Saturday, September 19, 2015

An Unusual Case of Scalp Ulceration


33 yo woman with six month history of extensive scalp ulceration


History: The patient is a 33-year-old unemployed cashier who presented for evaluation of facial erythema.  She has had insulin dependent diabetes since childhood but apparently, it was not treated for many years.  She is being seen at the Wound Care Clinic now for a large erosion of the scalp present for ~ six months. Other than insulin, her only medication is gabapentin for her scalp symptoms. 

She is married.  Lives with her husband and 18 cats.

EXAMINATION:  The examination shows a 33-year-old woman with a flat affect. She has some erythema on her cheeks and bridge of the nose.  A few, but not many, papules. 

She was wearing a  base-ball cap and when I asked her to remove it, I saw a 7 x 8 cm erosion on the scalp She says she has been picking on this for quite some time because it was itching and painful. 

The remainder of the exam was unremarkable other than showing poor dental hygiene.  



Pathology: Scalp: There is dense and deep dermal fibrosis with superficial fibrin deposition, and a mild to moderate superficial and deep perivascular lymphoplasmacytic infiltrate with occasional neutrophils and focal multinucleated giant cells.  These changes are consistent with chronic and impetiginized ulcer but are not specific for its etiology that could include previous traumatization (although the depth of the dermal fibrosis is unusual for a simple excoriation) or an infectious etiology.
Biopsy of face confirmed findings of rosacea.  No evidence of demodex or collagen vascular disease.

Lab:  CBC, Complete Chem profile normal.  ANA negative.  KOH prep from face failed to reveal demodex.

IMPRESSION:  This is a complex problem.  Her facial erythema will be treated with doxycycline.  Her scalp excoriation is worrisome. (A similar patient was described by Atul Gawande in the New Yorker a number of years ago (see reference).  I wonder if the 18 cats at home are significant.

Questions: Any thoughts you have will be appreciated.  It will be great if we can help this unfortunate woman.

References: 
1. Gawande A.  The Itch: Its mysterious power may be a clue to a new theory about brains and bodies.  New Yorker.  6/30/2008.  Full Free Text Online.

2. Dr. Sharquie from Baghdad sent us a recent article of his: The Major Psychodermatological Disorders in Iraq.  

3.  Cat’s Curse: A Case of Misdiagnosed Kerion
MB Mazlim, L Muthupalaniappen

Malays Fam Physician. 2012; 7(2-3): 35–38.  Free Full Text.

Abstract:  Kerion is an inflammatory type of tinea capitis which can be mistaken for bacterial infection or folliculitis as both conditions display similar clinical features. It occurs most frequently in prepubescent children and rarely in adults. We report a 26-year-old woman who presented with multiple tender inflammed nodules on her scalp. Her condition was misdiagnosed as bacterial abscess and treated with multiple courses of antibiotics without improvement. Later, her condition was re-diagnosed as kerion based on clinical appearance, history of contact with infected animal and Wood’s lamp examination. symptoms and lesions resolved completely with systemic antifungal treatment leaving residual scarring alopecia. The delay in the diagnosis and treatment of this patient resulted in permanent scarring alopecia.

4) See previous VGRD entry of a 9 yo girl with scarring alopecia. (January 2006)


5) Dr. Barry Ladiainski suggested this reference
Cervical Trophic Syndrome: A Distinct Clinical Entity?

Alison A. Fischer, MD; David M. Adelson, MD; Carlos Garcia, MD
Cutis. 2014 June;93(6):E6 - E7
Absract: Ulceration is not a typical feature of notalgia paresthetica or brachioradial pruritus; a history of self-mutilation due to underlying paresthesia is consistent with the diagnosis. This case report describes a patient with underlying spinal pathology; the authors discuss this case as a spectrum between trigeminal trophic syndrome and notalgia paresthetica whereby lesions of peripheral spinal nerves may lead to unilateral ulcerations restricted to a dermatome.

6) Trigeminal Trophic Syndrome: Report of 3 Cases Affecting the Scalp
Ranti S. Bolaji, MD; Barbara A. Burrall, MD; Daniel B. Eisen, MD. Cutis. 2013 December;92(6):291-296
Abstract: Trigeminal trophic syndrome (TTS) is a rare condition that results from a prior injury to the sensory distribution of the trigeminal nerve. Patients typically respond to the altered sensation with self-mutilation, most often of the nasal ala. We describe 3 patients with TTS who presented with self-induced ulcerations primarily involving the scalp. Two patients developed delusions of parasitosis (DOP) based on the resulting symptoms of TTS, which is a unique association. Trigeminal trophic syndrome may occur at extranasal sites and in any branch of the trigeminal nerve. The condition should be considered when ulcers are encountered in this nerve distribution. Symptoms such as formication may mimic DOP. Trigeminal trophic syndrome may be differentiated from DOP by the restriction of symptoms and ulcerations to the distribution of the trigeminal nerve.
 

 



5 comments:

  1. Henry Foong wrote: "Here is a reference which may be useful. Considering the fact that this patient has 18 cats at home, my strong suspicion is that of inflammatory kerion. the histological findings of suppurative and granulomatous dermatitis is consistent with M canis tinea capitis. Suggest do a Wood’s lamp examination, and culture the hair follicle for dermatophytes. if possible check peripheral hairs for hyphae. We see many such cases in Malaysia where the malays like to keep cats at home. See:
    Int J Dermatol. 2006 Mar;45(3):215-9.
    Kerion and dermatophytic granuloma. Mycological and histopathological findings in 19 children with inflammatory tinea capitis of the scalp.
    Arenas R, Toussaint S, Isa-Isa

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  2. Helge Riemann (Brunswick, Maine): "I had a similar case that I treated with aripiprazole. She stopped picking and her ulcer healed."

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  3. Submitted by Robert Shapiro (Hilo, Hawaii): Neuropathic itch with excoriation is a tempting diagnosis, apparently the working diagnosis of the clinicians, given the history of untreated diabetes and the use of neurontin. However, I concur that in this case that diagnosis would have to be a diagnosis of exclusion after fungal was attempted to be diagnosed, and possibly treated, even if proof is not possible, due to the risk of permanent alopecia if not treated, and the fact that it wouldn't resolve without that treatment. The escar present already may leave a permanent alopecia. In a kerion the fungus may not be able to be confirmed, as the inflammation may mask the fungus. Perhaps additional punch biopsies at the edge of the escar and in the mid area of the flat part might be done for fungal culture and PAS.
    They didn't say if they did a PAS on the previous biopsy, If positive, then additional biopsies would not be necessary. Due to this, they will need to consider if a trial of antifungals would be warranted in the absence of an ability (after trying) to prove fungus. ie kerion until proven otherwise. On the other hand, the escar itself could be a mass of fungal hyphae. That white area anterior to the escar may be a mass of hyphae as well. Steroids are sometimes used In a kerion with inflammation, but wouldn't be advisable in the setting of insulin-dependent diabetes. I'd see if it fluoresces, which is likely given the cats, i.e. M. Canis. I've seen zoophilic dermatophytes almost always with kittens and puppies not adult dogs and cats. Given 18 cats some may be kittens. I wonder if PCR can be done for dermatophyte.

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  4. From Khalifa Sharquie, Baghdad, Iraq: "This condition is a part of dermatitis artefacta that need psychogenic assurance,support and guidance and using topical ointment like Vaseline.The ointment will make her fingers slide off so will minimize damage to skin"

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  5. Dr. Mazlim Baseri (who wrote reference # 3) was contacted and she replied: "I read with interest the case you presented and indeed, kerion is at the top of my list.
    We cannot totally exclude granulomatous bacterial infection (botryomycosis) because from the history the patient's blood sugar control may not be optimal. Cultures of the pus would be helpful.
    Given the history of lack of response to antibiotics, exposure to cats and the inflammed, cicatricial nature of the alopecia - kerion is highly likely.
    Factitial /dermatitis artefacta is less likely given the highly inflammatory nature of the lesion.
    Wood's light may be useful but the finding depends on the type of dermatophyte involved - Microsporum canis would flouresce but not the endothrix dermatophytes like Trichophytons.
    I do treat patients with empirical antifungal while waiting for the culture results. Terbinafine, itraconazole or griseofulvin are among the common antifungals we use in Malaysia.
    A short course of antibiotics with gram positive coverage will also be helpful to treat any bacterial superinfection.
    The 'Cat's Curse' article was written to raise the awareness regarding kerion and its common presentation and I am glad if it has achieved its purpose."

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