Shades of Gray

Presented by Makayla Powers, PA-C

The patient is a 55 yo woman who presents with a six month history of hyperpigmentation on the presternal area, the right axilla, the posterior neck and the mid lower back.  It was initially thought to be CARP (Confluent and Reticulated Papillomatosis) and she was treated with doxycycline.  After one month of therapy there was no improvement.

O/E: Well-defined tan patches presternal.  More grayish areas on posterior neck and back.  Small hyperpigmneted macules in the right axilla.

Pathology: Courtesy of David Jones, M.D., Dermatopathologist, Berkshire Medical Center

The low power image shows a sparse but appreciable lichenoid and interface dermatitis that involves the papillary dermis and extends down the follicular elements. Higher power images identify the extensive pigment incontinence with melanophage that are associated with colloid/cytoid bodies. The vacuolar changes are best represented on the high power images that highlight rare dead and dying keratinocytes in the lower third of the epidermis. A pancytokeratin stain cross reacts with the colloid bodies as can be seen on macular amyloidosis.

Pancytokeratin Stain

The histological differential diagnosis for this pattern is fairly extensive and would require a distinct clinico-pathologic evaluation. Histologic differentials include but are not limited to non-specific post inflammatory pigment alteration, a fixed drug eruption, macular amyloidosis, Eryhtema dyschromicum perstans (EDP), and lichen planus pigementosum (LPP). 

The lack of a thick granular layer bodes against a true atrophic lichen planus or lichen planus pigmentosum. The lack of a deep dermal pigmentation makes ashy dermatosis less likely.

Discussion: Based on clinicopathological correlation, we favor Lichen planus pigmsntosum versus ashy dermatitis (EDP).  We are not confident that we can be more specific.  She was on no medications that could have contributed.  Since neither of these entities are predictably amenable to therapy and can improve over time, we are going to temporize and follow the patient in a few months. She is not eager for treatment at this time.

We welcome your comments.  Please send them to djelpern@gmail.com and they will be posted. 

References: 

1. Ashy Dermatosis and Lichen Planus Pigmentosus: The Histopathological Differences. Rutnin S, Udompanich S, Pratumchart N, Harnchoowong S, Vachiramon V. Biomed Res Int. 2019 Oct 28;2019:5829185. doi: 10.1155/2019/5829185. eCollection 2019. PMID: 31781623. Free PMC article.

2. Kumarasinghe SPW, et. al.  A global consensus statement on ashy dermatosis, erythema dyschromicum perstans, lichen planus pigmentosus, idiopathic eruptive macular pigmentation, and Riehl's melanosis. Int J Dermatol. 2019 Mar;58(3):263-272.
Abstract: Ashy dermatosis (AD), lichen planus pigmentosus (LPP), erythema dyschromicum perstans (EDP), and idiopathic eruptive macular pigmentation are several acquired macular hyperpigmentation disorders of uncertain etiology described in literature. Most of the published studies on these disorders are not exactly comparable, as there are no clear definitions and different regions in the world describe similar conditions under different names. A consensus on the terminology of various morphologies of acquired macular pigmentation of uncertain etiology was a long-felt need. Several meetings of pigmentary disorders experts were held to address this problem. A consensus was reached after several meetings and collation of e-mailed questionnaire responses and e-mail communications among the authors of publications on the above conditions. This was achieved by a global consensus forum on AD, LPP, and EDP, established after the 22nd International Pigment Cell Conference held in Singapore in 2014. Thirty-nine experts representing 18 countries participated in the deliberations. The main focus of the deliberations was terminology of the conditions; as such, we present here the consensus statement of the forum and briefly review the available literature on the subject. We have not attempted to discuss treatment modalities in detail.

3. Albert Wu, Sachin Vaidya.  Literature Review of Treatment Outcomes for Lichen Planus Pigmentosus, Erythema Dyschromicum Perstans, and Ashy Dermatosis. J Cutan Med Surg. 2018 Nov/Dec;22(6):643-645.  The conclusion was that there are not good treatments for either of these.

We asked OpenEvidence how to differentiate between Lichen planus pigmentosis and ashy dermatitis (erythema dyschromicum perstans and the answer was that it is difficult.  Also, no practical suggestions were forthcoming regarding a likely therapy.  Here is a link to the OE report.

 

Treatment Refractory Facial Dermatitis

History This 62 yo woman has had a facial dermatitis for 18 months. It has persisted despite treatment with triamcinalone cream, mupirocin ointment, metronidazole gel and oral doxycycline.  Her medications include atorvastin, bupropoin, citalopram and antiprzoale.  

Clinical: There is an erythematous papular eruption of the perioral region with some extension to the nasolabial folds and left lower lid.  Scraping for Demodex mites showed only one or two mites.

Clinical Image:

 

Photomicrographs: Courtesy of Dr. David Jones, Berkshire Medical Center

There are well formed granulomas localized to the upper and mid dermis predominantly,

 associated with follicular elements. Giant cells are present and there is a cuff of bland lymphocytes as well as a rare focus of central fibrinous debris. 

 

 

 

 

                                         

Diagnosis:  Granulomatous Rosacea is the best fit. 
In the differential diagnosis are Lupus milliaris dissemanituts faciei and rosacea. 

 References: 

1. K F Helm  1 , J Menz, L E Gibson, C H Dicken A clinical and histopathologic study of granulomatous rosacea.  J Am Acad Dermatol 1991 Dec;25(6 Pt 1):1038-43.

Abstract: A retrospective clinical and histopathologic study of 53 patients with granulomatous rosacea was undertaken. The patients had a broad clinical spectrum of lesions that ranged from primarily erythema to papulonodular lesions. Extrafacial lesions occurred in 15% of patients. Histologic examination showed mixed lymphohistiocytic inflammation (primarily lymphocytic inflammation in 40% of patients and primarily histiocytic with a few giant cells in 34%), epithelioid granulomas in 11% of patients, and epithelioid granulomas with caseation necrosis in 11%. Most patients had a good response to oral antibiotic therapy. Granulomatous rosacea is not a distinct disease but can be regarded and treated as a subtype of rosacea.

2. Efstathios Rallis and Chrysovalantis Korfitis. Isotretinoin for the Treatment of Granulomatous Rosacea: Case Report and Review of the Literature.
Abstract: Granulomatous rosacea is considered a clinical variant of rosacea and is characterized by hard yellow, brown, red or flesh-colored cutaneous papules or nodules that may be severe and may lead to scarring. The lesions typically appear on the cheeks and periorificial lesions; they are monomorphic in each patient and sit on relatively normal-appearing skin. The diagnosis should be established by excluding other granulomatous disorders and rosacea-like eruptions such as sarcoidosis, tuberculosis, and lupus miliaris disseminatus faciei. The clinical course is chronic and unpredictable, and management can be very difficult. 

3. Mitchell Brady, Vera Wang, Francesca Kartono, Lynn Sikorski. Granulomatous rosacea treated with tapinarof. JAAD Case Rep. 2025 Jul 11:64:1-2.  PMC Full Text 

 

Giant Adult Hemangioma

 presented by Drs. Douglas Johnson and George Reizner

(Note: We recognize that the person's eyes should be blacked out to preserve anonymity; but have decided not to do so initially as seeing this man as he is may help when thinking about therapy.  After 1 - 2 weeks, we will change the image to comply with standard presentations) 

The patient is an otherwise healthy 94 year old man who lives in a rural Amazonian village that has limited access to medical care. History reveals that he has had a growing vascular lesion of the left elbow for at least three years.  Biopsy showed a capillary hemangioma without atypical features.  He has congestive heart failure and hypertension but his only medication is metoprolol.  He declined surgery in 2024. His only abnormal finding is that he cannot dorsiflex his left thumb.

 

                MRI shows a well-demarcateds bright hyperintense mass

Pathology: Biopsy is reported to show a proliferation of blood vessels without atypia suspended in a fibrous stroma,  This image was taken from the path report.

Diarnosis:  Giant Hemangioms of the left elbow.  Another dermatopathologist questioned whether this is an angiosarcoma.

Few dermatologists have much experience with lesions like this.  What therapy, if any, should be offered this patient?  He lives in a medically underserved rural village..