Difficulties in treating steroid withdrawal: intermittent ACTH and low dose systemic cyclosporin used to treat a senile erythroderma patient

The tapering or termination of prolonged strong topical and/or systemic corticosteroid application for extensive generalized eczema has adverse effects on the body and thus presents a very difficult situation. The present case is that of a 68-year-old man with erythroderma following eczema, whose steroid withdrawal was successfully treated with intermittent ACTH and low dose systemic cyclosporin administration over a period of one year.     

Erfolgreiche Behandlung einer Pityriasis rubra pilaris (Typ 1) durch Kombinations-Therapie mit Infliximab und Methotrexat

Pityriasis rubra pilaris (PRP) is often difficult to treat. A 65‐year‐old women presented with a two week history of widespread erythroderma and scaling with areas of sparing (nappes claires). She also had follicular hyperkeratoses and palmar fissuring. The clinical picture and histology led to the diagnosis of PRP. She failed to respond to initial therapy which included topical and systemic corticosteroids. She was then treated with intravenous methotrexate (MTX) 15–30 mg weekly. Because of the poor response we intensified her regime with infliximab (5 mg/kg). Altogether our patient received infliximab three times together with MTX, which was later given orally. We report for the first time the successful combination of infliximab and MTX for the treatment of PRP.     

Statistical observations of pemphigus at the Nara University Hospital from 1975 to 1989

From 1975 to 1989, 12 patients with pemphigus vulgaris (PV) and 19 with pemphigus foliaceus (PF) were treated with systemic or topical corticosteroids at the Nara University Hospital. All 12 patients with PV were treated with oral corticosteroids (initial dose of prednisolone: 15-70 mg/day) and 4 of 12 patients showed prolonged clinical remission (up to 9 years) without corticosteroids. Of 19 patients with PF, 16 patients were treated with oral corticosteroids (initial dose of prednisolone: 8-40 mg/day) and 3 patients were treated with only topical application of corticosteroids. In PF, 7 of 16 patients treated with systemic corticosteroids and all 3 patients treated with topical corticosteroids also showed prolonged clinical remission (up to 10 years). These observations suggest that most of the patients with pemphigus respond well to the treatment of relatively small or moderate dosage of corticosteroids.     

Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab

A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.     

Case of punctate palmoplantar keratoderma type I treated with combination of low‐dose oral acitretin and topical salicylic acid and steroid

Palmoplantar keratodermas (PPK) are heterogeneous disorders characterized by abnormal keratinization. Especially, punctate PPK (PPPK), one of the subtypes of hereditary PPK, is a rare punctate keratoderma characterized by tiny “raindrop” keratoses having a tendency to coalesce on the edge of soles, which are exposed to sustained pressure. If typical punctate lesions are confined to the palms and soles and the patient has a family history and late onset, it can be considered as PPPK type I (PPKP1), also called Buschke–Fisher–Brauer disease. The exact etiology of PPPK has not been fully understood. Furthermore, no standardized treatment for PPPK has been established and treatment options are limited. Above all, traditional systemic retinoids have been used in several cases, but dose‐related adverse effects are common. Therefore, combination of low‐dose systemic retinoids and adjuvant topical therapy can be an alternative treatment option for PPPK. Herein, we report a case of PPKP1 treated with combination of low‐dose oral acitretin (10 mg/day) and topical salicylic acid and steroid. Despite low capacity, low‐dose acitretin showed excellent regression of the lesions by combined use of topical ointments. The supplementary topical therapy may be useful in reducing the dose of systemic retinoids and preventing potential toxicity.     

Clearing of psoriatic erythroderma following heliotherapy in the Dead Sea area

We report herein a patient suffering from psoriatic erythroderma and psoriatic arthritis treated successfully in the Dead Sea area. Sun exposure (heliotherapy) and emollients, without any additional topical or systemic treatments, resulted in clearing of the erythroderma within 4 weeks of treatment. Additional regimens of climatotherapy and balneotherapy, given for another 2 weeks, led to marked alleviation of his arthritic complaints. The remission of skin disease persisted for 5 months without further therapy.     

Studies of blood histamine levels during topical corticosteroid therapy compared with those during systemically administered corticosteroids

Blood histamine levels during topical corticosteroid therapy have not been reported, although decreased blood histamine levels are known to occur after the systemic administration of corticosteroid. Blood histamine levels and serum 11-hydroxycorticosteroids (11-OHCS) after systemic corticosteroid were compared with those after topical corticosteroid. Both blood histamine and serum 11-OHCS levels decreased in a parallel and dose-dependent manner during systemic administration of 0.5-1.0 mg/day of betamethasone. Blood histamine levels increased and serum 11-OHCS levels decreased during systemic administration of 0.25 mg/day to the betamethasone and topical corticosteroid group. Changes in blood histamine levels in the topical corticosteroid group appeared between 0.25 and 0.5 mg/day of systemically administered betamethasone. Fifteen cases of dermatological inpatients suffering from wide-spread eczema or exfoliative dermatitis were treated with the topical corticosteroids betamethasone 17-valerate, budesonide and diflorasone diacetate. In each case, blood histamine levels were examined and an equipotent dose of oral administeration of betamethasone was calculated. Thirteen cases in whom blood histamine levels were measured fell into the dosage range from 0.1 to 0.5 mg/day. In each case, serum 11-OHCS levels were also examined and an equipotent dose of oral administration of betamethasone was calculated by the same method. Fourteen cases in whom serum 11-OHCS levels were measured compared to a dose of less than 1.0 mg/day of betamethasone. Blood histamine levels in rats treated with 20 or 50 mg/kg/day of prednisolone were decreased significantly, and peripheral total leukocytes, lymphocytes, neutrophils and basophils were also decreased significantly.     

Life-threatening erythroderma: diagnosing and treating the "red man"

Exfoliative erythroderma, or diffuse erythema and scaling of the skin, may be the morphologic presentation of a variety of cutaneous and systemic diseases. Establishing the diagnosis of the underlying disease is often difficult and, not uncommonly, erythroderma is classified as idiopathic. Several cases are presented to demonstrate the diversity of presentation of this disease. Laboratory findings are typically unhelpful in establishing the etiology of erythroderma. Clinical data combined with multiple skin biopsies over time are necessary. Systemic complications of erythroderma include infection, fluid and electrolyte imbalances, thermoregulatory disturbance, high output cardiac failure, and acute respiratory distress syndrome. The initial approach to the management of erythroderma of any etiology includes attention to nutrition, fluid and electrolyte replacement, and the institution of gentle local skin care measures. Oatmeal baths and wet dressings to weeping or crusted sites should be followed by application of bland emollients and low-potency topical corticosteroids. Systemic dermatologic therapy may be required to maintain improvement achieved with local measures or to control erythroderma refractory to local measures. The prognosis of erythroderma is dependent on the underlying etiology.     

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.     

Recurrent terbinafine resistant Trichophyton rubrum infection in a child with congenital ichthyosis

Dermatophytosis in children caused by Trichophyton rubrum is preferably treated with topical or systemic terbinafine. We report the first case of terbinafine resistance in a child with recurrent T. rubrum dermatophytosis and congenital ichthyosiform erythroderma.     

A child with CARD14-associated papulosquamous eruption (CAPE) successfully treated with ustekinumab

CARD14-associated papulosquamous eruption (CAPE) is a rare inflammatory skin eruption that can have features of psoriasis, pityriasis rubra pilaris, and erythroderma. This skin condition is known for its resistance to topical or conventional systemic therapies. Successful treatment of CAPE with anti-IL-12/IL-23 and IL-17 inhibitors has been reported. We present a case of a 2-year-old girl with CAPE who was successfully treated with ustekinumab.     

Erythroderma induced by hypercalcitoninemia: two cases

INTRODUCTION: The most common cause of erythroderma is the extension of a previous cutaneous disease to the entire skin surface, as is the case in mycosis fungoides or drug reactions. Erythroderma related to endocrine disorders has only exceptionally been published. We report two cases of patients suffering from erythroderma probably induced by hypercalcitoninemia. OBSERVATIONS: Case 1. A 69 year-old man had a two year-history of fluctuating erythroderma, resistant to all topical or systemic therapy. After numerous unsuccessful investigations, a high serum calcitonin rate was detected. The pentagastrin test was highly positive, suggesting a medullar carcinoma of the thyroid. Thyroidectomy showed only hyperplasia of C cells. All skin signs disappeared within a few days after surgery. Two years later, there was no recurrence of the erythroderma and the serum calcitonin rate was normal. Case 2. A 58 year-old man suffered from congestive and pruriginous erythroderma for 2 1/2 months, which responded well to topical steroids. Biological investigations done for flushes, showed a raised calcitonin level and highly positive pentagastrin test. Thyroidectomy also revealed simple hyperplasia of the C cells. No recurrence of the erythroderma was noted during the 6 month follow-up. DISCUSSION: These are the first cases of erythroderma associated with hypercalcitoninemia. The causal role of calcitonin is probable in the first case, because of 1) long duration of skin signs before the hypercalcitoninemia; 2) quick post-operative recovery, in parallel with the decrease in calcitonin level; 3) absence of recurrence after thyroidectomy; 4) absence of other proven causes. Erythroderma may have resulted from dilation of the skin vessels, since calcitonin and calcitonin gene related peptide are powerful vasodilators.     

Low‐dose naltrexone: a novel treatment for Hailey–Hailey disease

Hailey–Hailey disease (chronic benign familial pemphigus) is a rare inherited dermatosis typically characterized by erosions at intertriginous sites preceded by minor trauma or stress. We report a case of treatment‐resistant Hailey–Hailey disease having failed topical and oral steroids, prophylactic aciclovir and doxycycline, and systemic therapies including dapsone, acitretin and ciclosporin. Low‐dose naltrexone 4·5 mg once daily was commenced following an incidental benefit in this patient's similarly affected sister. The clinical and psychological response to date has been considerable.     

大疱性鱼鳞病及维A酸相关作用研究进展

大疱性鱼鳞病是一种少见的常染色体显性遗传性皮肤病,临床以皮肤过度角化为主要表现,并有3种临床亚型。其发病主要由K1、K10和K2e角蛋白基因突变引起,为常染色体显性遗传。临床试验证实,多数患者在局部或系统使用维A酸后,皮肤状态明显改善。就大疱性鱼鳞病的病因、发病机制和维A酸相关作用机制进行综述。     

Bone mineral density in patients with atopic dermatitis

With the aim of evaluating the systemic effect of glucocorticoids (GCs), we measured bone mineral density (BMD) in 29 adult patients with chronic atopic dermatitis. BMD was measured in the lumbar spine and in the left femoral neck using dual–energy X–ray absorptiometry (DEXA). In the right calcaneus. BMD was measured using broad–spectrum ultrasound attenuation (BUA). For BMD, the patients with dermatitis did not differ from healthy controls. We did not find any statistically significant correlation between BMD and single risk factors, such as the barrier function of the skin (P = 0.08), the duration of the dermatitis (P = 0.58), and the use of oral GCs (P = 0.27) and potent topical GCs (P = 0.10). However, selected patients with severe disease, needing topical GCs of higher potency than hydrocortisone (HC), had lower values for lumbar BMD than the patients who had not used these preparations (–1.0 vs. +0.1 SD; P = 0.026). The lower lumbar BMD in this group could be explained by a long-term systemic effect of GCs.     

Long‐term safety and efficacy of continuous acitretin monotherapy for three children with different severe hyperkeratotic disorders in China

Long‐term systemic treatment with acitretin for severe hyperkeratotic disorders is needed to maintain quality of life of afflicted patients, but treatment has been limited owing to its potential side‐effects including skeletal malformations, particularly for children during their growth and development. A retrospective investigation was conducted with three children afflicted with a severe hyperkeratotic disorder, namely Darier's disease, bullous ichthyosiform erythroderma or lamellar ichthyosis, who were continuously maintained on 0.2–0.3 mg/kg per day acitretin for more than 12 years after an initial period at a larger acitretin dose to bring each disease under control. The patients had good responses to acitretin treatment, which was assessed for safety, skeletal abnormalities, growth retardation and other potential side‐effects. Acitretin monotherapy was an effective treatment for these children, and maintenance doses were well tolerated with no skeletal or other observable side‐effects during the course of the study.     

Generalized pustular psoriasis in a child: observation of long-term combination therapy with etretinate and calcipotriol for 16 years

Generalized pustular psoriasis (GPP) is a rare condition in young children. It is difficult to treat and may require long-term systemic therapy. We report the long-term course of a 3-year-old boy whose onset of psoriasis dated to age 7 months. He was treated with etretinate and psoralen plus ultraviolet A therapy initially and then with etretinate alone, and at age 12, topical calcipotriol was added. At the age of 19, he had been taking oral retinoids for 16 years, with a mean dose of etretinate of 0.22 mg/kg per day, a total amount of approximately 37 g, without evidence of stunted growth, ligamentous calcification, hyperostosis, or hepatic toxicity.     

Analysis of psoriatic patients registered in Asahikawa Medical College Hospital from 1983 to 2007

Psoriasis is a chronic inflammatory skin disease, which has been increasing during the last 50 years in Japan. The aim of the present study is to analyze psoriatic patients registered from 1983–2007 in Asahikawa Medical College Hospital, which is located in the northern part of Japan. A total of 607 cases were registered at the first inspection in the Department of Dermatology, Asahikawa Medical College. Men (403 cases, 66.4%) were predominant over women (204 cases, 33.6%). The clinical types of psoriasis were psoriasis vulgaris (91.5%), guttate psoriasis (4.2%), psoriasis arthropathica (2.8%), psoriatic erythroderma (0.6%), generalized pustular psoriasis (0.6%), localized pustular psoriasis (0.15%) and infantile psoriasis (0.15%). Topical corticosteroids (78.1%) and vitamin D3 (18.1%) products were the main previous topical agents. Previous systemic treatments included etretinate (7.7%), cyclosporine (1.5%) and methotrexate (0.3%). Use of topical vitamin D3 and cyclosporine therapies have been gradually increasing during the past 25 years. Regarding the previous phototherapy, topical psoralen and ultraviolet A therapy (PUVA) (4.9%) was predominant over ultraviolet B (0.9%), and systemic PUVA (0.7%). Use of ultraviolet B phototherapy has been increasing during the past 5 years. The results are essentially similar to those of a survey of psoriasis in Japan from 1982–2001. Although the incidence of psoriasis might be higher in Hokkaido Prefecture, there is essentially no variation in the disease profile of psoriatic patients.     

Epidermolytic hyperkeratosis

A 13-year-old boy presented to the dermatology clinic for treatment of a congenital ichthyosis with a history of generalized erythroderma and trauma related blistering at the time of birth. At the time of presentation he was noted to have red corrugated hyperkeratotic plaques involving the joint flexures, dorsal hands, and neck. Epidermolytic hyperkeratosis is a rare autosomal dominant genodermatosis that presents at birth with generalized erythema, blisters and erosions. In the subsequent months after birth erythema and blistering improves but patients go on to develop hyperkeratotic scaling that is especially prominent along the joint flexures, neck, hands and feet. The disease is caused by mutations in either keratin 1 or keratin 10. Treatment options include urea or alpha-hydroxy acid containing creams as well as topical and systemic retinoids. Epidermolytic hyperkeratosis is also known as bullous congenital ichthyosiform erythroderma (of Brocq) and disorder of cornification type 3.     

Erythroderma: an unusual presentation of pulmonary tuberculosis

We report an elderly Chinese man who presented with erythroderma. He was treated with topical corticosteroids and emollients, but did not improve. Chest X-ray revealed cavitation in the right upper pulmonary lobe, and laryngeal swab culture was positive for Mycobacterium tuberculosis complex. Polymerase chain reaction performed on a skin biopsy was positive for M. tuberculosis DNA. Within a month of starting antituberculous medication, his erythroderma had almost completely resolved. Pulmonary tuberculosis should be included in the list of differential diagnosis for erythroderma.