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PubMed1994

Punctate porokeratotic keratoderma--its occurrence with internal neoplasia.

Punctate porokeratotic keratoderma (PPK) represents a diffuse involvement of palms and soles by multiple, accuminate keratotic papules and plugs, histologically identified by parakeratotic cornoid lamellae. A possible association between PPK and internal malignancy has been previously noted by Herman in 1973. A patient with a 3-month history of PPK is described in which a bronchial carcinoma was recently diagnosed. This association led us to speculate that PPK could be a sign of internal neoplasia, as already established for other forms of palmoplantar keratoderma. We suggest that the presence of an underlying malignancy must be screened for when a diagnosis of PPK is proposed.

Bronchial Neoplasms↗
PubMed1988

Sézary cell count in exfoliative dermatitis.

Sézary cells were present in a number of less than 1000/mm3 in 52 of 78 cases of various benign diffuse dermatoses and mycosis fungoides. The count was higher than 1000/mm3 in two cases of Sézary syndrome in which the histopathology was that of mycosis fungoides and in six cases of exfoliative erythroderma of undetermined etiology with the histopathology of nonspecific chronic dermatitis. One of these six cases developed palmoplantar keratoderma, but abnormal cells were not present in the tissue. The diagnosis of Sézary syndrome in these cases of exfoliative erythroderma could not be established, even though the Sézary cell count was more than 1000/mm3, because the abnormal cells were absent in the skin biopsy specimen. It is concluded that the number of Sézary cells present is not significant for the diagnosis of Sézary syndrome. Our cases with the diagnosis of Sézary syndrome are an erythrodermic variant of mycosis fungoides rather than a progressive conversion from exfoliative erythroderma.

Adult↗
PubMedDate not supplied

[Extensive cutaneous candidiasis revealing cutaneous T-cell lymphoma: 2 cases].

BACKGROUND: During the course of immunodeficiency diseases, severe candidiasis can occur with extensive cutaneous and mucous membrane lesions. However, blood dyscrasias are very rarely revealed by diffuse candidiasis. We report two case of cutaneous T-cell lymphoma revealed by extensive and atypical cutaneous candidiasis. PATIENTS AND METHODS: Case No. 1:A 72-year-old woman presented a pruritic rash of circinate, serpiginous patches on glabrous skin and skinfolds with multiple intertrigo and rapidly worsening palmoplantar keratoderma. All mycological skin specimens tested positive for Candida albicans. Histological examination of a biopsy sample from a serpiginous patch revealed the presence of fungal elements while palmoplantar keratoderma biopsy showed an epidermotropic lymphocytic infiltrate in the superficial dermis evocative of mycosis fungoides. Blood tests showed a white cell count of 28 600/mm3 with 14% circulating Sezary cells and a T-cell clone. The T-cell lymphoma was treated with methotrexate, but the disease worsened a few months later, progressing to CD30- large T-cell pleomorphic lymphoma. The patient died of severe sepsis. Case No 2:A 60-year-old man presented a macular rash over the face, trunk and skinfolds as well as erythematous scaly annular plaques of the glabrous skin with lymphadenopathy. Cultures of skin scrapings were all positive for Candida albicans. Blood tests showed a white cell count of 15 000/mm3 with 30% circulating Sezary cells. A trunk patch biopsy revealed the histological appearance of mycosis fungoides. There was a T-cell clone in the peripheral blood and skin. DISCUSSION: In both cases, the patients presented with widespread annular and erythematous scaly lesions of the glabrous skin and skinfolds with evidence of Candida albicans on fungal tests of all skin scrapings. The discovery of circulating Sezary cells on a systematic smear for hyperleukocytosis led us to suspect underlying cutaneous T-cell lymphoma, which was confirmed by biopsy of the skin lesions accompanying the mycoses. Widespread cutaneous candidiasis can occur in patients with cell-mediated immunodepression. Cutaneous T-cell lymphoma can enhance such candidiasis through interference with skin integrity and impairment of cell-mediated immunity, with large amounts of IL10 and TGF-B, increased secretion of soluble interleukin-2 receptors (CD25) and impaired CD8 suppressor cell function.

Aged↗
PubMed1987

Retinoids in disorders of keratinization: their use in adults.

Hereditary disorders of keratinization may be a considerable handicap. Oral treatment with retinoids has been shown to be effective in many of these diseases. In the group of ichthyoses, the best results can be obtained in the various types of nonbullous congenital ichthyosis (erythrodermic autosomal recessive lamellar ichthyosis, nonerythrodermic autosomal recessive lamellar ichthyosis, autosomal dominant lamellar ichthyosis). It should be borne in mind, however, that retinoid therapy alone cannot lead to a complete response of these forms of ichthyosis and that this treatment cannot replace an appropriate topical treatment. During continuous treatment with etretinate a reduction of the dosis to 0.5 mg/kg is often necessary. Etretinate treatment of bullous congenital ichthyosiform erythroderma is more difficult, and it is advisable to begin with a low dosis of 0.25-0.5 mg/kg. The epidermolytic form of palmoplantar keratoderma is in our opinion no indication for retinoid treatment which seems to result inevitably in large erosions. Good or excellent results have been seen in other forms of palmoplantar keratoderma including mal de Meleda, Papillon-Lefèvre syndrome, erythrokeratodermia variabilis, verrucous epidermal nevi, Darier disease and pityriasis rubra pilaris. In patients with Darier disease it is wise to begin with a relatively low dosage of 0.5 mg/kg and to adjust the dosage to the further course of the disease. The same is true for the ichthyosis seen in the Netherton syndrome, which may be either a diffuse hyperkeratosis or ichthyosis linearis circumflexa. In view of the fact that any inherited keratinization disorder requires long-term treatment, the risk of bone toxicity should be carefully weighed against the benefit of this therapy. The results so far obtained indicate that the effect of etretin is comparable to that of etretinate in the treatment of inherited keratinization disorders. Intermittent therapy should be tried whenever possible. A combination therapy seems reasonable in pityriasis rubra pilaris of the adult type. We have seen good results by combination with PUVA treatment. Autosomal dominant ichthyosis vulgaris and X-linked recessive ichthyosis are inappropriate to treat with oral retinoid therapy because these diseases are too mild. Papillomatous epidermal nevi should also be excluded because they do not respond to the drug. Hailey-Hailey disease may even be worsened by this treatment. According to our experience, oral retinoid therapy has no effect in monilethrix.

Acitretin↗
PubMed1990

Punctate keratoses of the palms and soles and keratotic pits of the palmar creases.

Punctate keratoses of the palms and soles and keratotic pits of the palmar creases are frequently confused. A prospective study of 283 patients revealed a prevalence of 11% and 3%, respectively, in a metropolitan county hospital dermatology clinic. Punctate keratoses of the palms and soles and keratotic pits of the palmar creases are similar in size, number of lesions per palm, aggravation by trauma, and predominance in blacks and in men. These entities are different in appearance, distribution, age at onset, prevalence, symptoms, and prognosis. Punctate keratosis of the palms and soles and keratotic pits of the palmar creases should be considered independent entities. To help eliminate confusion, we propose that punctate keratoses refer only to the hyperkeratotic papules scattered diffusely in the palms and occasionally in the soles and that keratotic pits of the palmar creases refer only the hyperkeratotic, conical depressions confined to the palmar creases.

Adult↗
PubMed1995

Olmsted syndrome.

Nine cases of Olmsted syndrome have been reported in the world literature. In this syndrome, keratoderma usually starts during infancy on the palms and soles when the baby starts to use the feet for walking and the hands for grasping. Within weeks or months, there is progressive spread of solid, symmetrical, thick hyperkeratotic keratoderma to both palms and soles, surrounded by erythematous margins. Contraction of fingers and deep fissuring of the feet are common complications. Symmetrical, yellow-brown hyperkeratotic plaques and papules are also observed around body orifices such as the mouth, nares, inguinal region, and perianal and gluteal areas. Other clinical manifestations have been reported, including diffuse alopecia, thin nails, leukokeratosis of the oral mucosa, onychodystrophy, hyperkeratotic linear streaks, exaggerated keratosis pilaris, and large verrucous plaques in the axillae. In the differential diagnosis, other keratoderma and hyperkeratotic syndromes should be considered.

Adult↗
PubMed1981

Acrokerato-elastoidosis: a case report.

Acrokerato-elastoidosis belongs to the group of diffuse palmo-plantar keratoses without associated symptoms. We report a 25-year-old man who has had typical skin lesions for 5 years. The histopathological changes are described. By electron microscopy, changes in dermal elastic fibres as previously reported in patients with acrokerato-elastoidosis were absent.

Adult↗
PubMed1977

[Delayed manifestation of Costa's acrokeratoelastosis].

Acrokeratoelastoidosis, first described in 1952 bei O.G. Costa, belongs to the group of diffuse palmoplantar keratoses without associated symptoms. Probably it is an autosomal dominant trait. We observed a 49 year old female who has noticed her typical skin lesions for 2 years. The most marked histological features are proliferative hyperkeratosis, an almost complete lack of the fine subepithelial elastic fibre network and a marked rarefication of the coarse elastic fibres in the dermis.

Acrodermatitis↗
PubMed1993

[Pseudo-ainhum in Vohwinkel disease. Keratoma hereditarium mutilans].

An 11-year-old Turkish boy who has suffered from palmoplantar keratosis since his first year of life is presented. He is the only one of a large family to be affected. The diffuse keratosis extends to the back of the hands and feet and still has a progressive course. At the age of 6 he developed a symmetric high-tone acoustic impairment and at 10, an ainhum-like constricting band around the fifth digit of the left hand. This constellation of symptoms is highly characteristic for mutilating keratoma (Vohwinkel's disease), which is a rare disorder of keratinization. The majority of cases in the literature have had an autosomal dominant pattern of inheritance, although sporadic cases like this have also been reported as well. If constricting band proceeds to the point where spontaneous amputation seems imminent, a therapy with orally administered retinoids should be considered.

Ainhum↗
PubMed1979

[Pachydermatoglyphy. A sign of malignancy for acanthosis nigricans (author's transl)].

The pachydermatoglyphy corresponds from the clinical point of view to a diffuse palmo-plantar keratodermia, of a yellowish color, characterised by a rough thickening of the finger prints. The presence of the pachydermatoglyphy has been pointed out in six cases of malignant acanthosis nigricans. This was already found in previous descriptions. Pachydermatoglyphy is specially found in malignant acanthosis nigricans, but we have also observed such cases in erythrokeratodermia with pilar keratosis and deafness.

Acanthosis Nigricans↗
PubMedDate not supplied

Palmar-plantar keratoderma of Unna Thost associated with atopic dermatitis: an underrecognized entity?

We report six cases of palmar-plantar keratoderma of Unna Thost (PPKUT) associated with atopic dermatitis. All had typical features of PPKUT with diffuse, yellowish thickening on the palms and soles with a well-defined erythematous rim of demarcation on the sides associated with palmar-plantar hyperhidrosis. The changes were obvious since birth or arose during early life, and were persistent. We believe that the association between the two disorders is not coincidental but an underrecognized entity that may shed light on the underlying pathogenesis of these two conditions.

Adult↗
PubMed2001

Vohwinkel's syndrome in three generations.

Vohwinkel's syndrome or keratoderma hereditaria mutilans is a diffuse, honeycombed, palmar, and plantar keratosis usually accompanied by pseudoainhum near the distal interphalangeal creases. The mutilating keratoderma associated with sensorineural hearing loss is thought to have an etiologic basis, resting on a mutation of the GJB2 gene, which encodes the gap junction protein connexin26 (Cx26). This specific mutation results in impaired epidermal differentiation as well as inner ear function. We describe a patient with Vohwinkel's syndrome accompanied by high-frequency sensorineural hearing loss whose mother and son were similarly affected.

Adolescent↗
PubMed1998

Skin signs in the diagnosis of thallium poisoning.

A 45-year-old man developed a painful and rapidly progressive sensory-motor polyneuropathy associated with confusion and convulsions. This resulted in hypoventilation and led to respiratory failure and coma. A rapid and diffuse alopecia occurred after 3 weeks in the intensive care unit. Examination of hair roots under polarized light detected dystrophic anagen hairs with dark bands caused by empty spaces in the disorganized cortex. These dark zones were originally reported in patients with thallium poisoning and a toxicological investigation confirmed thallium exposure. The classical systemic symptoms and the various dermatological signs are reviewed, and the origins of contamination and physiopathology discussed.

Alopecia↗
PubMed2001

Ulcerating plantar keratoderma in association with systemic lupus erythematosus.

This report highlights the finding of ulcerative plantar keratoderma in two patients with systemic lupus erythematosus (SLE). Both patients suffered from painful plantar ulcerations and fissures; in one patient there was diffuse desquamation over the entire plantar surface, while the other patient's lesions were focal and accentuated over weight-bearing surfaces. Other etiologies for keratoderma including papulosquamoua disease, contact dermatitis, tinea and primary keratodermas were excluded. Both patients were resistant to multiple topical therapies including super-potent topical corticosteroids, vitamin D analogues and retinoids, but did report moderate relief with hydrocolloid dressings applied over super-potent topical corticosteroids and pressure off-loading measures. Lupus-associated keratoderma can be recurrent and recalcitrant to treatment, often necessitating aggressive therapy and particular attention to advanced wound care methodologies. While not a specific cutaneous sign of lupus, it should be recognized as a cause for considerable morbidity.

Aged↗
PubMed1996

[Squamous cell carcinoma of the lung associated with palmo-plantar hyperkeratosis].

Palmo-plantar hyperkeratosis (PPH) is a diffuse keratoderma of the palms and soles that has been reported to be associated with internal malignancies such as lung and esophageal carcinomas. When PPH is associated with malignancy the prognosis is poor. The present patient was a 48-year-old man with PPH who suffered from squamous cell carcinoma of the lung. The pathological stage was T4N2M1 and the patient died of recurrent tumor 17 months after surgery. Because the prognosis of lung carcinoma associated with PPH is poor, patients with these conditions should undergo frequent medical examinations, which should include chest radiography and cytologic examination of sputum.

Carcinoma, Squamous Cell↗
PubMed1984

Corneal changes, hyperkeratosis, short stature, brachydactyly, and premature birth: a new autosomal dominant syndrome.

We report on an autosomal dominant syndrome consisting of unique corneal epithelial changes, diffuse palmoplantar hyperkeratosis, distal onycholysis, brachydactyly, short stature, premature birth, and dental problems. This condition has been present in seven persons in three generations of one family. Corneal biopsies demonstrate mild dysplastic changes in the epithelium. Skin biopsies show hyperkeratosis and acanthosis. In both eye and skin specimens, results of stains for polysaccharides, amyloid, and tyrosine were unremarkable. Roentgenograms of the hands show short distal phalanges, short 4th metacarpals, and constriction of the heads of some of the metacarpals. In three of four affected relatives, a variable medullary narrowness is seen. In mode of inheritance, clinical appearance, and/or associated defects. This syndrome appears to differ from previously reported conditions that include palmoplantar hyperkeratosis and/or corneal changes.

Adult↗
PubMed1978

Regression of a T-cell lymphoma after administration of antithymocyte globulin.

A patient with Sézary syndrome developed a diffuse undifferentiated lymphoma of T-cell origin. After becoming resistant to multiple chemotherapeutic agents, the patient was treated with antithymocyte globulin. A 75% reduction in adenopathy and complete resolution of skin erythema was observed during an 8-day period. In addition the percent of circulating T cells and the ability of those cells to respond to phytohemagglutinin and concanavalin A were reduced after antithymocyte globulin therapy. The patient died of an intracerebral hemorrhage secondary to profound thrombocytopenia. The study suggests that tumor lysis may be achieved by passive antibody therapy in certain advanced lymphomas.

Antilymphocyte Serum↗