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Erythroderma, hair loss, and sinus histiocytosis with massive lymphadenopathy in a XYY-male.

This paper presents a case of sinus histiocytosis with massive lymphadenopathy (SHML) presenting as erythroderma in an XYY-male aged 27. The patient had little or no scalp, axillary, or pubic hair. Histologically, lymph nodes indicated dilatation of the sinuses with proliferation of benign-appearing histiocytes possessing abundant clear cytoplasm, admixed with giant cells and pericapsular fibrosis. Infiltrating histiocytes in the lymph nodes were OKM-1 (CD11b) positive, which is characteristic of monocyte-granulocyte lineages. In the present case, the abnormal presence of erythroderma, hair loss, SHML, and a chromosomal abnormality, XYY, were thought to be incidental features.

Adult↗

A case of bullous congenital ichthyosiform erythroderma (BCIE) caused by a mutation in the 1A helix initiation motif of keratin 1.

Bullous congenital ichthyosiform erythroderma (BCIE) is an autosomally dominant inherited disorder characterized by erythematous, erosive, and bullous skin lesions over the entire body at birth and abnormal hyperkeratosis on the palmoplantar sufaces as the patient grows older. BCIE is caused by a mutation in the keratin 1 (K1) and/or keratin 10 (K10) genes, and most pathogenic mutations are found within the helix initiation and termination motifs of the central helical rod domain (K1 and K10) or the upstream H1 homology domain (K10). In addition to inherited cases, sporadic cases due to a new mutation account for approximately half the total cases of BCIE. We report herein a typical sporadic case of BCIE with erythroderma, erosion, and blisters on the entire body surface at birth and palmoplantar and flexuaral areas of hyperkeratosis in the later stage. We found in this case a novel mutation, 559C to T, at amino acid position 187, which resulted in a leucine to phenylalanine substitution within the helix initiation motif of K1.

Amino Acid Substitution↗

Oral retinoic acid as therapy for congenital ichthyosiform erythroderma.

Oral retinoic acid has been therapeutically beneficial for three patients with congenital ichthyosiform erythroderma and one with congenital bullous ichthyosifrom erythroderma. Response to therapy was slow but definite with suppression of scale formation, marked reduction of painful cracking of the underlying epidermis and disappearance of skin irritation. Side effects have not occurred. Therapy had to be maintained to prevent recurrence of symptoms.

Administration, Oral↗

Erythroderma associated with mixed lymphocyte-endothelial cell interaction and Staphylococcus aureus infection.

We report 11 consecutive cases of erythroderma, a high percentage of which were associated with the growth of Staphylococcus aureus in cultures from blood, joint fluid or skin. In biopsies from all the patients we found close morphological associations between lymphocytes and endothelial cells, with some of the lymphocytes showing features of blastoid transformation to T helper lymphocytes. In extreme cases, sheets of T cells, including T helper lymphocytes, formed a syncytium with endothelial cells in the dermis. Marked capillary proliferation was noted both on light and electron microscopy. We suggest that erythroderma is precipitated by antigens such as protein A, a potent T cell mitogen present on the cell surface of Staphylococcus aureus, or by drugs, such as phenytoin. These antigens induce antigen presentation by individual endothelial cells, leading to T helper transformation and lymphocyte proliferation. Endothelial proliferation resulting from lymphocyte-endothelial interaction results in the vascular proliferation associated with this syndrome.

Adult↗

Quantifying systemic absorption of topical hydrocortisone in erythroderma.

The systemic absorption of topical hydrocortisone (HC) was quantified in seven patients with erythroderma, using the ratio of the areas under the curves for plasma concentration vs. time, following topical and intravenous administration. Over a period of 24 h, 19-93 mg of HC was absorbed systemically, corresponding to 4-19% of the total topical dose of 500 mg. Thus, topical HC therapy of erythroderma is accompanied by a pharmacologically significant systemic dose.

Administration, Cutaneous↗

Ichthyosis bullosa of Siemens and bullous ichthyosiform erythroderma--variants of the same disease?

A patient is described with features of both bullous ichthyosiform erythroderma (BIE) and ichthyosis bullosa--a separate entity first described in 1937 by Siemens. This combination of characteristics has not been previously reported. Bullous ichthyosiform erythroderma and ichthyosis bullosa of Siemens, occurring together in this patient may best be regarded as varying expressions of a single genodermatosis.

Aged↗

Serum immunoglobulins in psoriatic erythroderma.

The cause of psoriatic erythroderma (PE) is still unknown. Elevation of serum IgE has been reported in erythroderma, and as serum hyper-IgE is Th2 cell dominated it is of interest to investigate the serum IgE level in PE. In this study, the level of immunoglobulins in the sera of PE patients was analysed by a retrospective case-control study using psoriasis vulgaris (PV) patients as controls. The PE and PV patients were matched in a 1:3 pattern: the first three age and sex matched PV inpatients were selected. All of the subjects were nonatopic and without allergic history. The serum IgE level was found to be elevated in 81.3% of the PE group, which is much higher than that (6.3%) of the controls (odds ratio, 65.0; 95% CI, 11.7-361.2; P < 0.001; chi2 test). The mean level of serum IgE was much higher in the PE group (272.38 +/- 207.63 IU/mL vs. 53.20 +/- 86.05 IU/mL, P < 0.001, Student's t-test). No differences were found for other immunoglobulins. These results suggest that in PE the Th1/Th2 cell imbalance may be switched from Th1 dominant to Th2 dominant. The exact role of serum IgE in PE should be investigated further.

Adolescent↗

Psoriatic erythroderma associated with enalapril.

A 59-year-old man with a 35-year personal and positive family history of psoriasis was admitted to our department for treatment of psoriatic erythroderma. The patient had commenced therapy with enalapril 10 mg b.i.d. for the treatment of hypertension approximately 6 weeks before hospitalization. Five weeks after the initiation of enalapril, his psoriasis began to flare, and for a period of about 1 week it reached the extent of erythroderma. The patient did not associate the psoriatic flare with other factors such as infections, trauma, or stress. The patient presented with diffuse erythema and pronounced desquamation covering his entire trunk, scalp, and extremities (Figure). Nearly 100% of the body surface area was involved. The palms and soles were also affected, displaying erythema, hyperkeratosis, and painful fissures. The nails showed pits, oil spots, and subungual hyperkeratosis. The patient also had psoriatic arthritis affecting the interphalangeal joints of his fingers. Laboratory tests revealed an elevated erythrocyte sedimentation rate, an elevated creatinine level of 180 mmol/L, a blood urea nitrogen level of 10.8 mmol/L, and a uric acid level of 716 mmol/L. Urinalysis showed proteinuria of 1.5 g/24 h. The patient's renal condition was diagnosed as chronic tubulointerstitial nephritis, most probably related to his dermatologic disease. Allopurinol and dietary measures were recommended. Following treatment with methotrexate and replacement of enalapril therapy, the erythema and scaling gradually subsided and became confined to his pre-eruptive chronic plaques (approximately 5% of body surface area). Rechallenge with enalapril was not performed.

Angiotensin-Converting Enzyme Inhibitors↗

Re-expression of disease-characteristic features of non-bullous congenital ichthyosiform erythroderma (CIE) after grafting of the pathological keratinocyte cultures to athymic mice.

Epidermal keratinocytes separated from skin lesions of non-bullous congenital ichthyosiform erythroderma were investigated in an attempt at experimental reproduction of this keratinization disorder. In vitro studies on growth and differentiation of pathological keratinocytes isolated from the influence of the host's dermal and humoral components were performed using the immersed epidermal cell culture technique. Ten to 25-day-old confluent and stratified cultures were examined by means of photonic and electron microscopy, and stained with various differentiation markers for indirect immunofluorescence studies. The cultured epidermis showed low-grade differentiation and no clear-cut evenly distributed signs of the original disease. Grafting on congenitally athymic nude mice allowed further differentiation of the epidermal sheets and re-expression of the histologic and ultrastructural features of non-bullous congenital ichthyosiform erythroderma. Thus, the purely epidermal origin of this particular form of autosomal recessive ichthyosis could be confirmed. Large amounts of pathological keratinocytes generated from small skin biopsies may be used for experimental purposes after grafting on several athymic animals.

Animals↗

Detection of human herpes virus 6 (HHV 6) in the skin of a patient with primary HHV 6 infection and erythroderma.

Human herpes virus 6 (HHV 6) has been implicated as the causative agent of exanthema subitum in young children. Recently, we reported two cases of a severe, infectious, mononucleosis-like syndrome resulting from a primary HHV 6 infection in immunocompetent adults. Both of these patients had the skin condition generally referred to as "erythroderma". A skin-biopsy specimen from one of them, a 43 year old man, was examined. Using immunohistochemical staining and in situ hybridisation, lymphocytes infected with HHV 6 were found in the skin. It is proposed that the erythroderma in immunocompetent adults infected with primary HHV 6 is provoked by infiltration of infected inflammatory cells or infected neoplastic lymphocytes into the dermis.

Adult↗

Myelodysplasia presenting as erythroderma.

Erythroderma has not previously been reported to be a feature of myelodysplasia. We report two cases of myelodysplasia presenting with erythroderma, one of which was associated with skin infiltration by blast cells.

Aged↗

Vogt-Koyanagi-Harada disease: extensive vitiligo with prodromal generalized erythroderma.

The occurrence of vitiligo with prodromal erythema in Vogt-Koyanagi-Harada (VKH) disease is rarely observed. We describe a 68-year-old Chinese woman who developed extensive vitiligo 6 months after the onset of a generalized erythroderma and 12 months after the onset of acute bilateral uveitis. She also had a 4-year history of psoriatic plaques on the trunk which resolved completely without recurrence 15 months after the onset of erythroderma. The extensive cutaneous inflammation prior to the onset of vitiligo in our patient with VKH disease has not been reported before and strongly suggests an immunologic etiology for VKH disease.

Aged↗

Erythroderma as the initial presentation of the acquired immunodeficiency syndrome.

A 32-year-old black homosexual man, seronegative for human immunodeficiency virus antibody, presented with erythroderma. His peripheral blood was significant for circulating Sézary-like cells bearing the CD8(+) phenotype. Eighty-eight percent of his lymphocytes were CD8(+) as well. He seroconverted 3 months after the initial presentation. We conclude that erythroderma was the presenting sign of the acquired immunodeficiency syndrome.

Acquired Immunodeficiency Syndrome↗

Elevated n-alkanes in congenital ichthyosiform erythroderma. Phenotypic differentiation of two types of autosomal recessive ichthyosis.

Previously considered to represent a single genetic disorder, autosomal recessive ichthyosis was examined in clinical and lipid biochemical studies of 18 patients with this condition and instead disclosed to be two distinct diseases. Six patients displayed clinical features of classical lamellar ichthyosis (LI), which is characterized by monomorphous features, including large, dark, platelike scales, severe ectropion, and a uniformly severe, unremitting course. 11 patients displayed clinical features of nonbullous congenital ichthyosiform erythroderma (CIE) characterized by fine white scales, prominent erythroderma, a milder course, and a variable prognosis. CIE could be separated biochemically from LI by the invariable presence of elevated quantities of n-alkanes in scale (CIE, 24.8 +/- 1.9% vs. LI, 7.2 +/- 1.6%, and normal, 6.5 +/- 0.9%), which suggested a primary disorder in neutral lipid metabolism. In light of the distinctive clinical features of each, these biochemical studies indicate that autosomal recessive ichthyosis comprises two distinct disorders.

Alkanes↗

A case of eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma.

This report describes a patient with eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. A 50-year-old man presented with a complaint of precordial pain. However, the only abnormal finding on examinatioin was eosinophilia (1617 eosinophils/microl). Three years later, the patient developed chronic eczema, and was diagnosed with erythroderma posteczematosa. One year later, a tumor was detected in the right auricule, and a diagnosis of Kimura's disease was made, based on the biopsy findings. The patient developed progressive dyspnea 6 months later and was found to have cardiomegaly and a depressed left ventricular ejection fraction (17%). A diagnosis of eosinophilic myocarditis was made based on the results of a right ventricular endomyocardial biopsy. The eosinophilic myocarditis and erythrodrema were treated with steroids with improvement of both the eosinophilia and left ventricular function.

Angiolymphoid Hyperplasia with Eosinophilia↗

Dapsone-induced erythroderma with Beau's lines.

A 35-year-old female with borderline lepromatous (BL) leprosy who suffered from dapsone-induced erythroderma is reported. Sudden onset of erythroderma gave rise to a temporary arrest of the function of nail matrix with the resultant Beau's lines. She rapidly recovered with omission of dapsone and therapy with systemic corticosteroids and a topical emollient. In view of the potentially fatal hypersensitivity reaction, we suggest that any patient on multidrug therapy for leprosy needs an urgent referral to a dermatologist if the patient develops a skin rash during the first two months of treatment.

Adult↗

Congenital ichthyosiform erythroderma: particulate staining pattern of TGK.

A case of late onset non-bullous congenital ichthyosiform erythroderma (CIE) was studied. This patient was not born as a collodion baby and did not have skin abnormalities until 9-10 years of age. She gradually developed erythroderma and fine scales, callosities of her feet, and a mild ectropion. Since recent work has revealed that in the majority of CIE patients, transglutaminase (TGK) is distributed in the cytoplasm of granular cells and horny cells (11), TGK was studied in our case. It was found that TGK was distributed along the cell periphery of horny cells and also in the cytoplasm of granular cells. In the control skins, TGK was stained along the cell periphery of horny cells and granular cells. The marginal band formation was normal. Involucrine and loricrin, the building materials of the marginal band whose-cross-linking is mediated by TGK, were normally stained in the upper epidermis. Cytoplasmic TGK of granular cells and normal development of the marginal band may serve as a helpful diagnostic marker of CIE, particularly because the often confusing collodion baby of lamellar ichthyosis may lack TGK staining and the marginal band altogether.

Age of Onset↗

Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma.

Pemphigus foliaceus is a cutaneous autoimmune blistering disease that is characterized by lower morbidity and mortality than those observed in pemphigus vulgaris or paraneoplastic pemphigus. However, erythrodermic forms of the endemic variant of pemphigus foliaceus have been associated with a higher mortality. We report a case of nonendemic pemphigus foliaceus that presented as fatal bullous exfoliative erythroderma, and thus, we will emphasize the inclusion of this entity in the differential diagnosis and the use of skin direct immunofluorescence in the evaluation of patients with erythroderma.

Aged↗