Painful Nodule of the Helix

Presented by: Charles LaGoy, MS IV
University of New England College of Osteopathic Medicine

Abstract: 57 yo man with one year history of a painful nodule on helix.
History: This retired jockey presented to the dermatologist for a general skin exam and incidental complaint of a painful nodule on the left ear helix which had interfered with his sleep. He had not sought treatment for this previously. Patient is recovering from a Whipple procedure performed after a pancreatic condition left him unable to eat without great pain and subsequent weight loss of 45 lbs from his normal weight of approximately 115 lbs.

O/E: Tender nodule approximately 4mm in diameter on superior aspect of left ear helix. Some erosion with scaling at site.

Clinical Photo(s): These are representative photos since we did not take pictures of this man.





Easily constructed foam ear guard.

Lab: n/a

Histopathology: n/a
Diagnosis or DDx: Chondrodermatitis nodularis chronica helicis - CNH (A.K.A. painful nodule of the ear)

Questions: n/a
Reason Presented: The primary care physician is likely to encounter (CNH), also known as painful nodule of the ear. The condition is most common in males over 50 years old (1), with a male to female ratio of 10:1(2) It is thought that minor trauma to the small blood vessels supplying the pinna (1), perhaps coupled with exposure to solar radiation (3), leads to tissue damage and that inadequate blood supply prevents healing (1).
While Fitzpatrick’s Dermatology text notes that surgical excision is the definitive treatment (1), a recent small study by Moncrieff and Sassoon suggests that mechanical protection of the site is an effective conservative treatment with a lower recurrence rate than surgery (4). Their described treatment gave the patients significant leeway in designing a method of protecting the pinna from pressure while sleeping (3rd photo). The results were good with only a 13% failure rate compared to a 34% recurrence rate within a mean of 13 weeks for the surgery group (4).
The establishment of the diagnosis of CNH was left to clinical judgment in this study, whereas other authors believe that a biopsy is always required to rule out a neoplastic process (2). It is likely that this belief would complicate a decision to treat conservatively, as it might be reasonable to excise the entire nodule during the biopsy. However, a clinician with confidence in the diagnosis would likely serve the patient well by presenting the conservative treatment of these lesions, holding biopsy and/or excision for those cases that fail to respond adequately.

References:

1) Freedberg I, et al. Eds, Fitzpatrick's Dermatology in General Medicine, 6th ed. New York, McGraw-Hill 2003

2) Avitia S, Hamilton J, Osborne R, Chondrodermatitis nodularis chronica helicis. Ear, Nose and Throat Journal 2005 Jul 84(7) P406-407

3) Goette D, Chondrodermatitis nodularis chronica helicis: a perforating necrobiotic granuloma. Journal of the American Academy of Dermatology, 1980 Feb; 2(2):148-54

4) Moncrieff M, Sassoon E, Effective treatment of chondrodermatitis nodularis chronic helicis using a conservative approach. British Journal of Dermatology, 2004; 150: 892-894

Tinea incognito

Abstract: 48 yo man with 8-10 month history of a hand dermatitis
History: This patient has had a dermatitis of the dorsum of his right hand for 8 - 10 months. He saw his PCP on a number of occasions and was treated with triamcinalone 0.1% cream and clobetasol 0.05% ointment (a super potent topical corticosteroid!). Some of the redness and scale improved, but process recurred when topicals were stopped. Good general health. Takes no meds by mouth.
O/E: Fairly well-demarcated dermatitis on dorsum of right hand. No scale. There are faint papules which may represent areas of increased fungal load.
Clinical Photo(s)




Lab: KOH pep loaded with septate hyaline hyphae.
Histopathology: N/A
Diagnosis or DDx: Tinea incognito
Questions: N/A
Reason(s) Presented: Topical steroids can change appearance of tinea. They suppress local inflammation and ablate the scaly surface. When they are stopped the process recurs. The corticosteroid facilitates fungal growth by suppressing local inhibitory factors. The tip off here was the localization on one hand and the relatively sharp border. There's a saying "Do a KOH prep when it is indicated and do one when it isn't indicated and you won't make many mistakes."
Reference:
Tinea incognito in Italy: a 15-year survey.
Mycoses. 2006 Sep;49(5):383-7.
Romano C, Maritati E, Gianni C
Institute of Dermatological Sciences, University of Siena, Via Monte Santo 3,

Tinea incognito or steroid modified tinea is a dermatophytic
infection in whichtopical or systemic steroids, administered
as a result of dermatological misdiagnosis or preexisting
pathologies, have modified the clinical appearance of the fungal
infection, transforming the typical ringworm and mimicking other
skin diseases. This is a retrospective study of the agents,
clinical aspects, sources of infection of 200 cases (98 males,
102 females, mean age 42 years) of tinea incognito, observed in
Siena and Milan, Italy, in the period 1987-2002. The clinical
appearance of the infection was lupus erythematosus discoid-like,
eczema-like, rosacea-like, especially on the face, impetigo-like
and eczema-like on trunk and limbs. Less often the dermatophytosis
resembled psoriasis, purpura, seborrhoic dermatitis and lichen
planus. There was folliculitis in 9% of cases and dermatophytid
in 3% of cases. Antimycotic therapy brought about clinical and
mycological recovery in allpatients except one, who had iatrogenic
immunodepression.

Hand rash and joint pain

Abstract: 15 y/o girl with rash and joint pain for 4 weeks

History: 15 y/o girl reported having a pruritic rash on the extensor surface of her hands and wrists that started 4 weeks ago and seemed to be getting worse. She also noticed morning stiffness that lasted 1 hour and joint pain in her wrists and hands. She was started on fluoxetine for depression almost 4 months prior and had a URI 2 months prior. She has been healthy since then.

On review of symptoms pt denied constitutional symptoms, rash/pain elsewhere or GI symptoms. She denied contact with new materials/chemicals.

Pt was given ibuprofen and then naproxen without improvement. She was then given a 10-day prednisone taper starting at 60 mg without any improvement.

PMH included allergic rhinitis, depression and hearing loss. Family history revealed maternal grandfather with psoriasis, no history of autoimmune or joint problems.

O/E: Afebrile. Examination of skin revealed abrasion-like plaques on the extensor surfaces of the wrists and MCP joints. These plaques initially had an erythematous base which later became hyperpigmented. There was crusting and dry skin but no exudate or ulcerations. No nail findings. There were no skin findings elsewhere. The hands were slightly edematous but no synovial thickening or effusions were detected. The wrist, MCP and some PIP joints were tender to palpation bilaterally. The remainder of the MSK exam was unremarkable.

Clinical Photo(s): Taken at pt's 3rd visit, 4 wks after her rash first appeared

Lab: CBC unremarkable except for Eos of 8%. ESR 22. CRP, RF, ANA were negative.
Histopathology: pending

Diagnosis or DDx: ? JRA, psoriatic arthritis, drug rxn, pseudo-PCT, post-viral exanthem...
Questions: Any more ideas? Does lack of response to prednisone change the DDx/plan? (Pt was referred to rheumatology) Thanks.
Reason(s) Presented: Interest
References:
Presented by: Stephanie Chu MS4

Bazex Syndrome

Presented by: Dr. Eric Ersham

Abstract: 45 yo man with progressive hyperkeratosis on his fingers, toes and ears.

History: This 45-year-old man consulted us for a hyperkeratosis on his fingers, toes and ears which had been progressive for 6 months.

O/E: Hyperkeratosis localized on toes, fingers, and ears (helix).
The physical exam revealed a hard left cervical lymph node.

The patient was referred to an ENT specialist whose examination revealed two carcinomas: one on the left tonsil and one on the oesophagus.

Clinical Photos:






Laboratory: N/A

Histopathology: N/A

Diagnosis: Bazex's syndrome (Acrokeratosis neoplastica)

Comments: Considering the diagnosis of Bazex's syndrome lead to the discovery of the cancers of the tonsil and oesophagus in this patient. “Bazex is a rare psoriasiform dermatosis that is consistently associated with internal malignancy, usually squamous cell carcinoma (SCC) of the upper aerodigestive tract. Skin manifestations include acute onset of onychodystrophy and violaceous scaly patches on the helices, nose, and malar and acral surfaces.” (See Reference) Usually, skin manifestations precede diagnosis of the malignancy. This is an example of a paraneoplastic syndrome.

References: Acrokeratosis Neoplastica: There is a good chapter on eMedicine

Comment: This case is presented for interest. It illustrates the importance of considering paraneoplastic disorders.

Thank you,

Eric Ehrsam
Dermatologist
Graz, Austria

Pigmented Patch and Congenital Deafness

Abstract: 47 yo woman with an unusual pigmented lesion
Presented by: Mubina Nasrin and David Elpern
History: This 47 year old woman has had a slowly progressive pigmented patch on the posterior left shoulder since childhood. It has gotten darker over many years. In addition she has congenital sensoryneural hearing loss (as does a brother). His skin is reportedly normal.
O/E: There is a solitary large, somewhat "s" shaped lesion measuring 15 x 5-6 cms over the inferior aspect of the Left shoulder and upper back. The patch has speckled pigmented macules, well defined margins and a smooth surface.
Clinical Photo(s)

Lab: N/A
Histopathology: None done
Diagnosis or DDx: ? Speckled Lentiginous Nevus
Questions: Is this patient's deafness related to the pigmented lesion? Does she have the "speckled lentiginous nevus syndrome." DEafness has not been reported with this disorder in the past.
Reason(s) Presented: For discussion and ideas.
References: Rudolf Happle. Speckled lentiginous nevus syndrome: Delineation of a new distinct neurocutaneous phenotype
European Journal of Dermatology. Volume 12, Number 2, 133-5, March - April 2002
Summary : Speckled lentiginous nevus syndrome, a so far unrecognized cutaneous phenotype associated with neurological anomalies, is postulated on the basis of the following arguments. Phacomatosis pigmentokeratotica represents a twin spot phenomenon. One isolated half of this complex phenotype is observed rather often in the form of Schimmelpenning syndrome, whereas the other half of this twin nevus syndrome consists of a speckled lentiginous nevus associated with various neurological abnormalities incompatible with Schimmelpenning syndrome, such as hyperhidrosis, muscular weakness and dysesthesia. This second component of phacomatosis pigmentokeratotica may likewise occur separately. For the association of speckled lentiginous nevus with hyperhidrosis, muscular weakness, dysesthesia or other neurological abnormalities, the term "speckled lentiginous nevus syndrome" is proposed. Some case reports that may be categorized as examples of this new syndrome are reviewed. The postulated new phenotype is tentatively categorized as a paradominant trait. Future clinical studies will probably confirm the existence of speckled lentiginous nevus syndrome as a distinct neurocutaneous phenotype.

Eyelid Tumors

Submitted by Arash Abtahian from Shiraz, Iran
Abstract: 24 yo man with three year hx of eyelid lesions
History: This 24 man has come with 3 years hx of multiple translucent papule and nodules on the left upper lid. They are asymptomatic but he'd like them removed.
O/E: revealed multiple translucent papules and nodules some of which showed bluish hue.
Clinical Photo(s):



Lab: N/A
Histopathology: Apocrine Hidrocystoma
Diagnosis or DDx: Apocrine Hidrocystoma
Questions: What is the best treatment?
Reason(s) Presented: Therapeutic suggestions?
References: Apocrine Hidrocystoma eMedicine: "Apocrine hidrocystomas can be incised and drained; however, electrosurgical destruction of the cyst wall often is recommended to prevent recurrence. Punch, scissors, or elliptical excision also can remove tumors. Multiple apocrine hidrocystomas can be treated with carbon dioxide laser vaporization. Multiple apocrine hidrocystomas can also be effectively treated with trichloroacetic acid."

Black Papule in Child

Abstract: 11 yo boy with long history of black lesion left arm
History: The lesion has been present for years, little change but patient and parent are worried bout it.
O/E: 5 mm diameter papule with a peculiar serrated border
Clinical Photo(s)














Dermoscopic Image

Lab:
Pathology:

Diagnosis or DDx: Reed Nevus (aka Pigmented spindle cell nevus)
Questions: For inerest
Reason(s) Presented: When I first saw this patient, I thought it was a blue nevus. I sent the history and digital images to a few friends. Drs. Ian McColl and Stelios Minas thought it was a Reed nevus and recommended excision. Biopsy was done and confirmed their opinion.
References: The Reed Nevus is considered a variant of the Spitz nevus. It presents as a well-circumscribed deeply pigmented nodule on the extremities of young adults. Under the microscope, it resembles the Spitz nevus but is composed of spindled melanocytes with heavy melanin pigmentation. As in a Spitz nevus, this nevus is symmetrical with maturation and lack of deep dermal mitotic figures. Excision of suspected Reed nevi is recommended. For more information see: The Doctor's Doctor