isotretinoin and Syndrome

The association of systemic sarcoidosis and malignant lymphoma is known as the 'sarcoidosis-lymphoma syndrome'. Cutaneous involvement is rare in this syndrome. We report a 52-year-old woman who was diagnosed as having tumour-stage mycosis fungoides. Complete remission was achieved by combination therapy consisting of isotretinoin, interferon (IFN) alpha, electron beam irradiation, photochemotherapy and topical corticosteroids. Three years later, the patient developed systemic sarcoidosis characterized by yellowish papules on the abdominal wall and the eyelids that histologically revealed non-caseating granulomas, multiple fine-nodular interstitial pulmonary infiltrates on chest X-ray, hilar lymphadenopathy, decreased vital capacity and increased lymphocyte count in bronchoalveloar lavage fluid. As opposed to most of the reported cases, in our patient the manifestation of cutaneous lymphoma preceded the diagnosis of systemic sarcoidosis. We review the cases reported in the literature and discuss a possible causal and temporal relationship as well as the role of IFN alpha in the development of sarcoidosis.

Topics: Anti-Inflammatory Agents; Combined Modality Therapy; Female; Humans; Interferon-alpha; Isotretinoin; Keratolytic Agents; Methylprednisolone; Middle Aged; Mycosis Fungoides; Phototherapy; Sarcoidosis; Skin Neoplasms; Syndrome

We report on a 63-year-old female patient with Muir-Torre syndrome (MTS). In the course of this disease two carcinomas of the colon, a kerato-acanthoma and multiple sebaceous gland tumours, including four sebaceous carcinomas, appeared. This case is thought to be a hereditary form as one of daughters was also found to have a sebaceous epithelioma. MTS is a mostly autosomal-dominant disease with the association of sebaceous gland tumours and internal carcinomas. As the malignant tumours only show slight aggressiveness the prognosis is quite favourable. Oral isotretinoin therapy was successfully used for the inhibition of sebaceous gland proliferation. A narrower definition is presented and an updated survey of the published cases is given. Furthermore, the histopathologic peculiarities of sebaceous gland tumours, especially of sebaceous gland carcinomas, are discussed and compared to sebaceous gland tumours not connected with MTS. A total number of 100 of the 135 published cases of MTS were included and analysed regarding sebaceous gland tumours and other skin tumours. The data on internal carcinomas were taken from the work of Cohen et al. (1991) and 11 current cases were added.

Topics: Adenocarcinoma; Adenoma, Sweat Gland; Adenomatous Polyposis Coli; Administration, Oral; Carcinoma; Carcinoma, Squamous Cell; Chromosome Aberrations; Chromosome Disorders; Diagnosis, Differential; Facial Neoplasms; Female; Genes, Dominant; Humans; Isotretinoin; Keratoacanthoma; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Neoplastic Syndromes, Hereditary; Sebaceous Gland Neoplasms; Sweat Glands; Syndrome

The acne conglobata (AC)-, acne fulminans (AF)-, and isotretinoin-associated musculoskeletal syndromes are three distinct clinical entities. The AC-associated musculoskeletal syndrome occurs primarily in black men over the age of 22, who develop sacroilitis with or without a peripheral arthropathy. In contrast, the AF-associated musculoskeletal syndrome is found almost exclusively in white male teenagers. Fever, weight loss, and arthralgias are prominent components of this syndrome. A unique feature of the AF-associated musculoskeletal syndrome is osteolytic lesions that occur most frequently in the clavicle, sternum, long bones, and ilium. The isotretinoin-associated musculoskeletal syndrome occurs with equal frequency in male and female acne patients. Mild, transient myalgias and arthralgias are very common and do not require discontinuation of isotretinoin therapy. Asymptomatic, small, hyperostotic lesions of the spine occur in approximately 10% of acne patients with the isotretinoin-associated musculoskeletal syndrome.

Topics: Acne Vulgaris; Adolescent; Adult; Bone Diseases; Female; Humans; Isotretinoin; Male; Middle Aged; Muscular Diseases; Syndrome

Morbihan disease (MD), also known as Morbihan syndrome, "solid persistent facial edema and erythema", "rosacea lymphedema", and "solid facial edema in acne", is a rare and often unrecognizable entity, that presents with a slow occurrence of persistent lymphoedema of the upper two-thirds of the face (1,2). A 30-year-old woman presented to our Department with persistent, asymptomatic face edema and erythema lasting for 18 months. She was previously treated for rosacea with doxycycline (100 mg/day for four months) without improvement. Dermatological examination revealed erythematous, nonpitting, solid edema located on the mid-forehead, nose, and cheeks with sparse erythematous papules and pustules on the entire face including the chin and comedones, papules, and pustules on the back (Figure 1 and Figure 2). She was otherwise healthy and was not taking any medication. Laboratory tests with immunological tests and Quantiferon test together with MRI of the orbits, chest X-ray, chest high-resolution computed tomography, cranial X-ray, and abdominal ultrasound were all within normal limits. Histopathology revealed dermal edema, perivascular and peri-adnexal lymphohistiocytic infiltrate, and sebaceous gland hyperplasia. Based on the typical clinical picture, histopathological findings, and the exclusion of several differentials the diagnosis of MD was established. The patient was treated with oral isotretinoin (20 mg/day for eight months) without regression of solid edema and erythema on the face but with complete regression of acne on the trunk. She was started on oral corticosteroids (prednisolone, 20 mg/day for two months followed by reduction of the dose over three months), again with only slight short transient improvement and rapid relapse of facial erythema and edema. The patient refused any other suggested treatment. We treated our patient for a total 2 years and followed up for 5 years. The pathogenesis of MD is still unknown. It is considered a clinical variety or a complication of rosacea or acne which does not tend to regress spontaneously. It is believed that chronic inflammation in patients with MD is due to acne or rosacea causing structural damage to blood and lymph vessels (1,3,4). However, cases of MD without previous history of rosacea and acne have been reported supporting the distinct disease theory (3,4). Edema and erythema are localized on the upper half of the face affecting the forehead, glabella, eyelids, nose, and cheeks. Although the sy

Topics: Administration, Oral; Adrenal Cortex Hormones; Adult; Angioedema; Diagnosis, Differential; Erythema; Face; Female; Humans; Isotretinoin; Syndrome

Topics: Dermatologic Agents; Drainage; Edema; Facial Dermatoses; Humans; Isotretinoin; Male; Syndrome; Young Adult

Unilateral calf swelling and pain is not a common complaint in the pediatric emergency department. We present a case of a 17-year-old adolescent boy with no past medical history who presented with left leg swelling and pain while taking prednisone and isotretinoin. He was found to have an extensive occlusive thrombus throughout the deep venous system in his left leg. He was later diagnosed with May-Thurner syndrome, an anatomic variant in which the right iliac artery compresses the left iliac vein. We review the differential diagnosis, diagnostic workup, and initial ED management of deep venous thrombosis and provide a brief discussion of May-Thurner syndrome and the association of isotretinoin and vascular thrombi.

Topics: Adolescent; Angioplasty; Anticoagulants; Emergency Service, Hospital; Follow-Up Studies; Heparin; Humans; Iliac Artery; Iliac Vein; Isotretinoin; Male; Peripheral Arterial Disease; Phlebography; Pulmonary Embolism; Risk Assessment; Stents; Steroids; Syndrome; Vascular Malformations; Venous Thrombosis

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a clinically heterogeneous entity, encompassing a variety of debilitating conditions that have in common inflammation of the skeletal system and skin. To date, there is a paucity of documented efficacious treatment options. We report a 48-year-old man with skeletal and cutaneous signs and symptoms who improved dramatically after treatment with a combination of isotretinoin and pamidronate. This report provides an alternative treatment regimen for SAPHO that addresses the possible underlying pathophysiology of this likely underdiagnosed syndrome.

Topics: Acneiform Eruptions; Diphosphonates; Drug Therapy, Combination; Humans; Hyperostosis; Isotretinoin; Male; Middle Aged; Osteitis; Pamidronate; Psoriasis; Skin Diseases; Syndrome; Synovitis

Topics: Abnormalities, Multiple; Adolescent; Hair Diseases; Humans; Isotretinoin; Male; Skin Diseases; Syndrome; Treatment Outcome

Acne can be associated with severe musculoskeletal and constitutional symptoms. This is a case history of a 15 year-old boy with severe acne, treated with isotretinoin, who was admitted because of high fever and weight loss of 3 weeks duration. His complaints were of severe chest and back pain, and inability to walk. His laboratory results revealed elevated erythrocyte sedimentation rate and C-reactive protein. On a pelvic radiograph bilateral sacroiliitis was noticed. Radionuclide bone scan revealed increased uptake in the right sacroiliac joint, in small vertebral bodies and sternum. A diagnosis of acne-associated musculoskeletal syndrome was made and the patient was treated with high dose steroids. His symptoms resolved and his acne improved. This case report sheds light on the relationship between acne and associated musculoskeletal syndromes. Physicians should be aware of this association in order to provide prompt and efficient treatment.

Topics: Acne Vulgaris; Adolescent; Blood Sedimentation; C-Reactive Protein; Dermatologic Agents; Humans; Isotretinoin; Male; Musculoskeletal Diseases; Syndrome

Topics: Acanthosis Nigricans; Acne Vulgaris; Adult; Dermatologic Agents; Female; Humans; Hyperandrogenism; Insulin Resistance; Isotretinoin; Pyoderma Gangrenosum; Syndrome

Muir-Torre syndrome is a genodermatosis in which multiple internal malignancies are associated with cutaneous sebaceous tumours and kerato-acanthomas. A 57-year-old man presented with multiple sebaceous tumours, kerato-acanthomas, verrucous carcinoma of the nose, renal cell and transitional cell carcinomas of the left kidney, adenoma of the colon and a positive family history of colon carcinoma. He was treated with interferon (IFN-alpha2a) s.c. 3 x 10(6) U three times a week along with 50 mg isotretinoin daily as well as topical isotretinoin gel. During a follow-up of 29 months, only 1 sebaceous skin tumour developed and was removed, whereas more than 30 such skin tumours had been surgically removed during the last 3 years. No evidence of internal tumour development or recurrence was found. The combination of IFN with retinoids seems to be of promise to prevent tumour development in Muir-Torre syndrome.

Topics: Antineoplastic Combined Chemotherapy Protocols; Humans; Interferon alpha-2; Interferon-alpha; Isotretinoin; Male; Middle Aged; Neoplasms, Multiple Primary; Recombinant Proteins; Sebaceous Gland Neoplasms; Skin; Syndrome

Topics: Adult; Facial Dermatoses; Follow-Up Studies; Humans; Hyperplasia; Isotretinoin; Keratolytic Agents; Laser Therapy; Male; Neoplasms, Multiple Primary; Recurrence; Retreatment; Sebaceous Gland Neoplasms; Sebaceous Glands; Syndrome; Telangiectasis

Topics: Acne Vulgaris; Animals; Dog Diseases; Dogs; Female; Hair Follicle; Isotretinoin; Keratolytic Agents; Prognosis; Skin; Staphylococcus; Syndrome

The teratogenic potential of 13-cis retinoic acid (13-cis RA) was further assessed in cynomolgus macaques (Macaca fascicularis) following exposure to two different regimens during the preorganogenic period to determine the influence of time of treatment on the 13-cis RA malformation syndrome established previously (Hummler et al., Teratology, 42:263-272, 1990). In experiment (Exp) 1, 13-cis RA was orally administered once daily (2.5 mg/kg) on gestational day (GD) 16-25 and twice daily (2 x 2.5 mg/kg) on GD 26-27. In Exp 2, the same oral dose was administered once daily on GD 10-20 and twice daily on GD 21-24. Although the ear was affected at about the same incidence (6/9, 67%) in Exp 1 as previously reported, the defects were less severe. Except for hypoplastic vermis of the cerebellum (2/9, 22%), no other defects were seen. In Exp 2, the teratogenic effects of 13-cis RA on the craniofacial skeleton (1/9, 11%), external ear (5/9, 56%), and heart (2/9, 22%) were similar to that reported by Hummler et al.; however, no thymus or cerebellar malformations were observed. Analysis of the three different treatments (GD 16-27 in Exp 1, GD 10-24 in Exp 2, and GD 10-27 in Hummler et al.) suggests that the sensitive periods in the macaque are 1) craniofacial skeleton and heart GD 10-24, 2) thymus and cerebellum > GD 24, and 3) external ear GD 16-24, although the defects are less severe following the shorter exposure.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Abnormalities, Drug-Induced; Animals; Dose-Response Relationship, Drug; Ear, External; Female; Gestational Age; Humans; Isotretinoin; Macaca fascicularis; Maternal-Fetal Exchange; Pregnancy; Species Specificity; Syndrome

Topics: Acne Vulgaris; Acrocephalosyndactylia; Adult; Humans; Isotretinoin; Male; Syndrome

Abnormalities of the secondary palate were studied in an animal model in which features of Treacher Collins syndrome (TCS) and Nager or Miller syndromes (both of which are facially similar to Treacher Collins, but include limb malformations) were induced by acute maternal exposure to 13-cis-retinoic acid (13-cis-RA, isotretinoin, Accutane). Previous work in our laboratory has illustrated that excessive cell death in the proximal aspect of the maxillary and mandibular prominences of the first visceral arch and in the apical ectodermal ridge of the limb bud probably accounts for the characteristic craniofacial and limb abnormalities observed (Sulik et al, 1987; Sulik and Dehart, 1988). The current study shows that maternal treatment with 400 mg per kilogram 13-cis-RA at 8 days 14 hours (8d14hr) or 9d6hr post fertilization results in abnormalities of the secondary palate that vary in incidence and severity. Following the earlier treatment time, 82 percent (68 of 74) of the 18d fetuses were affected, with, severely hypoplastic, unfused palatal shelves present in 34 percent (25 of 74). The less severely affected fetuses had malformations that involved primarily the posterior aspect of the palatal shelves. This malformation (foreshortening of the posterior portion of the palate) constituted the major developmental alteration that resulted from treatment at the later time, at which time a 52 percent (26 of 50) malformation incidence was seen. The change in pattern of malformations with treatment time is consistent with the changing pattern of programmed cell death, which was observed to occur in the first visceral arch.

Topics: Animals; Branchial Region; Cell Survival; Cleft Palate; Female; Isotretinoin; Limb Deformities, Congenital; Mandibulofacial Dysostosis; Maxilla; Mice; Mice, Inbred C57BL; Mice, Inbred Strains; Morphogenesis; Palate; Pregnancy; Syndrome; Time Factors

Topics: Abnormalities, Multiple; Humans; Infant; Isotretinoin; Phenotype; Syndrome; Tretinoin

Topics: Abnormalities, Multiple; Brain; Brain Diseases; Humans; Isotretinoin; Syndrome; Tomography, X-Ray Computed; Tretinoin

Topics: Adult; Cornea; Female; Hearing Loss, Sensorineural; Humans; Ichthyosis; Isotretinoin; Keratitis; Neovascularization, Pathologic; Syndrome; Tretinoin

Two representative cases of familial Muir-Torre syndrome are presented. Multiple benign sebaceous neoplasms in both cases and a solitary keratoacanthoma in one were successfully treated with oral isotretinoin. Low-dose maintenance therapy has stabilized the cutaneous manifestations in the two patients, and no new epithelial neoplasms have appeared. This report emphasizes (1) the rationale for the use of isotretinoin in the Muir-Torre syndrome and (2) the potential for a familial pattern of inheritance and a possible association with the cancer family syndrome. It speculates on the prevention of future internal malignancies in Muir-Torre syndrome patients by maintenance oral isotretinoin treatment.

Topics: Administration, Oral; Female; Humans; Isotretinoin; Keratoacanthoma; Middle Aged; Neoplasms, Multiple Primary; Sebaceous Gland Neoplasms; Syndrome; Tretinoin

The neutrophil function and plasma leukotactic activity of a patient with Sweet's syndrome and cystonodular acne were evaluated during a 2 1/2-year period. These studies demonstrated that chemotaxis was frequently slightly increased, especially during an exacerbation of Sweet's syndrome, but showed some decrease during isotretinoin therapy. Other functions, such as phagocytosis, metabolic activation, and bacterial killing, also were slightly increased. In addition, the patient's serum contained a heat-stable, nonlipid chemoattractant that was present at all times except during a course of isotretinoin. Although his symptoms responded to aspirin, the plasma continued to show this chemoattractant. These findings are consistent with the hypothesis that excess chemoattractant in Sweet's syndrome attracts neutrophils, which then mediate an inflammatory response. In addition, aspirin may be used to control Sweet's syndrome symptoms, although it does not suppress the plasma chemoattractant.

Topics: Adult; Aspirin; Chemotaxis, Leukocyte; Dialysis; Hot Temperature; Humans; Isotretinoin; Leukocytosis; Male; N-Formylmethionine Leucyl-Phenylalanine; Neutrophils; Pentose Phosphate Pathway; Phagocytosis; Plasma; Skin Diseases; Syndrome; Tretinoin; Zymosan

Topics: Abnormalities, Drug-Induced; Brain; Ear; Heart Defects, Congenital; Humans; Isotretinoin; Syndrome; Teratogens; Tretinoin

Topics: Child; Humans; Isotretinoin; Male; Skin Diseases; Syndrome; Tretinoin