Acquired ichthyosis: a clinical review.

Acquired ichthyosis (AI) is a rare, nonhereditary cutaneous disorder that has been associated with numerous neoplastic, infectious, drugs, endocrine, metabolic, autoimmune, and malabsorptive diseases. Review all demographical, clinical, histological, and therapeutic features of AI and focus on all reported associated diseases. We performed a systematic literature review in Pubmed/Medline, Embase, and Cochrane collaboration databases, searching for all articles on AI, with no limits on publication date, participant age, sex or nationality. Eighty-four articles were included. Total number of included patients was 167 patients with a mean age at presentation of 39 years [range 0.5-85] and a sex ratio M:F of 5:2. The most common malignancy associated with AI is Hodgkin's lymphoma. AI occurred before, simultaneously or after the onset of malignancy or systemic disease. The severity of AI depends on the severity of the underlying disorder and regresses once the disease goes into remission and may also be a marker of disease recurrence or relapse. 8% have been reported to be drug related and all occurred weeks to months after drug intake and resolved after stopping or decreasing the dose of the drug. Data were derived from case reports and observational studies. Limitations include the accuracy of published data, potential patient selection, and reporting bias. AI can be associated with numerous systemic diseases and drugs. Physicians should be particularly alert to these associations to provide adequate screening and management of patients with AI.

Triple retinal arterial macroaneurysms in a hypertensive patient with hypothyroidism.

PURPOSE: To present the unique case of numerous, recurring retinal arterial macroaneurysms (RAMs) in a hypothyroid patient with hypertension. METHODS: 67-year-old woman's clinical findings, laboratory results, fundus fluorescein angiography, optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) are given. Over a two-year period, the patient was monitored. RESULTS: A 67-year-old woman presented to the outpatient clinic with vitreous and dense subretinal hemorrhages in her right eye. RAM rupture was discovered along the suprotemporal retinal arteriole. A diagnosis of systemic arterial hypertension was made. Two months later, the vitreous hemorrhage spontaneously resolved and the patient's vision improved. After nine months of initial presentation, the patient developed another RAM. Meanwhile the patient developed ichthyosis caused by hypothyroidism. Because fundus fluorescein angiography revealed that the first RAM was still active, an intravitreal injection of anti-VEGF was administered six months afterwards. More proximal RAM with macular edema developed after another six months necessitating laser photocoagulation. However macular edema didn't resolve and a second injection of intravitreal anti-VEGF was given. CONCLUSIONS: Patients with RAMs, particularly if multiple or recurring, should be thoroughly investigated and assessed, particularly for secondary causes of hypertension. OCT and OCTA are useful tools for RAM confirmation and follow-up. It is important to look into how RAM behavior interacts with coexisting macular edema, and other variables affecting hemodynamic status.

Seborrheic dermatitis with massive facial hyperkeratosis resembling acquired ichthyosis.

Seborrheic dermatitis is a common multifactorial skin disorder favoring the scalp, ears, face, and central chest. We present a case of an elderly woman with new-onset severe seborrheic dermatitis with massive facial hyperkeratosis mimicking ichthyosis. Clinicians should be aware of rare presentations of common conditions and should screen for associated medical comorbidities in new-onset severe skin conditions.

[Detecting skin cancer: paraneoplastic skin diseases]. / Tumorerkrankungen an der Haut erkennen ­ paraneoplastische Hauterkrankungen.

Skin diseases represent the second most common paraneoplastic manifestations after endocrine disorders. Paraneoplastic dermatoses may arise as the first clinical sign of occult malignancies, or manifest during the course of a previously diagnosed neoplasm. Dermatoses belonging to the group of obligate paraneoplastic syndromes manifest only in the presence of underlying malignancies. Other skin diseases may be associated with malignancy in a subset of patients.

Acquired Ichthyosis in the Setting of Active Pulmonary Tuberculosis.

Acquired ichthyosis is an uncommon disorder of cornification. It characteristically presents as symmetric scaling of the skin on the trunk and extensor surfaces of the extremities. It is clinically and histologically similar to ichthyosis vulgaris; however, acquired ichthyosis develops later in life and has been associated with various malignancies, infections, medications, autoimmune diseases, metabolic disorders, and malnutrition. We describe a case of a 35-year-old woman with active pulmonary tuberculosis and a history of breast cancer who presented with a several-month history of a widespread, scaly, pruritic skin eruption. Physical examination revealed fine, scaly patches on the extremities with relative sparing of the flexures and larger, scaly, ichthyosiform patches on the chest and back. Skin biopsy revealed orthokeratotic hyperkeratosis and a diminished granular layer, consistent with a diagnosis of acquired ichthyosis. Further evaluation, including positron-emission tomography/computed tomography scan, revealed hypermetabolic infiltrates and cavitation in the lungs, consistent with active pulmonary tuberculosis; there was no evidence of new or recurrent malignancy. The patient was treated with antituberculosis drugs and topical ammonium lactate cream. With incident cases rarely reported in the literature, this case of new-onset ichthyosis in the setting of active pulmonary tuberculosis highlights the distinctive clinical and histologic features of acquired ichthyosis and emphasizes the relationship of acquired ichthyosis with underlying systemic disease, particularly infection.

Porphyria Cutanea Tarda Presenting with Scleroderma, Ichthyosis, Alopecia, and Vitiligo.

Porphyria cutanea tarda (PCT) is a cutaneous porphyria that presents later in life with cutaneous findings in sun-exposed sites. We report a complex case of PCT in a 67-year-old woman with an unusual constellation of cutaneous findings: scleroderma, acquired ichthyosis, and nonscarring alopecia. Possible triggers for her PCT include tamoxifen treatment for breast cancer and carrier status of the hemochromatosis gene. High-dose chloroquine was used to successfully achieve clinical remission and normalize her uroporphyrins. While on chloroquine she developed extensive classic vitiligo. It is not clear if this is another feature of her complex and unusual PCT, or a consequence of her antimalarial therapy.

Successful Treatment of Primary Cutaneous Peripheral T-Cell Lymphoma Presenting Acquired Ichthyosis with Oral Bexarotene Monotherapy.

Acquired ichthyosis (AI) is a reactive cutaneous manifestation that can be associated with malignant hematological disease, including cutaneous T-cell lymphoma (CTCL). Since it is difficult to distinguish AI from ichthyosiform mycosis fungoides, to select the treatment for CTCL with ichthyosis-like appearance and to evaluate its efficacy is sometimes challenging. In this report, we describe a case of primary cutaneous peripheral T-cell lymphoma not otherwise specified presenting AI successfully treated with oral bexarotene. In the present case, the administration of oral bexarotene was not only effective for lymphoma cells infiltrating ulcers and nodules, but it also eliminated AI.

Acquired ichthyosis triggered by an osseous hemangiopericytoma: a case report and review of the literature.

Ichthyoses are a heterogeneous group of cutaneous keratinization disorders that can be congenital or acquired. Apart from neoplastic disorders, the acquired form of ichthyosis (AI) has been associated with a variety of diseases including infections, autoimmune/inflammatory and endocrine/metabolic diseases as well as nutritional conditions, medications and others. However, malignancy accounts for half of the reported cases, most commonly including lymphoproliferative disorders. We present a case of AI as a paraneoplastic skin manifestation of a primary, osseous hemangiopericytoma (HP) accompanied by multiple liver metastases. We also review the literature and discuss the necessity of investigating underlying diseases, especially malignancy, when adult-onset ichthyosis arises.

[Dermatomyositis and acute interstitial lung disease associated with MDA-5 antibodies: an atypical case]. / Dermatomyosite et pneumopathie interstitielle rapidement évolutive associées aux auto-anticorps anti-MDA-5 : une présentation clinique atypique.

BACKGROUND: A new antibody known as anti-MDA-5 has recently been identified in association with a specific phenotype of dermatomyositis (DM), in which muscular involvement is minimal or absent, but where there is a particularly high risk of onset of aggressive interstitial lung disease. Below, we report a case associated with the presence of anti-MDA-5 antibodies complicated by aggressive interstitial lung disease ; the cutaneous phenotype of this patient is novel due to the constitution of diffuse ichthyosis and of profuse subcutaneous calcinosis. PATIENTS AND METHODS: A 35-year-old man was hospitalised for a skin rash associated with arthralgia, dry cough, asthenia and weight-loss of 8 kg in 5 months. The dermatological examination revealed purple erythema of the upper eyelids and cheeks, diffuse acquired ichthyosis of the trunk and limbs, and striated erythema on the proximal and distal metacarpal and interphalangeal joints of the fingers. A diagnosis of DM was suspected in patients treated with prednisolone, 1.5 mg/kg/d. Immunological tests identified the presence of anti-MDA-5 antibodies. One month later, the cutaneous signs continued to progress and were marked by the development of painful ulcers on the back of the fingers, as well as dyspnoea accompanying the slightest effort. Chest images showed constituted pulmonary fibrosis lesions. At the same time, within several months, diffuse, pseudo-tumoural calcifications developed in the subcutaneous tissue of the trunk and limbs. The patient was treated successively with intravenous gammaglobulins, cyclophosphamide, mycophenolate mofetil, azathioprine and rituximab in combination with oral corticosteroids, but with no improvement in respiratory function or in the skin lesions. Because of the decline in the patient's respiratory status, a lung graft was envisaged but subsequently abandoned because of the patient's excessively precarious state of health. DISCUSSION: Anti-MDA-5 antibody appears to constitute a specific immunological marker for a special dermatological phenotype of adult DM, significantly associated with the presence of: 1) keratotic papules in the skin folds of the palms and fingers ; 2) cutaneous ulcers along the periungueal edges, Gottron's papules on the back of the hands and on the extensor surface of the elbows and knees ; 3) pain and ulceration in the oral mucosa, diffuse alopecia, and appearance of "mechanic's hands" and Gottron's sign on the elbows and knees. To our knowledge, the presence of extensive ichthyosis and profuse subcutaneous calcinosis has not been previously reported in this particular form of DM. The risk of aggressive interstitial lung disease is particularly high and worsens the prognosis, since 40% of patients with anti-MDA-5 die, usually within the first year. CONCLUSION: Herein, we describe a patient presenting amyopathic DM complicated by aggressive interstitial lung disease associated with the presence of anti-MDA-5 antibodies, but with a hitherto unreported cutaneous phenotype combining diffuse ichthyosis and profuse subcutaneous calcinosis.

Acquired ichthyosis associated with type 1 diabetes mellitus.

Acquired ichthyosis is an uncommon disease which is characterized by symmetric scaling of the skin. Acquired ichthyosis has been described in association with a variety of underlying causes, including malignancies, drugs, infections, endocrine, metabolic and autoimmune diseases. Acquired ichthyosis associated with diabetes mellitus has been reported only in one case. We report the case of a new-onset diabetes mellitus with a one-month history of generalized acquired ichthyosis and palmoplantar keratoderma corroborated with skin biopsy, which completely disappeared after regulation of blood glucose levels with insulin therapy.