BRIEF ARTICLE A Rare Presentation of Self-Resolving Purple Fingers

syndrome, also known as paroxysmal finger hematoma, is a rare, benign, self-limiting condition of unknown etiology. Patients typically present with episodic pain, swelling, and paresthesias in one or more digits accompanied by a subsequent hematoma localized to the palmar aspect of the digits. The hematoma spontaneously resolves within 2-14 days without going through the typical stages of ecchymosis. Risk factors such as trauma, bleeding disorder, or rheumatologic disease are not known to be associated with the etiology of Achenbach syndrome. It is thought that rupture of superficial veins and resultant hemorrhage can cause the signs and symptoms of the disease. We report a case of a 66-year-old female diagnosed with Achenbach syndrome after excluding other possible diagnoses. A 66-year-old Caucasian female presented to the dermatology clinic with waxing and waning non-palpable, purpuric, patches on the volar aspect of her hands bilaterally


INTRODUCTION CASE REPORT
Figure 1.Non-palpable, purpuric, patches on the volar aspect of multiple digits bilaterally.presented 1.5 years prior.She experienced tingling prior to the onset of the lesions and mild tenderness when the purpura was present.She denied any trauma to her hands.The episodes would occur from multiple times a month to monthly and spontaneously resolve within 2-7 days.No residual pigmentation was present after the lesions resolved.
The patient was evaluated by rheumatology prior to being evaluated by dermatology.Rheumatology ordered a complete blood count, complete metabolic panel, and coagulation studies which were unremarkable.Additional labs revealed a normal erythrocyte sedimentation rate (ESR) 27 and C-reactive protein (CRP) <3.0.Antinuclear antibody (ANA) , anti-double stranded DNA (dsDNA), anti-Jo 1, anti-Sjogren's syndrome A (SSA/Ro), anti-Sjogren's syndrome B (SSB/La), antiribonucleoprotein (RNP), anti-scleroderma 70 (Scl-70), anti-smith antibody (Sm), antimyeloperoxidase, and anti-proteinase 3 (PR3) were all negative.Anti-cardiolipin antibodies were also negative and complement studies were normal.Rheumatology did not find any evidence of vasculitis or connective tissue disease.A punch biopsy of the lesion was performed in the dermatology clinic.Hematoxylin and eosin revealed a perivascular lymphocytic infiltrate, extravasated erythrocytes, and pigmented macrophages consistent with a pigmented purpuric dermatosis (Figures 2  and 3).Immunofluorescent studies were negative.
The patient was diagnosed with Achenbach syndrome by diagnosis of exclusion.The patient was reassured of the benign nature of this syndrome and that no further workup was required.She was given DerMend bruise formula cream containing retinol, glycolic acid, arnica oil, ceramides, niacinamide, and phytonadione to use when her symptoms recurred.Achenbach syndrome, first described in the 1950's by German physician Walter Achenbach, is a rare, benign condition characterized by episodes of pain, swelling, and paresthesia followed by purpuric discoloration of the palmar aspect of the digits. 1 Presenting more often in women around their 5th decade of life, the discoloration of Achenbach syndrome typically resolves without treatment between 2 and 14 days. 2The most common location of presentation are the index and middle fingers, although, it has been reported that discoloration can impact the thumb, dorsal surface of the hand, and even the feet and toes. 3The etiology of the disease is not clear, but it is thought to be caused by fragility of superficial veins with resultant rupture and hemorrhage. 4Risk factors such as trauma, drug use, bleeding disorder, or rheumatologic disease are not known to be associated with Achenbach syndrome.However, history of acrocyanosis, gastrointestinal disorders, migraines, and gallbladder disease have been reported associations in the literature. 5,6dditionally, genes responsible for blood coagulation and inflammation, namely the F3, F2, and CRP genes, have been weakly linked to familial Achenbach syndrome. 7agnostic workup of a case of Achenbach syndrome will reveal normal complete blood count, coagulation markers, and inflammatory markers. 8Allen's test, arterialbrachial index, pulse oximetry, a Doppler ultrasound, and an echocardiogram will all be normal.Included in the differential diagnosis will be trauma, acute limb ischemia, thrombosis, Raynaud's syndrome, autoerythrocyte sensitization syndrome, factitious dermatitis, vasculitis, acrocyanosis, Gardner-Diamond syndrome, erythromelalgia, pernio, and drug-induced hematoma.Histopathology slides denote dermal hemorrhage, extravasated erythrocytes, and no evidence of vascular damage.Patients should be reminded of the benign nature of the disease and informed that no further workup is necessary.Treatment is not required because Achenbach syndrome is self-limiting, but sargegrelate hydrochloride, acetysalicyclic acid, long acting diprimadol, heparin, isosorbidedinitrate, mucopolysaccharidepolysuplphate gel, and cold applications have been reported. 5In our case, we provided DerMend bruise cream for the patient to use upon recurrence of symptoms.
Achenbach syndrome is a rare, benign condition marked by self-resolving hematoma on the palmar aspect of the digits.The underlying cause of this disease is unknown although genetic links to thrombotic pathways have been suggested.If Achenbach syndrome is diagnosed, physicians should avoid unnecessary testing and advise patients of its benign prognosis.

Figure 3 .
Figure 3. Higher power view of punch biopsy from acral skin revealing a perivascular lymphocytic infiltrate and extravasated red blood cells.