Case of autoimmune progesterone dermatitis presenting as necrotic migratory erythema successfully controlled by danazol

Autoimmune progesterone dermatitis (APD) is a rare cutaneous disorder with cyclic skin eruptions during the luteal phase of the menstrual cycle. Patients can present with various clinical manifestations, including urticaria and angioedema, erythema multiforme, eczema, fixed drug eruption and centrifugal erythema annulare. In our case, however, the patient’s skin lesions mimic necrotic migratory erythema (NME) which is most commonly associated with glucagonoma and rarely with liver disease, inflammatory bowel disease, malnutrition and other tumors. To our knowledge, this is the first case of NME-like APD and is successfully controlled by danazol. This also sheds lights on the etiologic diversity of NME.     

Autoimmune progesterone dermatitis with persistent amenorrhoea

A case of autoimmune progesterone dermatitis is reported. The patient developed a recurrent eruption, primarily on the extremities, after receiving oral progesterone for the treatment of persistent amenorrhoea. Intradermal injection of 17 alpha-hydroxyprogesterone produced a positive skin reaction after 30 min, but no delayed onset reaction was observed. A patch test with progesterone in petrolatum was negative. The lymphocyte transformation test was normal. Histamine release from passively sensitized peripheral blood leukocytes was increased by progesterone preincubated in normal serum as a stimulating antigen. Conjugated oestrogen therapy suppressed the rash. Cyclical eruptions with elevated basal body temperature persisted for more than 20 months without menstruation.     

Acquired perforating dermatosis associated with primary biliary cirrhosis and hashimoto thyroiditis

Acquired perforating dermatosis (APD) is an uncommon skin eruption of unclear etiology that most often is associated with diabetes mellitus or chronic renal insufficiency. There are rare reports of APD in association with liver disease or thyroid disease. We report a case of APD in a patient with both primary biliary cirrhosis and Hashimoto thyroiditis in the absence of diabetes mellitus and chronic renal insufficiency. The patient had a partial response to narrowband UVB phototherapy.     

Autoimmune progesterone dermatitis: Case report of an unexpected treatment reaction

Autoimmune progesterone dermatitis (APD) is a rare skin condition with a varying morphology, which appears on a monthly basis during the luteal phase of the menstrual cycle and resolves spontaneously with the endogenous decrease in progesterone during menses. We present the case of 39‐year‐old multiparous Caucasian woman with generalised, self‐limited urticaria in her perimenstrual period. APD was diagnosed in light of the cyclical nature of the symptoms. An intradermal test with the administration of 0.5 mg/mL of medroxyprogesterone acetate showed a positive result. She started using vaginal hormonal contraceptive that paradoxically exacerbate the symptoms, which ceased after the removal of the device. An oral combined contraceptive was initiated instead, with complete resolution of the symptoms. We share the case because of the rarity of the situation, emphasising the importance of a multidisciplinary team for differential diagnosis and patient follow up.     

A premenstrual urticarial eruption treated with bilateral oophorectomy and hysterectomy

Recurrent episodes of urticaria and erythema multiforme which occur in the luteal phase of each menstrual cycle are well recognized, albeit rare. This clinical entity was described by Hart (1977)¹ and attributed to a hypersensitivity to endogenous progesterone. We report a patient with a cyclical urticarial eruption which commenced during pregnancy and recurred pre-menstrually over a period of 11 years. Her symptoms were controlled by suppressing ovulation and finally by bilateral oophorectomy and hysterectomy. Post-operative challenge with an oral oestrogen resulted in a prompt recurrence of the eruption, whereas the administration of a progesterone had no effect. We suggest that this premenstrual eruption was oestrogen-rattier than progesterone-dependent.     

Recurrent erythema multiforme triggered by progesterone sensitivity

Determining the underlying etiology of recurrent erythema multiforme (EM) can be a difficult endeavor. Although infection with herpes simplex virus (HSV) has been implicated in some cases, the precise trigger of a given patient's recurrent EM often remains elusive. We discuss the case of a woman with a recurrent blistering eruption that was clinically and histopathologically consistent with EM. An investigation into the etiology of the patient's EM suggested that HSV was not the causative factor but instead pointed toward a hormonal influence that we interpret as autoimmune progesterone dermatitis (APD). This case is presented to highlight the importance of considering hormonal triggers in women with recurrent EM that consistently flares during the luteal phase of the menstrual cycle, the point at which serum progesterone levels peak. A brief review of the literature regarding the diagnosis, histopathology, etiology and treatment of APD is further provided. Nasabzadeh TJ, Stefanato CM, Doole JE, Radfar A, Bhawan J, Venna S. Recurrent erythema multiforme triggered by progesterone sensitivity.     

Detection of anti-type VII collagen antibody in Sjögren's syndrome/lupus erythematosus overlap syndrome with transient bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus (SLE) is a chronic, widespread, non-scarring, subepidermal blistering eruption associated with autoimmunity to type VII collagen. We describe a patient with Sjögren's syndrome/lupus erythematosus overlap syndrome who showed transient blistering eruptions over limited skin surface and in oral mucosa. At the time of aggravation, the patient's serum contained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl-split normal skin, as determined by an indirect immunofluorescence test, and that reacted to type VII collagen, as determined by immunoblotting on dermal extract. Our observations suggest that a chronic, widespread, blistering eruption is not a prerequisite for the diagnosis of bullous SLE, and a mild, transient, blistering eruption could be a manifestation of type I bullous SLE.     

破伤风抗毒素所致药疹42例临床特点分析

目的：明确破伤风抗毒素所致药疹的临床特点。方法：对42例破伤风抗毒素所致药疹的临床资料进行回顾性分析。结果：42例患者中破伤风抗毒素皮试阴性40例,阳性2例。发疹潜伏期6 ~12天,平均为6.5天,皮疹表现为注射局部红斑2例,全身泛发性皮疹40例(发疹型6例,荨麻疹型32例,多型红斑型2例)。42例患者中伴发热5例,呕吐5例,关节痛12例。糖皮质激素治疗有效。结论：破伤风抗毒素所致药疹最常见类型为荨麻疹型,可伴发热、消化道症状及关节痛。药疹的发生与皮试结果无关。     

Phototests in Japanese patients with papulovesicular light eruption: positive provocation with UVA

In six Japanese patients with papulovesicular light eruption (PVLE), photoprovocative tests were performed by irradiating UVA or UVB on the skin of the back on three consecutive days. One patient developed the typical lesion of PVLE at the test site with two consecutive daily irradiations of UVA. The remaining 5 patients had no abnormal phototest reactions. It was obvious that UVA plays an etiological role in at least some cases of PVLE.     

Toxic dermatitis caused by tramadol

BACKGROUND: Tramadol chlorhydrate (Topalgic) is a powerful analgesic recently introduced in France where its use has spread rapidly. We report a case where this drug induced a maculopapulous toxic skin reaction with secondary erythrodermia. CASE REPORT: A 47-year-old man was treated for lower back pain with tramadol chlorhydrate (50 mg b.i.d.). Otherwise, he was in good general health and was taking no other medications. Shortly after beginning the treatment, he developed a highly pruriginous maculopapulous eruption involving the entire skin surface, hyperthermia and general degradation. There was no skin exfoliation, mucosal involvement nor nodal enlargement. Tramadol was withdrawn and the patient was given corticosteroid therapy. Secondary erythrodermia developed after termination of the corticosteroids. The lesions regressed after tramadol withdrawal. DISCUSSION: Tramadol-induced skin reactions are uncommon and usually benign. In our case, the delay from onset of tramadol and the development of the maculopapulous eruption was very short (four days). The patient was taking no other medication. We hypothesize that the patient had been sensitized by cross-reaction with another compound and recall the fundamental aspects of tramadol and opiate drugs.     

柳氮磺胺吡啶致重症药疹1例

患者男,57岁。全身皮肤红斑、瘀斑伴瘙痒、发热10d。患者1个月前因"溃疡性结肠炎"口服"柳氮磺胺吡啶",20d后全身皮肤出现红斑、瘀斑伴瘙痒、发热。血常规检查示单核细胞及嗜酸性粒细胞增多,血小板减少;肝功能异常;胸部CT平扫示腋窝淋巴结肿大。结合病史、临床表现和辅助检查诊断为:重症药疹。予甲基泼尼松龙联合人免疫球蛋白等治疗痊愈。     

Autoimmune progesterone dermatitis.

Seven patients had autoimmune progesterone dermatitis. The morphological findings illustrate the polymorphous nature of the disease in which urticaria, erythema multiforme, and dyshidrosiform lesions were seen. Recurrence of the eruption five to ten days prior to the menses with spontaneous resolution following the menses was present in all cases. Intradermal skin testing to progesterone was done to confirm the diagnosis. Six of the seven patients has a history of use of artificial progestational hormones prior to the beginning of their eruption. It is postulated that the artificial progesterones may have been the trigger for the development of their autosensitivity. Treatment with conjugated estrogens resulted in remission of the disease in five of the seven cases reported.     

Association of a pediatric bullous eruption, cutaneous and muscular atrophy, hyperpigmentation and dysmorphism. A new entity?

We report the case of a young patient who presented with dysmorphism, bullous eruption of childhood, cutaneous and muscular atrophy and hyperpigmentation. Attempts were made to find out where this case fits in the nosological framework. This young boy without any particular family history was born with facial dysmorphism consisting of micrognathia, right microphtalmos, gothic palate and left facial palsy of the peripheral type. At the age of 2 years and 9 months, generalized fragility of the skin appeared in the form of a recurrent bullous eruption. The bullae left numerous atrophic and depigmented scars; they spared the mucosae, and there was no photosensitivity. At the same time, generalized skin atrophy developed: the subcutaneous venous network was abnormally visible and there was diffuse hyperpigmentation. The hair was fine, curly and thin. The teeth dystrophic and abnormally positioned. The cornea of the right eye was invaded by conjunctiva. The bullous eruption subsided when the child was about 6 years' old, but the skin atrophy became worse and was accompanied with amyotrophy of the limbs and retraction in flexion of the joints at the extremities. Chronic ulcerations were present on the lower limbs. Statural and ponderal growth, as well as mental development were normal. The biochemical examinations performed revealed no abnormality. Light and electron microscopy of the skin showed a normal dermis-epidermis junction, but the anchorage fibres were rarefied. The dermal connective tissue was abnormal, with thin collagen fibres and disorganized fibrillae. Fibroblasts were hypoplastic and numerous. The elastic network was meagre and elastic fibres had a slashed appearance. Biopsy of a palmar nodule showed cheloid-like lesions. The nosological discussion involved some congenital bullous diseases and certain forms of connective tissue dystrophia. Among the bullous diseases, congenital poikiloderma, as described by Weary and Kindler, is unaccompanied with amyotrophy, articular retraction or dysmorphism. According to Verret et al., the bulla is located at the dermis-epidermis junction. Recessive dystrophic epidermolysis bullosa seems to differ from our case in that skin atrophy is localized and amyotrophy, dysmorphism and disorders of pigmentation are absent. In addition, we found no histological evidence of collagenolysis. Mendes Da Costa's bullous dystrophy can be excluded, as there was no nanism, microcephaly or diffuse alopecia in our patient. Among connective tissue dystrophias, acrogeria Ehlers-Danlos type IV syndrome does not include bullous eruption, amyotrophy or articular retraction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Progesterone sensitive Interferon-gamma producing cells detected by ELISpot assay in autoimmune progesterone dermatitis

Autoimmune progesterone dermatitis (APD) is a rare cutaneous disorder, characterized by recurrent polymorphous cutaneous and mucosal manifestations. It is considered to be caused by a hypersensitive reaction to endogenous progesterone. However, in vitro T-cell activity to this hormone has been described in few patients. Here we report the case of a 30-year-old woman with recurrent pruritic erythematous, and erythema multiforme-like eruptions localized to the genital area. Positive cutaneous reaction to intradermal progesterone injection suggested the diagnosis of APD. The analysis of cellular immune response to progesterone, investigated by the ELISpot assay, showed a significantly higher level of Interferon-gamma (IFN-gamma) producing cells in this patient compared with a control group comprising five asymptomatic women in the luteal phase of the menstrual cycle. Our results suggest that the ELISpot technique, together with clinical evaluations and assessment of allergies, could be useful in the diagnosis of APD.     

Giant cell lichenoid dermatitis

A case of drug-induced lichenoid dermatitis with an unusual epidermotropic multinucleated giant cell inflammatory response is reported. The patient is a 52-year-old white woman who is steroid-dependent because of long-standing systemic lupus erythematosus. At the time of presentation of her generalized papulosquamous pruritic eruption, she was taking oral antihypertensive medications (methyldopa and chlorothiazide). After discontinuation of these medications and local treatment with topical corticosteroids, the skin eruption dramatically improved. Microscopically, the skin lesions had a lichenoid inflammatory pattern, with multiple cytoid bodies, multinucleated giant cells, and a mixed chronic inflammatory infiltrate that included lymphocytes, histiocytes, and eosinophils.     

Photosensitivity due to a fluorouracil derivative.

A photosensitive lichen planus-like eruption developed in a 63-year-old man during postoperative chemotherapy with 1-(2-tetrahydrofuryl)-5-fluorouracil. The action spectrum for the photosensitivity was in the long-wave ultraviolet light (UV-A). The reaction was reproducible on readministration of the drug and exposure to UV-A. At the time of this test, a flare-up phenomenon was observed at previously involved sites. The photopatch test was negative with the drug. Patients taking the drug who had no dermatitis were not sensitive to UV-A. These observations suggest that the photosensitivity that occurred in the patient was possibly photoallergic.     

A case of acute generalized exanthematous pustulosis (AGEP) possibly induced by iohexol

Acute generalized exanthematous pustulosis (AGEP) is an uncommon disease manifested as an erythematous pustular eruption. It is usually caused by systemic medication. We describe a patient with acute generalized pustular eruption induced by iohexol. A 52-year-old woman developed fever and a generalized pustular eruption on the neck, trunk and extremities three days after taking iohexol. The culture from pustules was sterile. Other systemic and laboratory examinations were normal. A skin biopsy from a lesion on the trunk showed the features of a drug-induced pustular eruption as a subcorneal blister including neutrophils and eosinophils, mild spongiosis, and a sparse infiltrate at neutrophils and eosinophils in the papillary dermis. The patient had no history of psoriasis. The lesions resolved with systemic corticosteroid therapy within one week and did not relapse. According to our investigation, iohexol-induced AGEP has not been previously reported. We present an interesting case.     

Pityriasis rosea and ketotifen

A 4-year-old female patient who developed a skin eruption similar to pityriasis rosea after treatment with ketotifen (Zaditen) is presented. The relationship between ketotifen and the eruption has been based on circumstantial evidence and confirmed by the positive results of the MIF test and the rat mast cell degranulation test.     

Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma

An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the DNA extracted from the specimen. A diagnosis of CD8+ cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8+ primary CTCL with clinical features resembling HV.     

The Photosensitivity Localized in a Vitiliginous Lesion Was Associated with the Intramuscular Injections of Synthetic Progesterone during an In Vitro Fertilization-embryo Transfer

The cutaneous diseases associated with progesterone are autoimmune progesterone dermatitis, erythema multiforme-like eruption, drug-induced progesterone dermatitis and solar urticaria. Estrogen and progesterone are widely used in oral contraceptives and hormone replacement therapies, and they are rarely known to cause a photosensitive reaction. The mechanism of contraceptive-induced photosensitivity is uncertain. Estrogen, rather than progesterone, in the combined oral contraceptive pill has been most frequently implicated in the induction of photosensitivity. A 32-year-old woman presented with an erythematous patch with an itching sensation on the centrofacial area of a residual vitiligious lesion. She had a history of being previously treated with narrow band UVB for 1 year. Her skin lesions had mostly subsided, but some lesions continued. She underwent an in vitro fertilization-embryo transfer 3 months previously, and she then took synthetic progesterone for 3 weeks starting at the 4th week of pregnancy. She was in good health with neither a family history of photosensitivity nor a personal history of any other drug ingestion or topical agent such as sunscreen in association with the beginning of her lesions. Phototesting revealed her to be markedly photosensitive in the UVB and UVA ranges. The intradermal skin reactions to progesterone combined with irradiation with UVA or UVB were positive. We report here on an unusual case of photosensitivity that was localized in a vitiliginous lesion, and this was associated with the intramuscular injections of synthetic progesterone that she had received during an in vitro fertilization-embryo transfer.