A Case for Diagnosis

presented by

Desire Gijima (1)

Stella Matsuda MD (2)

Honolulu, Hawaii

February 22, 2011

(1) Medical Student, Mayo Clinic, Rochester, USA

(2) Dermatologist, Honolulu, Hawaii, USA
 
Abstract

54 yo woman with progressive skin lesion on knees and forearms.

Patient

54-year-old female

Duration
2 years
Distribution
Knees (bilateral), right forearm/dorsal hand, left forearm
History

Patient presented to her dermatologist with 2 year history of bruise-like discoloration of both knees. She did not note any accompanying itch, pain or malaise.  Recent eruption had progressed to the right forearm/dorsal hand with a few patches on the left forearm, prompting patient to seek medical attention. No progression of lesion occurred after presentation, except for a subtle darkening of the knee patches. Initial treatment with clobetasol cream proved futile. Full skin examination did not reveal any other area involved.

Physical Examination

Bizarre appearing annular, dull pink-pigmented patches with hint of livedo pattern symmetrically located on both knees. A much larger, less prominent annular eruption stretching from dorsal hand to elbow was noted on the right forearm. On the left forearm, two smaller, faint patches were observed. No other areas involved.

Images

Laboratory Data

Labs: normal or negative

CBC, UA, Chem profile (except for SGOT 41 ig/L (0-40), SGPT 57 iu (1-41), Bili 1.5-1.6; FBS 101 with Hb A1c 6.0%)

SPEP, IEP, ANA, RA, Anticentromere AB, Anti SCL 70, HBsAg, HepC Ab, Anti TPO Microsomal Ab,D Dimer,
Anti Thyroglobulin Ab, Cardiolipin Ab, IgA, IgM, Protein C, Protein S, Protein S Activity, Factor V Leiden Mutation, Protein C Resistance, Cryofibrinogen, and Cryoglobulin  were all negative

PT Normal (But INR Low 1.0), PTT Normal, Protein C activity 195   (70-130)
Histopathology

Hawaii Path Labs & UCSF consultation: Activated mesenchymal cells in dermis with reactive fibroblasts
Geisenger Medical Labs: Pleomorphic interstitial infiltrate
Derm Path Institute: Atypical dermal spindle cell proliferation

Immunophenotyping to characterize these cells showed that the large cells are CD45(-),CD34(-),CD43(-),CD163(-),CD117(-), CD30(-), ALK-1(-).

Further immunophenotyping showed that the large cells are Factor XIIIa(+), CD45(-), CD34(-), CD43(-), CD163(-), CD117(-), CD30(-), ALK-1(-).

Diagnosis

Necrobiosis lipoidica diabeticorum, interstitial GA (although path negative), vasculopathy, interstitial granulomatous dermatitis

Reason for presentation

Diagnostic suggestions.

Questions

Has anyone had experience with a patient showing similar findings?

References

Keywords  
Comments from Faculty and Members

Khalid Al Aboud MD, Medical Director and Consultant Dermatologist, Saudi Arabia on February 22, 2011

Please keep dermatomyositis in your differential diagnoses.

Bhushan Kumar MD, Consultant Dermatologist, Chandigarh, India on February 22, 2011

Please stain for amyloid.

Samuel Moschella MD, Professor of Dermatology, Emeritus, Harvard Medical School; Physician, Lahey Clinic, Burlington, MA, USA on March 8, 2011

Information inadequate.  Needs further workup.

I would like you to refer to an article in Archives of Dermatology, Feb 2 2006, Vol 142 - In the discussion there is a good review of IGD which should give you more direction in the necessary cutaneous and systemic history review and the necessary supportive tests to be done to arrive a definitive diagnosis.  You need more history, another biopsy with stain for mucin and for possibly a delayed distinctive vasculopathy.

Ali Faisal MD, Dermatologist, Baghdad, Iraq on March 10, 2011

Any possibility of Granulomatous pigmented purpura? (sub-type of pigmented purpuric dermatosis)

Royall Pito MD, Prof. Derm (ret) Norfolk Island, South Pacific on March 11, 2011

Well worked up case!  I agree with Dr. Moschella that interstitial granulomatous dermatitis needs to be considered. Would appreciae f/u after repeat bx are done.

Christopher Lum MD, Dermatopathologist, Queens Medical Center/ Hawaii Pathologists Laboratory, Honolulu, Hawaii/ USA on March 21, 2011

As a point of discussion, I think its worth commenting on the immunophenotype of these large cells.

These large cells are consistent with a fixed dermal dendrocyte, Factor XIIIa positive. There is lack of CD34 staining, usually seen in activated or neoplastic lesions. The large cells don't appear to be histiocytes for their negative expression of CD45 and CD163. I think it would be histiocytes and lymphocytes as the primary cells in the entities described as an interstitial granulomatous dermatitis.

The histology ALONE is similar to that described by Kamino in her description of a Pleomorphic fibroma. Clearly and clinically this is not the case. This is a flat lesion with multicentricity and bilaterality. It is assuredly reactive, but from what? If not reactive, degenerative.

Old radiation change from her very remote thyroid history? An evolving connective tissue disease? Dermal reactive change secondary to erythema ab igne? Could this be dermal change from an unintentional, but self-induced process?

Dirk Elston MD, Dermatologist/ Dermatopathologist, Geisinger Medical Centre, Danville, PA, USA on March 25, 2011

The atypical cells could be fibroblastic or histiocytic in nature.  I recommend CD68 staining to confirm.  If positive, it might be reasonable to try the various treatments that have been used for GA.

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