Tackling The Tough Cases

Dermatologists are sleuths by nature. And they all have at least one interesting case that was difficult to treat or diagnose tucked away in the mental file cabinet. Here, we’ve asked doctors to share with you some of their most interesting cases. Case 1: Splish Splash, I Was Takin’ a Bath By Joel DeKoven, M.D., M.H.Sc., F.R.C.P.C. I was on holiday when Roger, a 50-year-old former patient of mine with Korsakoff’s psychosis, was admitted to the hospital after suffering a first-witnessed seizure. At the time, he was trying to cope with a raging rash in the perineal area that wasn’t responding to oral cephalexin (Keflex). On admission, he was afebrile and systemically well. However, there was an angry-looking eczematous eruption under his boxers involving the lower abdomen, upper medial thighs and perineum with many erythematous macules on the trunk and extremities. His white blood cell count was 26,000. A CT scan of the head was normal. Diagnosed with a possible allergic reaction to cephalexin complicated by cellulitis, he was treated with I.V. gentamicin (Garamycin) and vancomycin. His skin rapidly improved. He was discharged, only to return in the next month for two more admissions with the same problem. Each time, he rapidly improved on I.V. antibiotics with prednisone added for good measure. Back from holidays, I boldly proclaimed at the outset that this was not a drug reaction, though it might be a recurrent streptococcal infection; prednisone would not be helpful. However, cultures from the skin, throat, blood and nares were negative. Nevertheless, he cleared rapidly once again. I then remembered our first meeting the year before. He had presented with a striking recurrent erythematous eruption conforming almost perfectly to his beard area, his third episode in the last 2 years. The diagnosis: impetiginized dermatitis possibly with an allergic contact etiology. A swab of the eruption showed Staphylococcal aureus and he cleared with oral cephalexin and topical mometasone furoate ointment. Patch testing uncovered 2+ reactions to mercaptobenzothiazole, black rubber mix and paratertiary butylphenol formaldehyde resin. This only helped to explain a recurrent vesicular foot dermatitis that he’d had some years ago. On his next admission, I recommended that all antibiotics be stopped and that he take prednisone. I also told him to avoid all skin contact with rubber. One week after discharge to his group home, he began to flare again. He came to see me in the office accompanied by Brent, a worker at his group home. I told them the key was rubber. I asked whether he had a rubber-lined chair, if he sat on rubber covered furniture, whether he cycled nude on a bike or exercise bike. For a full minute there was silence as Roger, Brent and I blankly stared at each other. Then, Brent’s eyes lit up, “ We have a rubber mat on the bottom of the bathtub!” he exclaimed. Ah, Roger with his daily 30-minute baths! Three admissions to the hospital and three flares when he went back home. Salvation in the hospital shower. Case 2: Bound to Biopsy By Steve Feldman, M.D. A 45-year-old woman presented to my office because of a lesion on her right eyelid. She reported a history of sarcoidosis lesions of the face that had been confirmed by biopsy. Another dermatologist had previously evaluated her eyelid and treated it with intralesional corticosteroids. When I examined her, I found a 0.5-mm lesion with ill distinct borders. The lesion had somewhat of an “apple jelly” color consistent with sarcoidosis. There were also telangiectasias that I felt were consistent with previous steroid injection. I have a very low threshold for obtaining biopsies of questionable skin lesions. Because this lesion seemed atypical, I obtained a small shave biopsy. Whitish material was present in the biopsy specimen. I thought that it seemed that this was just a small, atypical cyst, perhaps modified in appearance from the previous corticosteroid injection. Or, I considered that maybe the white material was some leftover corticosteroid. But, the biopsy report showed a basal cell carcinoma. I referred her for micrographic surgery. This isn’t the first time that a basal cell carcinoma fooled me. Having a low threshold for obtaining biopsies of atypical skin lesions probably isn’t such a bad idea. Case 3: Nailing Down the Problem By Lance H. Brown, M.D., and Ronald R. Brancaccio, M.D. A 40-year-old woman presented with a 10-month history of an intermittent, chronic eruption, which was confined to her lips. She’d already been evaluated by several other physicians, including dermatologists, who treated her with a variety of topical formulations. Treatments included topical cortisone creams of varying strengths — none of which provided long-term relief. Our patient had also attempted to relieve the problem in several ways herself. She tried changing her toothpaste, dentifrices and lipstick and used a variety of different lip balms, all without any improvement. We entertained the possibility of an allergic contact dermatitis to her lip cosmetics or oral dentifrices and the patient was patch tested to an expanded screening and cosmetic patch test series. The results revealed a 2+ reaction to toluene sulfonamide-formaldehyde resin, the allergen in nail polish, at 48- and 96-hour reading periods. The patient’s dermatitis completely resolved after discontinuation of the culprit nail polish. Contact dermatitis to nail polish is relatively common and usually presents as an ectopic dermatitis, most commonly on the eyelids and neck. Allergic cheilitis caused by nail polish is relatively uncommon. The sensitizer in nail polish is a toluene sulfonamide-formaldehyde resin, now called tosylamide formaldehyde resin. This case reiterates the importance of patch testing to a large array of allergens. Additionally, you should entertain the possibility of an ectopic contact dermatitis when your diagnosis is uncertain. Case 4: Acne Fulminans or Something More? By Scott T. Guenthner, M.D., Marc A. Darst, M.D., and Charles W. Lewis, M.D. A 22-year-old man presented to the dermatology clinic with ulcerations on his back and neck, which he’d had for 10 to 12 months. Previously, he had been treated with isotretinoin (Accutane) 80 mg q.d. for nearly 24 weeks and prednisone 30 mg to 60 mg q.d. He had a history of severe acne since age 11. At initial presentation, he was taking isotretinoin 40 mg q.d. and prednisone 60 mg q.d. In addition, he was taking minocycline (Minocin) 100 mg b.i.d. He had a family history of ulcerative colitis and was a mild smoker. Upon questioning, he explained that he was experiencing mild arthralgias and fevers. An examination of his skin showed a few giant comedones and large irregularly-shaped ulcerations on his back and neck. Oral, hair and nail examinations were normal. Although the clinical diagnosis was presumed to be acne fulminans, due to the long nature of the disease and the poor response to therapy, a decision was made to obtain a skin biopsy to confirm the diagnosis. Other diagnoses that were included in the differential diagnosis were pyoderma gangrenosum, a deep fungal infection, and an atypical mycobacterial infection. Histopathologic findings revealed an ulcerated epidermis, diffuse mixed inflammatory infiltrate of histiocytes, eosinophils and neutrophilic microabscesses. The infiltrate extended through the dermis, subcutis and focally into skeletal muscle. The histopathologic differential diagnosis included acne fulminans and pyoderma gangrenosum but the dermatopathologists couldn’t entirely rule out Wegener’s granulomatosis. Laboratory tests were then obtained. Most importantly, the patient had a positive skin culture for Staphylococcus aureus, and a c-ANCA of +1:160, which is sometimes consistent with a diagnosis of Wegener’s granulomatosis. We then ordered a chest X-ray and subsequent CT of the chest, which showed nodular densities and old interstitial scarring in the lungs. Concurrently, the patient was evaluated by an ENT physician who biopsied a nasal lesion that was found on examination for the patient’s new complaints of nasal pain and rhinorrhea. This biopsy was found to be consistent with a granulomatous dermatitis consistent with Wegener’s granulomatosis. We confirmed the diagnosis of Wegener’s based on the combination of the patient’s clinical symptoms, c-ANCA value, the skin and nasal biopsy results, and the pulmonary findings on CXR and CT examination. The patient was started on cyclophosphamide (Cytoxan) 100 mg q.d. and prednisone 40 mg q.d. The patient’s ulcers rapidly healed within 6 to 8 weeks of initiating treatment with cyclophosphamide. He also experienced tremendous clinical improvement overall. This case illustrates the importance of histopathologic confirmation of unusual dermatologic presentations and diseases, as well as the complete evaluation of patients with multisystem complaints. Case 5: The Golfer’s Stubborn Toe By, Aditya K. Gupta, M.D., Ph.D., F.R.C.P.(C), and Elizabeth A. Cooper, B.E.Sc, H.B.Sc. A 56-year old patient presented with an abnormal-appearing right great toenail. The patient indicated that the nail had changed color within the last several months. During the history taking, we discovered that the man regularly participated in sports, playing golf every weekend. He would shower at the clubhouse, using the communal locker room area. From time to time, he would get “athlete’s foot,” which he’d treat with over-the-counter medications. The patient was right-handed and the leading foot was the right foot. He had no family history of onychomycosis and wasn’t diabetic. He smoked two packs of cigarettes each week and had done so for the last 25 years. On examination, the right great toenail was abnormal appearing with a longitudinal spike running down the center of the nail to within 2 mm of the proximal nail fold. All the nails were slightly discolored with a yellow tinge. The other toenails otherwise appeared normal. There was hyperkeratosis, fissuring and maceration of the web space between the right fourth and fifth toes. No odor was present, which is consistent with tinea pedis interdigitalis. Also, there was no convincing evidence of tinea pedis plantaris , although the soles of the feet demonstrated fine scaling. The feet were somewhat cool on touch; however, both dorsalis pedis pulses were palpable. The posterior tibial pulses could not be felt in either foot. The fingernails were normal, and no clinical evidence of tinea manuum was present. No other significant dermatitis was evident. The patient wasn’t taking any oral medications. Mycological examination of the involved nail revealed Trichophyton rubrum infection. The toe web area gave a positive light microscopic examination for fungal filaments; it was culture negative. Baseline liver function tests were within normal limits. The patient was started on itraconazole pulse therapy and received 3 pulses (200 mg twice daily for 1 week with 1 weeks on, 3 weeks off). There was minimal response to therapy. One year later the patient was placed on a standard course of terbinafine, 250 mg/day for 12 weeks. Once again, there was minimal improvement. At 9 months after starting the terbinafine, it was decided to mechanically remove the diseased portion of the toenail. This could be performed without the use of local anesthetic. Subsequently, the patient was retreated with 4 weeks of oral terbinafine, 250 mg day. The diseased nail slowly grew out with a successful response to combined mechanical and oral therapy. Points to make This difficult-to-treat case of onychomycosis, highlights several important point about treating this persistent disease. • Oral therapy for onychomycosis isn’t always successful. • Sometimes, you should consider combined therapy — in this case, a combination of mechanically removing the affected nail and oral therapy. • The longitudinal streak of onychomycosis may be removed mechanically without anesthesia. A nail clipper and a curette work fine, and in some instances a local anesthetic may be necessary. • Once you’ve removed the diseased nail, then the nail generally shows a much better response to therapy (oral or topical lacquer). • The longitudinal nail presentation (dermatophytoma) may represent an area of nail where the fungal organisms are aggregated with the keratin, making it more difficult for the antifungal drug to reach high concentrations in the area.