multiple cylindromas, spiradenomas, trichoepitheliomas, and syringomas that developed on her face. Her 52-year-old sister also suffered from multiple trichoepitheliomas. Although there is variable clinical and histological expression in Brooke-Spiegler syndrome, all of the tumors encountered in our case were related to folliculosebaceous-apocrine units.     

Olmsted syndrome

Olmsted syndrome is an uncommon inherited disorder of keratinization that presents mutilating palmoplantar keratoderma, perioral hyperkeratosis, leukokeratosis and alopecia. We report a case of this rare syndrome diagnosed in a 48-year-old woman and confirms the existence of a generalized abnormality in keratin expression. Immunoreactivity in our case suggests an abnormal expression of keratins 5 and 14 similar to the observed in other hyperproliferative disorders.     

Drug-induced Sweet's syndrome secondary to hepatitis C antiviral therapy

Pegylated interferon-alpha in combination with ribavirin currently represents the therapeutic standard for the hepatitis C virus infection. Interferon based therapy may be responsible for many cutaneous side effects. We report a case of drug-induced Sweet's syndrome secondary to hepatitis C antiviral therapy. To our knowledge, this is the first reported case of Sweet's syndrome in association with pegylated interferon-alpha therapy.     

Parotid oncocytoma in the Birt-Hogg-Dubé syndrome

Birt-Hogg-Dubé syndrome consists of fibrofolliculomas, trichodiscomas, and acrochordons and has been associated with several noncutaneous tumors. We report the first case of Birt-Hogg-Dubé syndrome in association with a parotid oncocytoma, a rare salivary gland tumor.