1/24. A case of Unna-Thost disease accompanied by epidermophyton floccosum infection. We report herein a case of 61-year-old man with Unna-Thost disease (nonepidermolytic hereditary palmoplantar keratoderma) who had been suffering from refractory dermatophyte infection. Diffuse palmoplantar hyperkeratosis developed in his infancy. Coarse scaling, fissures, marked erythema, and nail deformities appeared in his early adult life. Microscopic examination revealed fungal elements in scales and nail particles. Cultures of those scales isolated epidermophyton floccosum. Genealogical study demonstrated that his mother, sister, brother, daughter, and niece had had similar tylosis, and all of the affected individuals except his daughter had been proved to have E. floccosum or trichophyton rubrum infection. Oral itraconazole (100mg/day) was highly effective in treating his dermatophytosis without any adverse effects. Scaling, fissures and underlying erythema disappeared within four months. ( info) |
2/24. To B or not to B: is tylosis B truly benign? Two North American genealogies. Tylosis is a rare, autosomal dominant syndrome presenting with hyperkeratosis of the palms and soles of the feet. Two types have been identified. Late onset tylosis (type A) is reported to be associated with a high incidence of esophageal carcinoma, whereas early onset tylosis (type B) appears to be a benign disorder. This distinction has significant implications for surveillance and prognosis. We report two families exhibiting early onset type B tylosis, spanning five and seven generations, respectively, and believe these to be the first two extensive genealogies of tylosis type B reported in north america. They serve to verify the features of type B tylosis and its benign prognosis. The world literature is reviewed and clinical relevance is discussed. Recommendations for follow-up of afflicted individuals are proposed. ( info) |
3/24. Transgrediens et progrediens palmoplantar keratoderma (Greither's disease) with particular histopathologic findings. We describe a patient with transgrediens et progrediens palmoplantar keratoderma (Greither's disease). Ten of the 25 members of this patient's family in six consecutive generations were affected by the disorder. The pedigree was consistent with an autosomal dominant inheritance pattern, with variable penetrance. The proband's physical examination showed typical signs of Greither's disease. The most striking findings were seen in histopathologic study and consisted of round, focal areas of orthohyperkeratosis located on delled areas of the epidermis. These histopathologic features were present in both the lesions of the palms and dorsum of the hands. We review the clinical manifestations and histopathologic findings of this particular variant of palmoplantar keratoderma and the differential diagnosis. ( info) |
4/24. Non-small-cell lung cancer with nonfamilial diffuse palmoplantar keratoderma. Palmoplantar keratoderma (PPK) is a congenital or acquired disorder characterized by the abnormal thickening of the skin of the palms and soles. The thickening can present as a diffuse, focal or punctate pattern. It has been reported to be associated with internal malignancies such as lung and esophageal carcinomas. When PPK is associated with malignancy the prognosis is poor. patients with these conditions should undergo frequent medical examinations, which should include chest radiography and cytologic examination of the sputum. The present patient is a 47-year-old-man with PPK who suffered from metastatic non-small-cell carcinoma of the lung. ( info) |
5/24. A keratin 9 Gene mutation (Asn160Ser) in a Japanese patient with epidermolytic palmoplantar keratoderma. We described a 5-year-old Japanese girl with epidermolytic palmoplantar keratoderma and examined her for a keratin 9 gene mutation. physical examination disclosed diffuse yellowish hyperkeratosis with an erythematous border limited strictly to the palms and soles. Histological examination revealed hyperkeratosis with vacuolar degeneration in the spinous and granular layers of the epidermis. sequence analysis demonstrated an A to G transition at the middle position of codon 160 in the 1A domain of the keratin 9 gene. The amino acid at codon 160 was deduced to have changed from asparagine (Asn) to serine (Ser). This is the first case with an Asn160Ser mutation in a Japanese. The substitution of Ser for Asn at codon 160 of the keratin 9 gene is assumed to be fatal for keratin filament assembly regardless of race or ethnicity. ( info) |
6/24. carcinoma of stomach in a patient with familial tylosis. The association of tylosis with esophageal cancer has been extensively reported but association with gastric cancer is rare. We report a 55-year-old man with familial tylosis and carcinoma of the stomach for which radical gastrectomy was done. Repeat endoscopy 3 years later is normal. ( info) |
7/24. An unusual case of palmoplantar keratoderma. A 55-year-old woman with palmoplantar keratoderma presented an associated hyperhidrosis with distinct odour and maceration. She had had the lesions for about 20 years and this seemed to be an isolated case in her family. This case appeared very unusual because there were no signs of acanthokeratolysis in the biopsies. Two months of treatment with acitretin (Neotigason; 25 mg daily), produced a spectacular result: clearance of all the lesions on both hands and a strong diminution of the lesions on the soles. The Unna-Thost variant of palmoplantar keratoderma usually appears in the first few months of life, and it rarely appears in the third decade. The condition is inherited as an autosomal dominant with high penetrance and expressivity. Our subject appeared to be an exception to these two facts. ( info) |
8/24. Indication for the identity of palmoplantar keratoderma type Unna-Thost with type Vorner. Thost's family revisited 110 years later. Palmoplantar keratoderma (PPK) type Unna-Thost is known to be the most common form of a hereditary disorder of keratinization of palms and soles. The disease is clinically identical with PPK type Vorner which is histologically characterized by epidermolytic hyperkeratosis. By reinvestigation of the family originally seen by Thost in 1880, the features of epidermolytic hyperkeratosis were found histologically confirming the diagnosis PPK of Vorner. This proves the identity of PPK type Thost with PPK of Vorner. Because of the histological variability of epidermolytic hyperkeratosis, detailed light and electron microscopic studies are necessary in cases of diffuse types of PPK. ( info) |
9/24. multiple myeloma in a patient with systemic lupus erythematosus, myasthenia gravis and non-familial diffuse palmoplantar keratoderma. The coexistence of autoimmune diseases and malignancies including lymphoproliferative diseases is often reported in the literature. Here we report an unusual case with two autoimmune diseases--myasthenia gravis (MG) and systemic lupus erythematosus (SLE) associated with unique palmoplantar keratoderma (PK) which preceded the development of multiple myeloma (MM) for twenty and seven years respectively. MG associated with non-malignant thymoma developed in 1981 and was successfully treated with thymectomy and physostigmine. Thirteen years later SLE was diagnosed and until now it is also accompanied by skin lesions corresponding to non-familial, diffuse palmoplantar keratoderma which is resistant to treatment. In 2001 the patient revealed inguinal and abdominal lymphadenopathy first diagnosed as extramedullary plasmacytoma and then as multiple myeloma on the basis of bone marrow infiltration and monoclonal gammopathy. Therapy with VAD regimen achieved complete remission of the MM and significant improvement of the skin changes lasting for six months. We failed to collect sufficient numbers of CD 34 cells for peripheral blood stem cell transplantation. Now the malignancy is in partial remission after CHOP therapy and the skin lesions have returned to their initial status. To our knowledge, this is the first case to be reported with coexistence of these four diseases. ( info) |
10/24. Spiny keratoderma of the palms and soles. Six cases of a dermatosis characterized by "music box spine" keratotic papules limited to the palms and soles have been reported. The names that have been used for this type of dermatosis most commonly incorporate the term porokeratosis; for example, "porokeratosis palmaris et plantaris," or "punctuate porokeratotic keratoderma." We suggest the name "spiny keratoderma of the palms and soles," because the clinical, histologic, and electron microscopic features of this entity are distinct from porokeratosis. In addition, unlike porokeratosis, this entity has not shown malignant potential. We report a case of "spiny keratoderma of the palms and soles" and show that topical 5-fluorouracil is an effective treatment. ( info) |