Adult Onset Atopic Dermatitis with Secondary Follicular Mucinosis with Cyclosporine Induced Spiny Follicular Hyperkeratosis and Hair Casts
A 54 year-old female presented to us with multiple itchy red raised lesions over body and lichenified plaques over bilateral forearms with severe dryness of skin allover body developing gradually over last four years. Lesions used to get aggravated in winter season. She complained of lack of sleep due to severe itching and also of feeling depressed and emotional stress aggravating her disease. There was no any other significant medical history.\nExamination revealed severe xerosis allover body, Hertoge's sign (loss of lateral eyebrows), bilateral Dennie-Morgan's folds and symmetric lichenified plaques on extensor aspect of both forearms and arms with multiple excoriated papulonodular lesions over abdomen and bilateral lower legs [ and , respectively]. Mucosal examination, palms, soles and nails appeared to be normal. A clinical diagnosis of adult onset AD was thought. Biopsy from forearm revealed orthohyperkeratosis, hypergranulosis, irregular epidermal hyperplasia and superficial perivascular lymphocytic infiltrate with occasional eosinophils [ and ]. In addition to these, there were several hyperplastic follicles showing plenty of bluish-gray stringy mucin collected in large pools within the follicular epithelium in the same section [, and ]. Based on clinicopathological correlation and using Hanifin and Rajka's criteria for atopic dermatitis[] patient was diagnosed as adult onset AD with secondary follicular mucinosis. Her complete hemogram was normal except for 11 gm% hemoglobin. Serum IgE levels were marginally raised. No any other significant biochemical abnormality was detected. In view of affected quality of life patient was started on oral cyclosporine 5 mg/kg day in 3 divided doses after recording two normal baseline blood pressure readings along with potent topical steroids, moisturizers and oral antihistamines for pruritus. Two weeks later patient came back with multiple white colored spiny follicular keratoses developing allover body along with increased hair fall and hair casts. Lesions were most concentrated and prominent on chin and neck area [Figures , and ].\nExamination revealed multiple whitish follicular keratoses up to 1 mm in diameter and up to 5 mm in length []. Also there were numerous hair casts over scalp which were freely mobile along the shaft and mimicking pseudonits []. A repeat biopsy including follicular keratoses was done from abdomen and a hair mount was examined. Histology revealed normal epidermis with dilated follicle containing compact orthokeratotic plug extending above surface with mild perifollicular lymphocytic infiltrate [-]. Hair mount revealed loosely adherent whitish casts without any fungal elements [].\nBased on clinicopathological correlation a diagnosis of SFH with hair casts was made. With working diagnosis of SFH, we reviewed literature and found one case of cyclosporine induced follicular keratoses and strong association of SFH with MM. Accordingly cyclosporine was stopped and patient was investigated. There were no any signs or symptoms suggestive of MM or inflammatory bowel disease. Her total serum proteins, albumin: Globulin ratio, erythrocyte sedimentation rate, serum calcium, serum retinol level, repeat renal function tests, serum protein electrophoresis were all normal and no myeloma band was detected. Ultrasound of abdomen revealed no abnormality. Her radiograph of spine and scalp was also normal. Ear, nose and throat examination was also within normal limits. Both Kappa and Lambda light chains were also normal. Thus no any underlying malignancy was detected.\nAfter three weeks of stopping cyclosporine all the skin lesions cleared [] and 50% reduction in hair casts was noted. Thus a diagnosis of adult onset AD with cyclosporine induced SFH and hair casts was made. Patient is advised to take methotrexate 10 mg/week along with folic acid supplementation as an alternative to cyclosporine. Remaining medicines were continued as it is.\nThis is probably the first case of SFH from India and second case of cyclosporine induced SFH. Also we could not find report of secondary follicular mucinosis developing in adult onset AD.