Sunday, December 19, 2021
Monday, November 16, 2020
In June of 2019 we presented a 26 yo man whose main problem was scrotal lymphedema related to hidradenitis supprativa (HS). In 2018, he had surgery at a major academic center to excise active HS lesions in both crural folds. The operative report states that 20x8 cm were excised from the right groin and 20x6 cm from the left groin. This seems to have been antecedent to the development of scrotal lymphedema. His HS been recently managed with adalimumab, 40 mg per week. Gradually, the active inflammatory lesions have improved, although he still has some fistulous drainage.
His main problem now is a buried penis. It’s interesting how he has adapted to this. The patient is reclusive. He lives with his parents and spends his days playing video games. When discussing these he displays a vast, enthusiastic knowledge.
He is very anxious about spending time in hospital and prefers to stay home where his attentive parents care for him.
We are presenting him to discuss "the buried penis," an unusual disorder, and to see if anyone has helpful suggestions. There were some recent in-depth reviews of “the buried penis” and we will see if the authors are interested in commenting.
1. Evaluation and management of adult acquired buried penis. Ho TS, Gelman J. Transl Androl Urol. 2018 Aug;7(4):618-627. Free Full Text.
Tuesday, October 20, 2020
This 20 year-old woman developed a painful bilateral erythema
of the skin at the margins of the thumbs and index fingers. Around two weeks later, she noticed bleeding
under one thumb nail and color changes under the other thumb nail.
She was seen at a walk-in clinic where no therapy was given
but a battery of blood tests were done.
Among them was an ANA which was reported as positive with a homogeneous pattern and a titer of 1:640. Her RF, C Reactive Protein and EST were normal as well as all other tests.
The positive ANA alarmed her and her parents and she
sought a dermatological opinion.
The clinical images taken 3 weeks after onset show mild erythema at the margins between dorsal and palmar skin and the nail changes.
Further history revealed that she had been on doxycycline for acne
when the eruption occurred.
drug eruption from doxycycline with photoonycholysis. I presume the positive ANA is a "false positive." The test should probably not have been
ordered. As an incidental finding, taken out of context it can be anxiety-provoking.
1. Doxycycline-induced photo-onycholysisDidier Rabar, Patrick Combemale, François Peyron. J Travel Med. Nov-Dec 2004;11(6):386-7.
Abstract: Because there is a widespread resistance to other drugs, doxycycline is often prescribed as chemoprophylaxis for malaria. Although this drug is commonly used for the treatment of acne vulgaris, no large studies have been conducted on the safety of doxycycline. However, several side effects, especially skin and nail disorders, are induced by this drug. In this article, we report a case of photoonycholysis in a woman undergoing doxycyclineprophylaxis for malaria.
Following Two Weeks of Doxycycline. KC S, Karn D, Shrestha S. J Nepal Health Res Counc 2016 Jan - Apr;14(32):66-8
Abstract: Photo-onycholysis is a form of phototoxic reaction characterized by spontaneous separation of the nail plate from the nail bed. It usually follows drug intake and tetracycline is a well-known culprit. We present a case of 19 years gentleman who developed this rare side effect following two weeks of ingestion of doxycycline.
3. Antinuclear antibodies in healthy people and non-rheumatic diseases – diagnostic and clinical implications. Bogna Grygiel-Górniak, Natalia Rogacka, and Mariusz Puszczewicz. Reumatologia. 2018; 56(4): 243–248. PMID: 30237629. Free Full Text.
Thursday, October 08, 2020
The patient is a 90 yo man who has symptoms suggestion of Parkinson’s disease. Four months ago his neurologist started him on Neupro®, a Rotigotine Transdermal Therapeutic System. His dosage was slowly increased. The patient’s mental status is excellent, and although his back was pruritic, he could not see it. A light-complected White with a history of skin cancer, he was seen for a regular follow-up examination during which a peculiar pattern of rectangular erythematous patches was noted.
Diagnosis: Probable allergic contact dermatitis to Neupro® (Rotigotine)
page for rotigotine states:
“General side effects for rotigotine may include constipation, dyskinesia, nausea, vomiting, dizziness, fatigue, insomnia, somnolence, confusion, and hallucinations. More serious complications can include psychosis and impulse control disorders like hypersexuality, punding, and pathological gambling. Mild adverse skin reactions at the patch application site may also occur.” However, PubMed has only one reference to this dermatitis. The case report was of a patient who developed ACD to the active ingredient, but not to the vehicle.
It is likely that ACD to rotigotine is not as uncommon as the literature would lead us to assume.
Nadia Raison-Peyron, Bernard Guillot. Allergic contact dermatitis caused by rotigotine in a transdermal therapeutic system. Contact Dermatitis. 2016 Aug;75(2):121-2.
Abstract: Allergic contact dermatitis caused by transdermal therapeutic systems (TTSs), resulting from the active drug, an adhesive, or an excipient, is rare. We report the first case of allergic contact dermatitis caused by rotigotine, a non-ergolinic dopamine receptor agonist, in a TTS in a patient treated for Parkinson’s disease. In this patient the rotigotine patch test was positive and there were negative results with the rotigotine patch tests in 5 controls.
Monday, September 21, 2020
Presented by Douglas Johnson, M.D.
This 63 yo Korean woman presented to the ER with fatigue.
Noted to have large ulcerated plaque on the right forehead and frontal scalp. She had been seen in wound clinic on one occasion 2 years before and at the time was noted to have a 2 x 2.5 cm ulcer on the right forehead.
The patient believes that this was caused by a cosmetic procedure she had done in Korea by a plastic surgeon many years ago.
Seems like she may have had a facelift.
Pathology: The initial biopsy revealed a normal epidermis with a heavy
mixed inflammatory infiltrate suspicious for infection, although special stains for bacteria, fungi and virus was negative.
Wound culture grew Staph Capre (a commensal organism).
Second routine biopsy and tissue biopsy for cultures is pending.
What are your thoughts as to the diagnosis.
Wednesday, August 19, 2020
The patient is an active 10-year-old boy who was seen today for evaluation of a vascular anomaly on his right knee that has been present since birth. It was biopsied in March 2017 and the histologic diagnosis was "lymphatic malformation consistent with a lymphangioma."
Occasionally, his knee will swell and he will get some bleeding spots. He is learning boxing and that can precipitate swelling. He never has pain. Has not had any infection. I referred him to a pediatric dermatologist 3 years ago but they were out of network and he never went.
The lesion measures 7 cm in diameter on the right knee. There are areas of dark purplish pigmentation in confluent vesicles.
Pathology: There were multiple dilated irregular thin walled
vessels lined by a single layer of bland endothelial cells.
circumscriptum. I expect there is a deep
component. The patient has no
lymphedema. Note: The new terminology for this is "Microcystic Lymphatic Malformation (Ref 4.)
Question: Should this tumor be evaluated and if so how? Would any therapy be better than active surveillance?
Alyssa Miceli, Kristen M. Stewart In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan. 2020 Aug 12. Free Full Text.
Lymphangiomas are uncommon, benign malformations of the lymphatic
system that can occur anywhere on the skin and mucous membranes. Lymphangiomas
can be categorized as deep or superficial based on the depth and size of the
abnormal lymphatic vessels or as congenital or acquired. The deep forms of
lymphangioma include two specific well defined congenital entities: cavernous
lymphangiomas and cystic hygromas. Superficial forms of lymphangioma include
lymphangioma circumscriptum and acquired lymphangioma, which is also referred
to in the literature as lymphangiectasia. Although both entities share similar
clinical and histologic features, the term lymphangioma circumscriptum infers
lymphatic channel dilation due to a congenital malformation of the lymphatic
system. Whereas, the term lymphangiectasia, or acquired lymphangioma, denotes
dilated lymphatic channels of previously normal lymphatics that have become
obstructed by an external cause.
2. The Possible Role of Color Doppler and Pulse Wave Doppler Ultrasound
in the Diagnosis of Cutaneous Lymphangioma Circumscriptum. Yang Sun, et. al. J Craniofac Surg. Jan/Feb 2020
Abstract: Cutaneous lymphangioma circumscriptum (CLC) is an uncommon congenital lymphatic malformation. Its dermoscopic features have been reported, however, if blood has infiltrated the lacunas, a pink hue overwhelms the lacunar structures, rendering dermoscopic features indistinguishable. In addition, dermoscopy cannot assess the subcutaneous extent of the lesion before surgery. The high-frequency array transducer Sonography with a high-frequency transducer is excellent in its resolution to evaluate skin lesions and is unaffected by infiltration of blood. Here, the authors report the use of ultrasound (color Doppler and pulse wave Doppler) for the diagnosis and management of CLC.An 18-year-old man presented 10 years previously with an asymptomatic 0.5 × 0.7 cm cluster of dark reddish vesicular lesions on his buttock. The lesions had recently increased in size and number. There was associated pachyderma. The lesions were hyperechoic and well defined on grayscale ultrasound and hypervascular on color Doppler ultrasound. Cluster cystics of lymphatic spaces were found throughout the whole dermis, especially in the papillary dermis. The lymphatic channels extended downwards to the reticular dermis and the superficial layer of the subcutaneous tissue. The pathological findings revealed lymphangiomas invading the subcutaneous tissue. The flat endothelial cells were partly positive for D2-40, which is a marker of lymphatic endothelial cells. Ultrasound was able to delineate the margins of the lesions. Diagnosis of CLC using preoperative color Doppler and pulse wave Doppler could reduce unnecessary repeated pre- and postoperative biopsies.
3. Lynphangioma Overview National Organization for Rare Disorders.
4. Micro- and Macrocystic Lymphatic Malformation. Lindsay McCormack, Krystal Jones, Jennifer T Huang. J Pediatr. 2020 Apr;219:275-276. Free Full Text
5. Treatment of superficial vascular anomalies with topical sirolimus: A multicenter case series. Melissa Dodds, et. al. Pediatr Dermatol. 2020 Mar;37(2):272-277.
Abstract: Background: Systemic sirolimus (rapamycin) has recently been found effective in treating complex vascular anomalies by reducing the size and associated complications. Many vascular anomalies have a cutaneous component, and thus, we sought to determine whether topical administration of sirolimus may be an effective therapy, as data on the use of topical sirolimus are limited.
Results: Eleven patients had combined venous lymphatic malformations, three had tufted angiomas, two had a lymphatic malformation, one had a venous malformation, and one had a verrucous venous malformation. All (100%) patients reported some degree of improvement and 50% of patients reported marked improvement in one or more symptoms, most commonly blebs and lymphatic drainage, and bleeding.
Conclusion: Topical sirolimus appears to be a safe and useful non-invasive therapy that is well-tolerated in the treatment of the cutaneous portion of a variety of vascular anomalies.
Monday, August 17, 2020
Pathology:Aggregates of undifferentiated baseloid epithelium, some with central clear cell change and necrosis, associated with a peripheral rim of lymphocytes and scattered plasma cells. In situ hybridization for EBV was negative.
These changes were interpreted as most consistent with a diagnosis of lymphoepithelioma-like carcinoma of the skin.
Thanks to Dr. Lynne Goldberg of Boston University Skin Pathology for the photomicrographs.
|Appearance at re-excision|
ENT Connsult: No evidence of pharyngeal pathology
MRI: Normal with no adenopathy
The patient was referred for Mohs Micrographic Surgery. The tumor was cleared with one stage, but a second stage was done to assure adequacy of excision. (The patient's insurance was not charged for the 2nd stage).
Lymphoepithelioma-like carcinoma of the skin (LELCS) is a rare tumor of unknown etiology, low malignant potential, and microscopic resemblance to undifferentiated nasopharyngeal carcinoma. Clinically (as in this case), it presents as a flesh-colored firm nodule or plaque on the face, scalp, or shoulder of middle-aged to elderly individuals. Histologically, LELCS is composed of islands of enlarged epithelial cells with large vesicular nuclei surrounded and permeated by a dense lymphoplasmacytic infiltrate. LELCS exhibits immunoreactivity with high-molecular-weight cytokeratins and epithelial membrane antigen, indicating the epithelial origin.
Initially, the ENT consult felt that the patient should be referred to a radiotherapist for XRT; but the literature (see ref 3) does not support that. He will be followed up at regular intervals by his dermatologist.
This case presentation highlights the difficulty in treating a rare disorder. No one has enough expertise and one must fall back on the literature that may not be clear. Only about 80 of these tumors have been reported. Consultants may give opinions that are not based on science but on their own belief systems. Where does the buck end? The psychiatrist Michael Balint, described “The Collusion of Anonymity” in his book “The Doctor, His Patient and the Illness.”4 This holds that “the burden of responsibility is much too great, and everyone, including the patient, naturally tries to lighten it by involving someone else, or, if possible, a number of others. This may be described as the process of dilution of responsibility.
If all goes well with this collusion of anonymity all parties concerned feel justifiably proud and gratified. If anything goes wrong nobody is individually responsible.
The collusion of anonymity is one method of lightening the burden of responsibility. Present day practice in medicine is hardly more than the sum of the various specialties.
Nowadays everybody preaches that when a patient is ill the whole person is ill, not only his skin, stomach, his heart or his kidneys. This truth, while constant lip service is paid to it, is unfortunately ignored in most medical practices. A very experienced and disillusioned GP said, "nowadays it is enough for a doctor to know about 20 prescriptions and the address of about 30 consultants."